Hyper-IgG4 disease: report and characterisation of a new disease
© Neild et al; licensee BioMed Central Ltd. 2006
Received: 17 May 2006
Accepted: 06 October 2006
Published: 06 October 2006
We highlight a chronic inflammatory disease we call 'hyper-IgG4 disease', which has many synonyms depending on the organ involved, the country of origin and the year of the report. It is characterized histologically by a lymphoplasmacytic inflammation with IgG4-positive cells and exuberant fibrosis, which leaves dense fibrosis on resolution. A typical example is idiopathic retroperitoneal fibrosis, but the initial report in 2001 was of sclerosing pancreatitis.
We report an index case with fever and severe systemic disease. We have also reviewed the histology of 11 further patients with idiopathic retroperitoneal fibrosis for evidence of IgG4-expressing plasma cells, and examined a wide range of other inflammatory conditions and fibrotic diseases as organ-specific controls. We have reviewed the published literature for disease associations with idiopathic, systemic fibrosing conditions and the synonyms: pseudotumour, myofibroblastic tumour, plasma cell granuloma, systemic fibrosis, xanthofibrogranulomatosis, and multifocal fibrosclerosis.
Histology from all 12 patients showed, to varying degrees, fibrosis, intense inflammatory cell infiltration with lymphocytes, plasma cells, scattered neutrophils, and sometimes eosinophilic aggregates, with venulitis and obliterative arteritis. The majority of lymphocytes were T cells that expressed CD8 and CD4, with scattered B-cell-rich small lymphoid follicles. In all cases, there was a significant increase in IgG4-positive plasma cells compared with controls. In two cases, biopsies before and after steroid treatment were available, and only scattered plasma cells were seen after treatment, none of them expressing IgG4. Review of the literature shows that although pathology commonly appears confined to one organ, patients can have systemic symptoms and fever. In the active period, there is an acute phase response with a high serum concentration of IgG, and during this phase, there is a rapid clinical response to glucocorticoid steroid treatment.
We believe that hyper-IgG4 disease is an important condition to recognise, as the diagnosis can be readily verified and the outcome with treatment is very good.
Systemic fibrosis synonyms
Pseudotumour, inflammatory pseudotumour, fibrous pseudotumour
Lung[86-89], liver[87,90-93], breast, pancreas[83,95-99], orbit, RPF[98-101], mesentery
Inflammatory myofibroblastic tumour, myofibroblastoma
Pancreas, lung[46,103,104], brain, breast
Plasma cell granuloma
Systemic fibrosis, generalized form of Ormond's disease, systemic idiopathic fibrosis, idiopathic systemic sclerosing disease
PRF[109,111-113], orbit, lung[114,115]
RPF[101,118-121], brain[118,122], orbit
Multifocal fibrosclerosis, multifocal idiopathic fibrosclerosis
RPF[124,125,128-133], orbit[124,129-131,134] thyroid[127,128,132,133], pancreas
Conditions associated with systemic fibrosis
Systemic[109,111,113,135], lung[32,32,53,115,136,136,137], liver, breast, pancreas[5,139-141],
Fibrous pseudotumour of the orbit, Graves' orbitopathy
Chronic sclerosing sialadenitis
Weber-Christian syndrome steatonecrosis, necrosing panniculitis
Systemic, RPF[135,152-155] Biliary cirrhosis, pancreas
Primary inflammatory pancreatitis (P), lymphoplasmacytic sclerosing P[7,160,161], autoimmune P, sclerosing pancreaticocholangitis, pancreatic pseudo-tumour
RPF[5,97,139,161,164,165], cholangitis[95,160,164,166,167], systemic[83,168-171] gastric ulcer, lung[173,174] panniculitis, mesentery
Bronchiolitis obliterans with organizing pneumonia
Cryptogenic organizing pneumonia, pulmonary hyalinizing granuloma.
Systemic[50,176], pancreas[173,174], liver, RPF[115,136,137], renal[46,136], mediastinum[178,179]
Benign pleural mesothelioma[180,181], calcifying pseudotumour[182,183]
RPF[11,26-28,32,32] peritoneum[56,184,185] pleura[54,55,186]
In another patient, we observed the rare behaviour of idiopathic RPF behaving like a tumour, in which the inflammatory mass was invading both the kidney and liver and presented as a progressive cholangitis. This case was one of a series of patients seen in our gastroenterological department, who had sclerosing pancreatitis and chronic inflammation associated with immunoglobulin (Ig)G4-expressing plasma cells [3, 4]. In biopsies from both these cases, we found typical fibrosis with lymphoplasmacytic inflammation and IgG4-bearing plasma cells .
Sclerosing (autoimmune) pancreatitis is a unique form of pancreatitis, characterized by hypergammaglobulinaemia and a lymphoplasmacytic inflammation of the pancreas that responds to glucocorticoid treatment. It has several synonyms (Table 2). A Japanese group investigating this condition  found that the sera of their patients had a polyclonal band in the rapidly migrating fraction of gammaglobulins, which was caused by a high concentration of the IgG4 gammaglobulin fraction. They also reported that serum concentrations of IgG4 were significantly and specifically raised in patients with sclerosing pancreatitis, and were closely associated with disease activity .
The same Japanese group found that of the 22 patients with sclerosing pancreatitis they studied, three also had concomitant hydronephrosis caused by a periureteric mass, diagnosed as RPF (Ormond's disease). Histological examination of the periureteric tissue showed abundant infiltration of IgG4-bearing plasma cells. Treatment with corticosteroids lowered serum concentrations of IgG4, and the authors proposed that IgG4 might have a pathological role in a systemic fibrosing process that includes pancreatic and retroperitoneal lesions .
Kamisawa et al have also published several reports emphasising the systemic nature of this process. Patients with autoimmune pancreatitis may also have involvement of lymph nodes and salivary glands as well as local tissues [7, 8], in a pattern suggestive of multifocal fibrosclerosis [9, 10].
We describe our index case, and report on the histological features of 11 other patients seen at our hospital with idiopathic RPF, for whom we have now been able to review the histology and examine sections for evidence of IgG4-expressing plasma cells. We also list the synonyms and disease associations that we believe are all part of the same disease process.
We report on the histological features of 16 biopsy specimens from 12 patients seen at the Middlesex hospital with a primary diagnosis of RPF, for whom we have now been able to review the histology and examine sections for evidence of IgG4-expressing plasma cells. Only archival paraffin wax-embedded material was used for this study. All the biopsies were taken for diagnostic reasons with the informed consent of patients.
All the samples were routinely fixed in formalin and embedded in paraffin wax. Dewaxed sections (μm) were stained with haematoxylin and eosin. Immunohistochemical analysis was performed using antibodies (all Dako UK Ltd, Cambridgeshire, UK) against CD3, CD4, CD8, CD20, and CD138, and the kappa and lambda light chains of immunoglobulins, by the standard streptavidin-biotin-peroxidase method. For IgG4, a mouse human IgG4 monoclonal antibody was used (1:100 dilution, MC011; The Binding Site, Birmingham, UK). Negative controls were created by substituting the primary antibody with similarly diluted non-immunized mouse serum.
We examined a wide variety of other inflammatory conditions and fibrotic diseases as organ-specific controls, which included liver (autoimmune hepatitis, chronic hepatitis C), pancreas (chronic and alcoholic pancreatitis), salivary gland (chronic sialadenitis), gallbladder (chronic cholecystitis), kidney (tubulointerstitial nephritis), duodenum (chronic duodenitis), colon (non-specific colitis), and other fibrotic processes (Dupuytren's, keloid scars). Because an association between RPF and asbestos exposure has been reported , we also included cases of malignant mesotheliomas. In addition, specimens from cases of "benign pleural plaques" were stained as controls.
Positive controls comprised tissue obtained from pancreas, salivary gland and liver from patients seen in our gastroenterological department who had sclerosing pancreatitis and chronic inflammation associated with IgG4-expressing plasma cells, details of which have been reported previously [3, 4].
The degree of inflammation was assessed as severe [>100 inflammatory cells per high power field (), moderate (30–100 cells/HPF) and mild (30–10 cells/HPF). The presence of lymphoid-follicle formation, eosinophilic infiltration, venulitis and obliterative arteritis (on elastic van Gieson-stained sections) was also recorded. The number of IgG4-positive cells was estimated by counting 10 HPF, and scoring as follows: 0 (no IgG4 plasma cells/HPF), 1 (≤ 20 cells/HPF), 2 (20–50 cells/HPF) or 3 (≥ 50 cells/HPF) . Fibrosis was assessed as - (no fibrosis), + (mild), ++ (moderate) or +++ (severe).
Rare associations with IgG4-related conditions (Case reports)
Head and neck
Submandibular gland fibrosis
Pancreas, cholangitis[187,188], pseudotumour
Pancreas[171,190], RPF, BOOP
Hashimoto's disease, Graves' disease
Other autoimmune conditions
Systemic lupus erythematosus
Idiopathic thrombocytopenic purpura
Central nervous system
Spinal cord compression
Primary biliary cirrhosis
Familial multifocal fibrosclerosis Familial RPF
Proliferative crescentic glomerulonephritis, membranous glomerulonephritis
Many of these reports are case reports. We have not attempted to list every report but rather to draw up a comprehensive list of all possible associations with at least one citation.
A 39-year-old Moroccan steel fitter had lived in the UK for 31 years. He presented in May 2003 with abdominal pain, weight loss, sweating, generalized lymphadenopathy, and severe anaemia and thrombocytosis. Investigations showed haemoglobin of 6.7 g/dL (normal range 13.0–17.0 g/dL), platelets 1031 × 109/L (150–400 × 109/L), erythrocyte sedimentation rate (ESR) 120 mm/h (0–15 mm/h), IgG 21.4 g/L (8.0–18.0 g/L), IgA 2.9 g/L (0.9–4.5 g/L), IgM 0.6 g/L (0.6–2.8 g/L) and C-reactive protein (CRP) 289 mg/L (normal 0–5 mg/L). Biochemistry was normal. Chest X-ray showed apical pleural thickening. An intravenous urogram (IVU) showed left hydronephrosis; an ultrasound scan (USS) confirmed this, and showed dilatation of the bile and pancreatic ducts and the gallbladder. Computed tomography (CT) showed multifocal upper zone and patchy nodular airspace shadowing, with right posterior pleural thickening and subcarinal lymphadenopathy. Biopsies of pleural, ileal and colonic tissue, bone marrow, and left inguinal lymph node were all either normal or showed reactive change. Cultures of blood, urine and sputum were all negative. The patient was also negative for human immunodeficiency virus types 1 and 2, hepatitis B and C, human T-lymphotropic virus, toxoplasmosis and treponemal disease. Mantoux test was weakly positive, and although bronchoalveolar lavage was smear-negative, there was one positive culture for a mycobacterium after 3 weeks.
The patient was commenced on quadruple therapy for tuberculosis (TB) in July 2003, for an organism that was strongly isoniazid-resistant. The patient presented again in November with fatigue, weight loss and fever, which was thought to be medication-related. He was given prednisolone 20 mg for 6 weeks of, taken off all TB therapy, and after 2 months was feeling so well that he was told his TB had been cured.
He re-presented 3 months later (March 2004) with a 6-week history of night sweats, low-grade fever, lethargy, weight loss, left pleuritic chest pain and axillary lymphadenopathy. Blood tests again showed microcytic anaemia, and the same acute phase response. Antinuclear antibody (ANA), antineutrophil cytoplasmic antibodies, mitochondrial antibodies and smooth-muscle antibodies were all negative. CT showed many of the previous changes but with a right hydronephrosis that was confirmed on IVU (left hydronephrosis was no longer present). A repeat bone-marrow biopsy, inguinal lymph node and transjugular liver biopsies, endoscopy, colonoscopy, peritoneal fluid aspiration and bone scan were all either normal or non-specific. The patient was treated with quintuple anti-TB therapy in order to cover his original mycobacterium infection as well as an infection with Mycobacterium fortuitum that had been cultured from sputum on this occasion. He was also treated with steroids, initially 60 mg of prednisolone, tapered down to 20 mg over 3 months.
The patient was readmitted 6 months later with generalized abdominal pain and vomiting. He had stopped his steroids 11 days earlier because of side effects. Investigations showed only a marked inflammatory response. CT scan revealed no hydronephrosis, but now showed dilated, thick-walled small bowel in the left iliac fossa. During an exploratory laparotomy (October 2004), an inflamed omental mass adherent to the small-bowel mesentery was removed. Histology was reported as showing only some fibrosis with a mild chronic inflammatory cell infiltrate of lymphocytes and plasma cells. The patient was recommenced on steroids and quadruple anti-TB therapy.
Patient no., sex, age
IgG4-positive plasma cells‡
(1) Male, 52
(2) Male, 73
(3) Male, 39
(4) Male, 53
(5) Male, 45
(6) Female, 58
(7) Female, 55
(8) Male, 47
(9) Male, 48
(10) Male, 73
(11) Female, 56
(12) Male, 53
Pathology of IgG4 disease
We reviewed the histology and examined sections for evidence of IgG4-expressing plasma cells of 16 biopsies from 12 patients with a clinical diagnosis of idiopathic RPF seen at our hospital in the past 10 years. Biopsies comprised nine samples of retroperitoneal tissue, two of liver, and one each from kidney, colon and omentum. In addition, there were two further samples of retroperitoneal tissue taken from two patients after steroid therapy.
We also examined biopsy specimens from liver (two patients), large bowel (one) and renal tissue (one). In all of them, we found mild fibrosis and a small increase in lymphoplasmacytic inflammatory cell infiltrate, although IgG4-positive plasma cells were not seen in one liver biopsy, nor in the colon biopsy (Table 4).
In summary, raised numbers of IgG4-positive plasma cells are seen in both peritoneal and non-peritoneal tissues in patients with hyper-IgG4 disease. The degree of IgG4-positive staining did not necessarily correlate with elevated serum IgG. Our clinical impression was that the number of IgG4-positive plasma cells was associated with disease activity.
RPF is probably the most common example of the conditions associated with raised serum IgG4 and an IgG4-positive plasma cell infiltrate, which we call hyper-IgG4 diseases. The other most frequently recognised associations are sclerosing pancreatitis, Reidel's thyroiditis, retro-orbital tumour, panniculitis, and sclerosing cholangitis (see Table 2 for synonyms).
It is known that RPF can be associated with a similar fibrotic process in other organs, particularly adjacent tissues such as the mediastinum and pleura. Patients with RPF can have systemic symptoms and be febrile. We used RPF and our knowledge of its aetiology and pathogenesis to review the potential mechanisms of IgG4 disease.
Idiopathic RPF generally presents in a non-specific manner with malaise, fatigue, fever and weight loss [11, 14–16]. Pain in the back (lumbosacral) region, flank or lower abdomen is most common. The pathognomonic feature of RPF is a thick retroperitoneal fibrotic mass covering the abdominal aorta and compressing the ureters. The process of fibrosis can result in obstruction of the ureters and renal failure, or signs and symptoms may be related to the encasement or entrapment of other structures by the inflammatory mass, such as hydrocoeles or unilateral leg oedema. With renal involvement, severe hypertension is common.
The disease presents typically between the ages of 50 and 70 years, and men are affected 2–3 times more commonly than women . The inflammatory nature of idiopathic RPF is demonstrated by elevated ESR and CRP, reduced haemoglobin and elevated gammaglobulins at presentation. An autoimmune nature is suggested by the finding of ANA in 10–20% of cases, an occasional association with different autoimmune diseases and good response to glucocorticosteroids. In one series of RPF associated with periaortitis, 10 of 16 patients were ANA-positive .
Although there are no randomised studies of treatment for RPF, patients who present during the acute inflammatory phase respond quickly to glucocorticosteroids. Patients with late presentation and dense fibrosis will not respond to medical treatment and surgery is often necessary.
Causes and associations
RPF has many causes, although in about 70% of cases the cause is unknown (idiopathic). The rest (secondary) develop as a result of malignant disease, radiation therapy, abdominal surgery, pancreatitis, retroperitoneal haemorrhage, urine extravasation, and infections. Carcinoid syndrome can cause systemic fibrosis , and RPF has been described without metastatic tumour .
RPF has also been associated with the use of several drugs, but most commonly the ergotamine derivates, especially methysergide  and, more recently, those used as dopamine agonists to treat Parkinson's disease, such as pergolide and cabergoline .
From an aetiological perspective, drugs associated with RPF are important as they also cause fibrosis in various other sites. Systemic fibrosis is a prominent feature of methysergide use, with involvement of heart and lungs, particularly heart valves [20, 22]. A similar distribution of pathology has been reported with the dopamine agonists pergolide and cabergoline [23–25].
Of pathogenetic importance is the observation that withdrawal of these offending drugs usually results in a rapid improvement in symptoms, reduction in inflammatory markers and regression of pathology . Drug-induced cases are not associated with ANA .
Recently, asbestos exposure has been proposed as a causal factor for both pleural and retroperitoneal fibrosis [11, 26–28]. Asbestos fibres also cause interstitial lung fibrosis (asbestosis), pleural fibrosis, pleural plaques, lung cancer, and pleural and peritoneal mesothelioma . Patients exposed to asbestos, and particularly those with asbestosis, have an increased incidence of ANA and other autoantibodies, and raised levels of IgG and IgA. There is also a correlation between the degree of immunological abnormality and the likelihood of progression [30, 31].
A case-control study in Finland of 43 patients with RPF (86% of eligible cases) found that the age-standardised incidence of RPF was 0.10 per 100000 person-years . For every patient, five population-based matched controls were selected. There was a strong association with asbestos exposure. The odds ratio (OR) was 5.5 for <10 fibre-years of asbestos exposure and 8.8 for ≤ 10 fibre-years. Other risk factors were previous use of ergot derivates (OR = 9.92), abdominal aortic aneurysm (OR = 6.73) and smoking for >20 pack-years (OR = 4.73) .
We also saw pleural calcification in three of our patients with RPF, in whom it was not present at the presentation of the illness. One of those cases is included in our archival review (patient 5, Table 4). This raises the question of whether some of the pleural pathology associated with IgG4 disease can heal with calcification as well as fibrosis .
The relationship of aortic aneurysms with an inflamed and thickened aortic wall, extensive periaortal inflammation and RPF is well known . As the inflammatory aneurysms of the abdominal aorta differ from RPF only in the diameter of the inflamed aorta, it has been suggested that both syndromes represent only variations of the same disease, which has been named 'chronic periaortitis' . RPF has been suggested as the most severe form of chronic periaortitis [35, 36]. Vasculitis of the vasa vasorum was thought to be a factor in the aneurysmal dilatation [34, 37].
Several observations suggest an immunological pathogenesis for this periaortitis. As well as the periaortal tissue, the media and adventitia of the aorta are infiltrated by polyclonal B lymphocytes, activated CD4-positive T lymphocytes, and plasma cells . In 85% of patients, the necrotic media contains deposits of IgG antibodies in close proximity to extracellular ceroid, and the serum often contains antibodies against ceroid and oxidised low-density lipoprotein .
Although it was initially proposed that the aortitis was caused by an autoimmune response to the components of atherosclerotic plaques  with antibodies to atheroma, more recent opinion has suggested that in view of the accompanying systemic symptoms and acute phase response, the aortitis is a primary autoimmune disease [17, 36, 39, 40].
Autoimmune and other rare associations
While there is no one predominant association, a large range of autoimmune diseases has been described in association with RPF (Table 3), and 10–20% of cases will have ANA or other autoantibodies.
RPF in the paediatric population is rare with, only 23 cases reported in the English-language literature by 2003 . A family has been reported in which two children had idiopathic retroperitoneal fibrosis, and they and their father had clinical and laboratory evidence of systemic immunological diseases .
Two brothers from a first-cousin consanguineous marriage were reported, who had different combinations of RPF, mediastinal fibrosis, sclerosing cholangitis, Riedel sclerosing thyroiditis, and pseudotumour of the orbit. One of the brothers had fibrotic contracture of the fingers . Other reports are of RPF in three siblings with sickle-cell trait , and mediastinal and retroperitoneal fibrosis in two sisters with seronegative spondyloarthropathy .
RPF invading adjacent organs
Rarely, the inflammatory tissue seen in RPF can invade the renal parenchyma [46–48] and it can occasionally cause a tumour-like mass away from the retroperitoneum . There are four isolated surgical case reports in the literature, with two being described as idiopathic RPF, one as systemic hyalinizing granuloma and the other as xanthofibrogranuloma. We have seen one case in which the IgG4-positive inflammatory mass (Figure 2) invaded the kidney and liver (patient 1, Table 4), and another in which it caused a large tumour like mass in the left iliac fossa (patient 2). Both cases responded promptly to steroids.
Although all our archival cases of idiopathic RPF have shown evidence of IgG4 involvement, it remains to be seen whether IgG4 plays any role in other types of RPF such as periaortitis, drug-induced and asbestos-related RPF. We certainly found it (in retroperitoneal tissue) in one of our patients who developed pleural calcification.
Part of the spectrum of disease in patients with hyper-IgG4 disease is parenchymal lung pathology, often described as either bronchiolitis obliterans with organizing pneumonia or cryptogenic organizing pneumonia . This is seen typically as migratory pulmonary infiltrates, and is steroid-responsive. Nevertheless, there are at present no studies that have looked for evidence of IgG4 plasma cells in these conditions . Other conditions associated with migratory pulmonary infiltrates that may be related, but not yet investigated, include pulmonary eosinophilia, hypersensitivity to drugs, parasitic infection, allergic bronchopulmonary aspergillosis and Churg-Strauss vasculitis . Pleural thickening, with and without calcification, is well described [32, 32, 53]. There are also reports of calcifying pseudo-tumour of the pleura [54, 55] and peritoneum .
Differential diagnosis of febrile illness
Fever of unknown origin (FUO) in an immunocompetent patient is defined as an illness of more than 3 weeks' duration with fever higher than 38.3°C (101°F) on several occasions, and with an uncertain diagnosis after 1 week of investigation . Infection accounts for about one-third of cases (25–32%) of FUO, followed by non-infectious inflammatory disease (21–31%) and neoplasm (12–17%) [58–62]. Although many patients with FUO will be found to have bacterial infection, rarer diagnoses will be considered with time and extensive investigation. Nevertheless, at the end of all investigations, 18–30% of patients will have no diagnosis, although the majority of this idiopathic group will improve spontaneously . We propose that hyper-IgG4 disease can be added to this list of systemic inflammatory diseases.
Conditions associated with elevated IgG4
IgG4 is the rarest of the IgG subclasses to be expressed, accounting for only 3–6% of total IgG in normal serum. It has a low affinity for target antigen. It is unique among the IgG subclasses, as it is unable to bind Clq complement and cannot activate the classic complement pathway . IgG4 is a T-helper 2 (Th2)-dependent isotype. Interleukin (IL)-4 directs naive human B cells to switch to IgG4 and IgE production ; IL-10 and IL-13 are the other T-cell-derived cytokines that activate this differentiation.
High serum IgG4 concentrations are found in a limited number of other conditions, including atopic dermatitis , some parasitic diseases (helminthic diseases) , and pemphigus vulgaris (PV) and pemphigus foliaceus (PF)[67, 68].
In allergic individuals, a feature of successful immunotherapy is the induction of allergen-specific IgG4 antibodies, even with the persistence of allergen-specific IgE [69, 70]. High levels of the immunosuppressive cytokine IL-10 are found both in patients chronically infected with helminths and in those receiving allergen immunotherapy . IL-10 also stimulates IgG4 production .
Th2 responses to allergens are suppressed by both CD4+ CD25+ T regulatory cells (Tregs) and IL-10-producing Tregs , and suppression by these subsets is decreased in allergic individuals. In animal models, Tregs can be induced by high- or low-dose inhaled antigen, and prior induction of Tregs prevents subsequent development of allergen sensitization and airway inflammation in inhaled challenge models. Allergen-injection immunotherapy may induce IL-10 Tregs, leading to both suppression of Th2 responses and a switch from IgE to IgG4 antibody production .
Asymptomatic infections with helminth parasites are correlated with high levels of IgG4, and parasite-specific IgG4 antibodies can inhibit IgE-mediated degranulation of mast cells . In most helminth infections, the Th2 response leads to rapid parasite expulsion or sequestration. During murine Schistosoma mansoni infection, however, parasites persist and the chronic Th2 response can induce severe pathological fibrosis in the gut and liver . Evidence both from these mouse models and from human studies suggests that this progression to severe disease with intense fibrosis occurs in only a minority of cases, and is a consequence of the strongly profibrotic type 2 cytokine response mediated by IL-4 and IL-13 .
Pemphigus and pemphigoid
PV and PF are autoimmune skin diseases caused by autoantibodies against desmoglein 3 and desmoglein 1 respectively. These antibodies are predominantly of the IgG4 subclass [67, 68]. Desmogleins are keratinocyte transmembrane proteins localized in the desmosome, and are members of the adhesion molecule family of cadherins. The interaction of antidesmoglein antibodies with their target antigens leads to loss of cell adhesion (acantholysis) and the formation of intraepithelial blisters of the skin and mucous membranes. The antibodies predominantly, but not exclusively, bind to the extracellular domains of the proteins and exert their pathogenic effect not through inhibition of the proteins' adhesive properties, but through an indirect pathway involving cellular signalling pathways, resulting in destabilization of desmoglein 1-based adhesive sites and desmosomes .
Bullous pemphigoid (BP) is an autoimmune blistering disease associated with autoantibodies against the hemidesmosomal glycoprotein BP180. The noncollagenous (NC)16A domain of BP180 has major antigenic sites recognized by BP sera. IgG4 and IgE are the major immunoglobulins that preferentially react with two distinct epitopes within this domain. Levels of these autoantibodies correlated with disease activity in BP .
In sclerosing pancreatitis, evidence had been reported to suggest an immunological pathogenesis even before the description of a role for IgG4. In one study of 17 patients, ANA and antilactoferrin antibodies were detected in 76%, carbonic anhydrase II antibody in 59%, rheumatoid factor in 29% and anti-smooth-muscle antibody in 18% of patients. CD8+ and CD4+ cells were significantly increased in peripheral blood. CD4+ cells produced increased levels of interferon gamma compared with controls, but IL-4 was not increased . However, in another Japanese study, which divided patients with sclerosing pancreatitis into a seronegative and seropositive group, found no differences in presentation, pathology or natural history between the groups .
HLA associations with IgG4-associated diseases
PV was consistently found to be associated with DR4 and DR14 and, more precisely, with DRB1*0402 and DRB1*1401 subtypes . Susceptibility to PF has been correlated with the presence of DR4, DR14 and DR1 alleles; however, in contrast to PV, no single DR4 or DR14 allele was shown to be associated with the disease.
In an immunogenetic study of patients with autoimmune pancreatitis, a significant increase in DR4 was found. It was concluded that the DRB1*0405-DQB1*0401 haplotype was associated with autoimmune pancreatitis in the Japanese population .
Aortoarteritis is a chronic inflammatory disease, mainly affecting the aorta and its major branches. In a Chinese Han population, the DRB1*04 and DRB1*07 alleles were significantly associated with aortoarteritis. Furthermore, there was no significant difference in the frequency of the DRB1*0405 subtype between the patient and control groups .
In China, the HLA-DRB5*0101 allele and the IL-13 promoter A/A genotype were both found to be elevated in schistosomal hepatic fibrosis, although the two genes are located on different chromosomes. Subjects with both genotypes had odds ratios much higher (OR = 24.5) than the sum of the ratios for each individual genotype. The study strongly suggested that a pathogenic Th2 response directly influenced the prognosis of post-schistosomal liver fibrosis.
We believe that hyper-IgG4 disease is an important condition to recognise as the diagnosis can be verified by histology and simple blood tests, and the outcome with treatment during the acute phase is very good. A raised IgG concentration in the absence of hypergammaglobulinaemia is typical, but ideally IgG4 should also be measured. Even when pathology appears localised, as with RPF, constitutional symptoms can be present with evidence of an acute phase response. Rarely a systemic form of the disease will present with severe constitutional symptoms, and hyper-IgG4 disease can be added to list of unusual causes of FUO. Although the role and relevance of IgG4 is unknown, the dense fibrosis that accompanies healing would appear to be a typical example of Th2-mediated pathology.
We thank Ms P Munson for performing IgG4 immunostaining. We thank Drs Robin Woolfson and Helen Lachmann for reading the manuscript and making valuable contributions to its preparation. We would like to thank our colleagues Dr FD Thompson, Dr MA Mansell, Dr RG Woolfson and Dr BR Leaker for their help with the management of patients. Written consent was obtained from the patient for publication of the case history.
- Streuli R, Egger C, Truniger B: Systemic fibrosis (generalized xanthofibrogranulomatosis). Nephrol Dial Transplant. 1997, 12: 608-609.PubMedGoogle Scholar
- Kastendieck H, Husselmann H: [Xanthofibrogranulomatosis. Classification, localization, morphology, pathogenesis] (author's translation). Virchows Arch A Pathol Anat Histol. 1978, 380: 237-259.PubMedGoogle Scholar
- Webster GJ, Wittman J, Seward E, Pereira SP, Hatfield AR: Autoimmune pancreatitis/choliopancreatopathy – evidence of multisystem involvement [abstract 048]. Gut. 2006, 54: a1-117.Google Scholar
- Deheragoda MG, Church NI, Rodriguez-Justo M, Seward E, Novelli MR, Pereira S, Hatfield ARW, Webster GJM: IgG4 immunostaining in autoimmune pancreatitis and in extra-pancreatic disease [abstract]. Pancreatology. 2006, 6: 193.Google Scholar
- Hamano H, Kawa S, Ochi Y, Unno H, Shiba N, Wajiki M, Nakazawa K, Shimojo H, Kiyosawa K: Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet. 2002, 359: 1403-1404.PubMedGoogle Scholar
- Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, Fukushima M, Nikaido T, Nakayama K, Usuda N, Kiyosawa K: High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001, 344: 732-738.PubMedGoogle Scholar
- Kamisawa T, Funata N, Hayashi Y: Lymphoplasmacytic sclerosing pancreatitis is a pancreatic lesion of IgG4-related systemic disease. Am J Surg Pathol. 2004, 28: 1114.PubMedGoogle Scholar
- Kamisawa T, Nakajima H, Egawa N, Funata N, Tsuruta K, Okamoto A: IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy. Pancreatology. 2006, 6: 132-137.PubMedGoogle Scholar
- Kamisawa T, Funata N, Hayashi Y, Tsuruta K, Okamoto A, Amemiya K, Egawa N, Nakajima H: Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut. 2003, 52: 683-687.PubMedPubMed CentralGoogle Scholar
- Kamisawa T: IgG4-related sclerosing disease. Intern Med. 2006, 45: 125-126.PubMedGoogle Scholar
- Uibu T, Oksa P, Auvinen A, Honkanen E, Metsarinne K, Saha H, Uitti J, Roto P: Asbestos exposure as a risk factor for retroperitoneal fibrosis. Lancet. 2004, 363: 1422-1426.PubMedGoogle Scholar
- Aoki S, Nakazawa T, Ohara H, Sano H, Nakao H, Joh T, Murase T, Eimoto T, Itoh M: Immunohistochemical study of autoimmune pancreatitis using anti-IgG4 antibody and patients' sera. Histopathology. 2005, 47: 147-158.PubMedGoogle Scholar
- Oshiro H, Ebihara Y, Serizawa H, Shimizu T, Teshima S, Kuroda M, Kudo M: Idiopathic retroperitoneal fibrosis associated with immunohematological abnormalities. Am J Med. 2005, 118: 782-786.PubMedGoogle Scholar
- Baker LR, Mallinson WJ, Gregory MC, Menzies EA, Cattell WR, Whitfield HN, Hendry WF, Wickham JE, Joekes AM: Idiopathic retroperitoneal fibrosis. A retrospective analysis of 60 cases. Br J Urol. 1987, 60: 497-503.PubMedGoogle Scholar
- Yaqoob MM, Junaid I: The patient with urinary tract obstruction. Oxford Textbook of Clinical Nephrology. Edited by: Davison AM, Cameron JS, Grunfeld J-P, Ponticelli C, Ritz E, Winearls CG, et al. 2005, Oxford: Oxford University Press, 2465-2470.Google Scholar
- van Bommel EF: Retroperitoneal fibrosis. Neth J Med. 2002, 60: 231-242.PubMedGoogle Scholar
- Vaglio A, Corradi D, Manenti L, Ferretti S, Garini G, Buzio C: Evidence of autoimmunity in chronic periaortitis: a prospective study. Am J Med. 2003, 114: 454-462.PubMedGoogle Scholar
- Modlin IM, Shapiro MD, Kidd M: Carcinoid tumors and fibrosis: an association with no explanation. Am J Gastroenterol. 2004, 99: 2466-2478.PubMedGoogle Scholar
- Chander S, Ergun EL, Chugani HT, Chugani DC, Juhasz C, Shields AF, Weaver DW: High 2-deoxy-2-[18F]fluoro-D-glucose accumulation in a case of retroperitoneal fibrosis following resection of carcinoid tumor. Mol Imaging Biol. 2002, 4: 363-368.PubMedGoogle Scholar
- Graham JR, Suby HI, LeCompte PR, Sadowsky NL: Fibrotic disorders associated with methysergide therapy for headache. N Engl J Med. 1966, 274: 359-368.PubMedGoogle Scholar
- Shaunak S, Wilkins A, Pilling JB, Dick DJ: Pericardial, retroperitoneal, and pleural fibrosis induced by pergolide. J Neurol Neurosurg Psychiatry. 1999, 66: 79-81.PubMedPubMed CentralGoogle Scholar
- Graham JR: Cardiac and pulmonary fibrosis during methysergide therapy for headache. Am J Med Sci. 1967, 254: 1-12.PubMedGoogle Scholar
- Horvath J, Fross RD, Kleiner-Fisman G, Lerch R, Stalder H, Liaudat S, Raskoff WJ, Flachsbart KD, Rakowski H, Pache JC, Burkhard PR, Lang AE: Severe multivalvular heart disease: a new complication of the ergot derivative dopamine agonists. Mov Disord. 2004, 19: 656-662.PubMedGoogle Scholar
- Bleumink GS, Molen-Eijgenraam M, Strijbos JH, Sanwikarja S, van Puijenbroek EP, Stricker BH: Pergolide-induced pleuropulmonary fibrosis. Clin Neuropharmacol. 2002, 25: 290-293.PubMedGoogle Scholar
- Pritchett AM, Morrison JF, Edwards WD, Schaff HV, Connolly HM, Espinosa RE: Valvular heart disease in patients taking pergolide. Mayo Clin Proc. 2002, 77: 1280-1286.PubMedGoogle Scholar
- Maguire GP, Meggs LG, Addonizio J, Del Guercio LR: Association of asbestos exposure, retroperitoneal fibrosis, and acute renal failure. N Y State J Med. 1991, 91: 357-359.PubMedGoogle Scholar
- Boulard JC, Hanslik T, Doleris LM, Prinseau J, Baglin A: Asbestos and idiopathic retroperitoneal fibrosis. Lancet. 1995, 345: 1379.PubMedGoogle Scholar
- Sauni R, Oksa P, Jarvenpaa R, Parker JE, Roto P: Asbestos exposure: a potential cause of retroperitoneal fibrosis. Am J Ind Med. 1998, 33: 418-421.PubMedGoogle Scholar
- Manning CB, Vallyathan V, Mossman BT: Diseases caused by asbestos: mechanisms of injury and disease development. Int Immunopharmacol. 2002, 2: 191-200.PubMedGoogle Scholar
- Nigam SK, Suthar AM, Patel MM, Karnik AB, Dave SK, Kashyap SK, Venkaiah K: Humoral immunological profile of workers exposed to asbestos in asbestos mines. Indian J Med Res. 1993, 98: 274-277.PubMedGoogle Scholar
- Pfau JC, Sentissi JJ, Weller G, Putnam EA: Assessment of autoimmune responses associated with asbestos exposure in Libby, Montana, USA. Environ Health Perspect. 2005, 113: 25-30.PubMedPubMed CentralGoogle Scholar
- Nishi K, Myou S, Ooka T, Fujimura M, Matsuda T: A case of pleural involvement followed by idiopathic retroperitoneal fibrosis. Nihon Kyobu Shikkan Gakkai Zasshi. 1993, 31: 876-880.PubMedGoogle Scholar
- Walker DI, Bloor K, Williams G, Gillie I: Inflammatory aneurysms of the abdominal aorta. Br J Surg. 1972, 59: 609-614.PubMedGoogle Scholar
- Mitchinson MJ: Chronic periaortitis and periarteritis. Histopathology. 1984, 8: 589-600.PubMedGoogle Scholar
- Parums DV, Dunn DC, Dixon AK, Mitchinson MJ: Characterization of inflammatory cells in a patient with chronic periaortitis. Am J Cardiovasc Pathol. 1990, 3: 121-129.PubMedGoogle Scholar
- Vaglio A, Salvarani C, Buzio C: Retroperitoneal fibrosis. Lancet. 2006, 367: 241-251.PubMedGoogle Scholar
- Lindell OI, Sariola HV, Lehtonen TA: The occurrence of vasculitis in perianeurysmal fibrosis. J Urol. 1987, 138: 727-729.PubMedGoogle Scholar
- Parums DV, Brown DL, Mitchinson MJ: Serum antibodies to oxidized low-density lipoprotein and ceroid in chronic periaortitis. Arch Pathol Lab Med. 1990, 114: 383-387.PubMedGoogle Scholar
- Vaglio A, Buzio C: Chronic periaortitis: a spectrum of diseases. Curr Opin Rheumatol. 2005, 17: 34-40.PubMedGoogle Scholar
- Moroni G, Del Papa N, Moronetti LM, Vitali C, Maglione W, Comina DP, Urgnani F, Sandri S, Ponticelli C, Cortelezzi A: Increased levels of circulating endothelial cells in chronic periaortitis as a marker of active disease. Kidney Int. 2005, 68: 562-568.PubMedGoogle Scholar
- Miller OF, Smith LJ, Ferrara EX, McAleer IM, Kaplan GW: Presentation of idiopathic retroperitoneal fibrosis in the pediatric population. J Pediatr Surg. 2003, 38: 1685-1688.PubMedGoogle Scholar
- Doolin EJ, Goldstein H, Kessler B, Vinocur C, Marchildon MB: Familial retroperitoneal fibrosis. J Pediatr Surg. 1987, 22: 1092-1094.PubMedGoogle Scholar
- Comings DE, Skubi KB, Van Eyes J, Motulsky AG: Familial multifocal fibrosclerosis. Findings suggesting that retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, Riedel's thyroiditis, and pseudotumor of the orbit may be different manifestations of a single disease. Ann Intern Med. 1967, 66: 884-892.PubMedGoogle Scholar
- Phills JA, Geggie P, Hidvegi RI, Oliva LA: Retroperitoneal fibrosis in three siblings with the sickle cell trait. Can Med Assoc J. 1973, 108: 1025-1029.PubMedPubMed CentralGoogle Scholar
- Goldbach P, Mohsenifar Z, Salick AI: Familial mediastinal fibrosis associated with seronegative spondylarthropathy. Arthritis Rheum. 1983, 26: 221-225.PubMedGoogle Scholar
- Chalaoui J, Gregoire P, Sylvestre J, Lefebvre R, Amyot R: Pulmonary hyalinizing granuloma: a cause of pulmonary nodules. Radiology. 1984, 152: 23-26.PubMedGoogle Scholar
- Kell HH, Schumann HJ: Retroperitoneal xanthofibrogranuloma simulating kidney tumor. Zentralbl Chir. 1973, 98: 968-971.PubMedGoogle Scholar
- Kohler HP, Laeng RH, Egger C, Streuli R: Systemic fibrosis (generalized form of Ormond's disease). Report of a case which achieved complete remission with cyclophosphamide and corticosteroids. Schweiz Med Wochenschr. 1995, 125: 2131-2136.PubMedGoogle Scholar
- Takashima T, Onoda N, Ishikawa T, Koyama T, Inaba M, Nishizawa Y, Nakatani T, Wakasa K, Hirakawa K: Tumor-forming idiopathic retroperitoneal fibrosis: report of a case. Surg Today. 2004, 34: 374-378.PubMedGoogle Scholar
- Duvic C, Desrame J, Leveque C, Nedelec G: Retroperitoneal fibrosis, sclerosing pancreatitis and bronchiolitis obliterans with organizing pneumonia. Nephrol Dial Transplant. 2004, 19: 2397-2399.PubMedGoogle Scholar
- Hirano K, Kawabe T, Komatsu Y, Matsubara S, Togawa O, Arizumi T, Yamamoto N, Nakai Y, Sasahira N, Tsujino T, Toda N, Isayama H, Tada M, Omata M: High-rate pulmonary involvement in autoimmune pancreatitis. Intern Med J. 2006, 36: 58-61.PubMedGoogle Scholar
- King TE: BOOP: an important cause of migratory pulmonary infiltrates?. Eur Respir J. 1995, 8: 193-195.PubMedGoogle Scholar
- Shimizu T, Furuichi S, Takahashi N, Koya Y, Akashiba T, Horie T: [A case of intrathoracic extension of retroperitoneal fibrosis]. Nihon Kokyuki Gakkai Zasshi. 2001, 39: 748-752.PubMedGoogle Scholar
- Ammar A, El Hammami S, Horchani H, Sellami N, Kilani T: Calcifying fibrous pseudotumor of the pleura: a rare location. Ann Thorac Surg. 2003, 76: 2081-2082.PubMedGoogle Scholar
- Cavazza A, Gelli MC, Agostini L, Sgarbi G, De Marco L, Gardini G: Calcified pseudotumor of the pleura: description of a case. Pathologica. 2002, 94: 201-205.PubMedGoogle Scholar
- Kocova L, Michal M, Sulc M, Zamecnik M: Calcifying fibrous pseudotumour of visceral peritoneum. Histopathology. 1997, 31: 182-184.PubMedGoogle Scholar
- Petersdorf RG, Beeson PB: Fever of unexplained origin: report on 100 cases. Medicine (Baltimore). 1961, 40: 1-30.Google Scholar
- de Kleijn EM, Vandenbroucke JP, van der Meer JW: Fever of unknown origin (FUO). I A. prospective multicenter study of 167 patients with FUO, using fixed epidemiologic entry criteria. The Netherlands FUO Study Group. Medicine (Baltimore). 1997, 76: 392-400.Google Scholar
- Knockaert DC, Vanneste LJ, Bobbaers HJ: Fever of unknown origin in elderly patients. J Am Geriatr Soc. 1993, 41: 1187-1192.PubMedGoogle Scholar
- Mourad O, Palda V, Detsky AS: A comprehensive evidence-based approach to fever of unknown origin. Arch Intern Med. 2003, 163: 545-551.PubMedGoogle Scholar
- Zhiyong Z, Bingjun L, Xiaoju L, Xinjian F, Ping F, Wenya W: Fever of unknown origin: a report from China of 208 cases. Int J Clin Pract. 2003, 57: 592-596.PubMedGoogle Scholar
- Bleeker-Rovers CP, de Kleijn EM, Corstens FH, van der Meer JW, Oyen WJ: Clinical value of FDG PET in patients with fever of unknown origin and patients suspected of focal infection or inflammation. Eur J Nucl Med Mol Imaging. 2004, 31: 29-37.PubMedGoogle Scholar
- Van der Zee JS, van Swieten P, Aalberse RC: Inhibition of complement activation by IgG4 antibodies. Clin Exp Immunol. 1986, 64: 415-422.PubMedPubMed CentralGoogle Scholar
- Punnonen J, Aversa G, Cocks BG, McKenzie AN, Menon S, Zurawski G, de Waal MR, de Vries JE: Interleukin 13 induces interleukin 4-independent IgG4 and IgE synthesis and CD23 expression by human B cells. Proc Natl Acad Sci U S A. 1993, 90: 3730-3734.PubMedPubMed CentralGoogle Scholar
- Aalberse RC, Van Milligen F, Tan KY, Stapel SO: Allergen-specific IgG4 in atopic disease. Allergy. 1993, 48: 559-569.PubMedGoogle Scholar
- Hussain R, Poindexter RW, Ottesen EA: Control of allergic reactivity in human filariasis. Predominant localization of blocking antibody to the IgG4 subclass. J Immunol. 1992, 148: 2731-2737.PubMedGoogle Scholar
- Jones CC, Hamilton RG, Jordon RE: Subclass distribution of human IgG autoantibodies in pemphigus. J Clin Immunol. 1988, 8: 43-49.PubMedGoogle Scholar
- Futei Y, Amagai M, Ishii K, Kuroda-Kinoshita K, Ohya K, Nishikawa T: Predominant IgG4 subclass in autoantibodies of pemphigus vulgaris and foliaceus. J Dermatol Sci. 2001, 26: 55-61.PubMedGoogle Scholar
- Ebner C, Siemann U, Bohle B, Willheim M, Wiedermann U, Schenk S, Klotz F, Ebner H, Kraft D, Scheiner O: Immunological changes during specific immunotherapy of grass pollen allergy: reduced lymphoproliferative responses to allergen and shift from TH2 to TH1 in T-cell clones specific for Phl p 1, a major grass pollen allergen. Clin Exp Allergy. 1997, 27: 1007-1015.PubMedGoogle Scholar
- Platts-Mills T, Vaughan J, Squillace S, Woodfolk J, Sporik R: Sensitisation, asthma, and a modified Th2 response in children exposed to cat allergen: a population-based cross-sectional study. Lancet. 2001, 357: 752-756.PubMedGoogle Scholar
- Akdis CA, Blesken T, Akdis M, Wuthrich B, Blaser K: Role of interleukin 10 in specific immunotherapy. J Clin Invest. 1998, 102: 98-106.PubMedPubMed CentralGoogle Scholar
- Jeannin P, Lecoanet S, Delneste Y, Gauchat JF, Bonnefoy JY: IgE versus IgG4 production can be differentially regulated by IL-10. J Immunol. 1998, 160: 3555-3561.PubMedGoogle Scholar
- Robinson DS, Larche M, Durham SR: Tregs and allergic disease. J Clin Invest. 2004, 114: 1389-1397.PubMedPubMed CentralGoogle Scholar
- Ramalingam TR, Reiman RM, Wynn TA: Exploiting worm and allergy models to understand Th2 cytokine biology. Curr Opin Allergy Clin Immunol. 2005, 5: 392-398.PubMedGoogle Scholar
- Waschke J, Bruggeman P, Baumgartner W, Zillikens D, Drenckhahn D: Pemphigus foliaceus IgG causes dissociation of desmoglein 1-containing junctions without blocking desmoglein 1 transinteraction. J Clin Invest. 2005, 115: 3157-3165.PubMedPubMed CentralGoogle Scholar
- Dopp R, Schmidt E, Chimanovitch I, Leverkus M, Brocker EB, Zillikens D: IgG4 and IgE are the major immunoglobulins targeting the NC16A domain of BP180 in bullous pemphigoid: serum levels of these immunoglobulins reflect disease activity. J Am Acad Dermatol. 2000, 42: 577-583.PubMedGoogle Scholar
- Okazaki K, Uchida K, Ohana M, Nakase H, Uose S, Inai M, Matsushima Y, Katamura K, Ohmori K, Chiba T: Autoimmune-related pancreatitis is associated with autoantibodies and a Th1/Th2-type cellular immune response. Gastroenterology. 2000, 118: 573-581.PubMedGoogle Scholar
- Wakabayashi T, Kawaura Y, Satomura Y, Urabe T, Watanabe H, Motoo Y, Sawabu N: Duct-narrowing chronic pancreatitis without immunoserologic abnormality: comparison with duct-narrowing chronic pancreatitis with positive serological evidence and its clinical management. Dig Dis Sci. 2005, 50: 1414-1421.PubMedGoogle Scholar
- Tron F, Gilbert D, Mouquet H, Joly P, Drouot L, Makni S, Masmoudi H, Charron D, Zitouni M, Loiseau P, Ben Ayed M: Genetic factors in pemphigus. J Autoimmun. 2005, 24: 319-328.PubMedGoogle Scholar
- Kawa S, Ota M, Yoshizawa K, Horiuchi A, Hamano H, Ochi Y, Nakayama K, Tokutake Y, Katsuyama Y, Saito S, Hasebe O, Kiyosawa K: HLA DRB10405-DQB10401 haplotype is associated with autoimmune pancreatitis in the Japanese population. Gastroenterology. 2002, 122: 1264-1269.PubMedGoogle Scholar
- Dang A, Wang B, Zhang Y, Zhang P, Huang J, Liu G, Zheng D, Qiu C, Liu L: Association of the HLA-DRB1 gene with susceptibility to aortoarteritis in a Chinese Han population. Hypertens Res. 2002, 25: 631-634.PubMedGoogle Scholar
- Hirayama K: Immunogenetic analysis of post-schistosomal liver fibrosis. Parasitol Int. 2004, 53: 193-196.PubMedGoogle Scholar
- Voss SD, Kruskal JB, Kane RA: Chronic inflammatory pseudotumor arising in the hepatobiliary-pancreatic system: progressive multisystemic organ involvement in four patients. AJR Am J Roentgenol. 1999, 173: 1049-1054.PubMedGoogle Scholar
- van der Vliet HJ, Perenboom RM: Multiple pseudotumors in IgG4-associated multifocal systemic fibrosis. Ann Intern Med. 2004, 141: 896-897.PubMedGoogle Scholar
- Clark A, Zeman RK, Choyke PL, White EM, Burrell MI, Grant EG, Jaffe MH: Pancreatic pseudotumors associated with multifocal idiopathic fibrosclerosis. Gastrointest Radiol. 1988, 13: 30-32.PubMedGoogle Scholar
- Goto Y, Ohaki Y, Ibaraki N: A clinicopathologic case report of inflammatory pseudotumors involving the conjunctiva and lung. Jpn J Ophthalmol. 2004, 48: 573-577.PubMedGoogle Scholar
- Hosler GA, Steinberg DM, Sheth S, Hamper UM, Erozan YS, Ali SZ: Inflammatory pseudotumor: a diagnostic dilemma in cytopathology. Diagn Cytopathol. 2004, 31: 267-270.PubMedGoogle Scholar
- Alam M, Morehead RS, Weinstein MH: Dermatomyositis as a presentation of pulmonary inflammatory pseudotumor (myofibroblastic tumor). Chest. 2000, 117: 1793-1795.PubMedGoogle Scholar
- Tuncozgur B, Ustunsoy H, Bakir K, Ucak R, Elbeyli L: Inflammatory pseudotumor of the lung. Thorac Cardiovasc Surg. 2000, 48: 112-113.PubMedGoogle Scholar
- Uetsuji S, Nakagawa A, Kwon AH, Komada H, Imamura A, Kamiyama Y: Inflammatory pseudotumor of the liver: report of a case and review of the literature. Surg Today. 1996, 26: 517-521.PubMedGoogle Scholar
- Sato Y, Harada K, Nakanuma Y: Hepatic inflammatory pseudotumour related to autoimmune pancreatitis. Histopathology. 2004, 45: 418-419.PubMedGoogle Scholar
- Zen Y, Harada K, Sasaki M, Sato Y, Tsuneyama K, Haratake J, Kurumaya H, Katayanagi K, Masuda S, Niwa H, Morimoto H, Miwa A, Uchiyama A, Portmann BC, Nakanuma Y: IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis?. Am J Surg Pathol. 2004, 28: 1193-1203.PubMedGoogle Scholar
- Spivach A, Turoldo A, Pistan V, Colautti I, Zanconati F: Inflammatory pseudotumour of the liver: case report and review of the literature. Chir Ital. 2005, 57: 229-237.PubMedGoogle Scholar
- Zen Y, Kasahara Y, Horita K, Miyayama S, Miura S, Kitagawa S, Nakanuma Y: Inflammatory pseudotumor of the breast in a patient with a high serum IgG4 level: histologic similarity to sclerosing pancreatitis. Am J Surg Pathol. 2005, 29: 275-278.PubMedGoogle Scholar
- Toosi MN, Heathcote J: Pancreatic pseudotumor with sclerosing pancreato-cholangitis: is this a systemic disease?. Am J Gastroenterol. 2004, 99: 377-382.PubMedGoogle Scholar
- Esposito I, Bergmann F, Penzel R, di Mola FF, Shrikhande S, Buchler MW, Friess H, Otto HF: Oligoclonal T-cell populations in an inflammatory pseudotumor of the pancreas possibly related to autoimmune pancreatitis: an immunohistochemical and molecular analysis. Virchows Arch. 2004, 444: 119-126.PubMedGoogle Scholar
- Chutaputti A, Burrell MI, Boyer JL: Pseudotumor of the pancreas associated with retroperitoneal fibrosis: a dramatic response to corticosteroid therapy. Am J Gastroenterol. 1995, 90: 1155-1158.PubMedGoogle Scholar
- Zhao MF, Tian Y, Guo KJ, Ma ZG, Liao HH: Common bile duct obstruction due to fibrous pseudotumor of pancreas associated with retroperitoneal fibrosis: a case report. World J Gastroenterol. 2004, 10: 3078-3079.PubMedPubMed CentralGoogle Scholar
- Wiesner W, Kocher T, Beglinger C, Harder F, Steinbrich W: Pseudotumor of the pancreatic head associated with idiopathic retroperitoneal fibrosis. Dig Surg. 2001, 18: 418-421.PubMedGoogle Scholar
- Van Hoe L, Oyen R, Gryspeerdt S, Baert AL, Bobbaers H, Baert L: Case report: pseudotumoral pelvic retroperitoneal fibrosis associated with orbital fibrosis. Br J Radiol. 1995, 68: 421-423.PubMedGoogle Scholar
- Kondo T, Onitsuka S, Kobayashi H, Ryoji O, Toma H: A case of retroperitoneal pseudotumor (xanthogranuloma). Hinyokika Kiyo. 1998, 44: 21-24.PubMedGoogle Scholar
- Ruiz-Cabello JM, Martinez Salmeron JF, Perez MJ, Salinas C, Rodrigo MM: Inflammatory mesenteric pseudotumor. Rev Esp Enferm Apar Dig. 1988, 73: 311-314.Google Scholar
- Sakurai H, Hasegawa T, Watanabe S, Suzuki K, Asamura H, Tsuchiya R: Inflammatory myofibroblastic tumor of the lung. Eur J Cardiothorac Surg. 2004, 25: 155-159.PubMedGoogle Scholar
- Berman M, Georghiou GP, Schonfeld T, Feinmesser M, Horev G, Vidne BA, Saute M: Pulmonary inflammatory myofibroblastic tumor invading the left atrium. Ann Thorac Surg. 2003, 76: 601-603.PubMedGoogle Scholar
- Jeon YK, Chang KH, Suh YL, Jung HW, Park SH: Inflammatory myofibroblastic tumor of the central nervous system: clinicopathologic analysis of 10 cases. J Neuropathol Exp Neurol. 2005, 64: 254-259.PubMedGoogle Scholar
- Ilvan S, Celik V, Paksoy M, Cetinaslan I, Calay Z: Inflammatory myofibroblastic tumor (inflammatory pseudotumor) of the breast. APMIS. 2005, 113: 66-69.PubMedGoogle Scholar
- Greiner C, Rickert CH, Mollmann FT, Rieger B, Semik M, Heindel W, Wassmann H: Plasma cell granuloma involving the brain and the lung. Acta Neurochir (Wien). 2003, 145: 1127-1131.Google Scholar
- Urschel JD, Horan TA, Unruh HW: Plasma cell granuloma of the lung. J Thorac Cardiovasc Surg. 1992, 104: 870-875.PubMedGoogle Scholar
- Stewart TW, Friberg TR: Idiopathic retroperitoneal fibrosis with diffuse involvement: further evidence of systemic idiopathic fibrosis. South Med J. 1984, 77: 1185-1187.PubMedGoogle Scholar
- Mitchinson MJ: Systemic idiopathic fibrosis and systemic Weber-Christian disease. J Clin Pathol. 1965, 18: 645-649.PubMedPubMed CentralGoogle Scholar
- Morad N, Strongwater SL, Eypper S, Woda BA: Idiopathic retroperitoneal and mediastinal fibrosis mimicking connective tissue disease. Am J Med. 1987, 82: 363-366.PubMedGoogle Scholar
- Binder SC, Deterling RA, Mahoney SA, Patterson JF, Wolfe HJ: Systemic idiopathic fibrosis. Report of a case of the concomitant occurrence of retractile mesenteritis and retroperitoneal fibrosis. Am J Surg. 1972, 124: 422-430.PubMedGoogle Scholar
- Hershkowitz M, Fogari R, Chandra M: Idiopathic retroperitoneal fibrosis: implications for a systemic disorder. Clin Exp Rheumatol. 1983, 1: 157-160.PubMedGoogle Scholar
- Nakamura Y, Kohzaki S, Suyama N, Yamaguchi T, Kohno S, Hayashi K: Systemic idiopathic fibrosis with inflammatory pulmonary lesions. Br J Radiol. 1997, 70: 956-958.PubMedGoogle Scholar
- Kuramochi S, Kawai T, Yakumaru K, Mikata A, Torikata C, Kasuga Y, Fujiwara T: Multiple pulmonary hyalinizing granulomas associated with systemic idiopathic fibrosis. Acta Pathol Jpn. 1991, 41: 375-382.PubMedGoogle Scholar
- Kastendieck H: Xanthofibrogranulomatosis – a tumor-like lesion of the intestinal adipose tissue with a tendency towards generalization. Verh Dtsch Ges Pathol. 1978, 62 (501): 501.PubMedGoogle Scholar
- Miyachi S, Kobayashi T, Takahashi T, Saito K, Hashizume Y, Sugita K: An intracranial mass lesion in systemic xanthogranulomatosis: case report. Neurosurgery. 1990, 27: 822-826.PubMedGoogle Scholar
- Grunow N, Goertchen R: Generalized retroperitoneal xanthofibrogranulomatosis with brain involvement. Pathologe. 1988, 9: 305-308.PubMedGoogle Scholar
- Verswijvel G, Stragier J, Verhelle J, Roskams T, Oyen RH: Bilateral xanthogranulomatous perinephritis without renal parenchymal involvement. Eur Radiol. 2000, 10: 900-902.PubMedGoogle Scholar
- Arvis G, de Boisgisson P, Schrameck E: Retroperitoneal xanthogranuloma. Apropos of 4 cases. J Urol Nephrol (Paris). 1975, 81: 550-557.Google Scholar
- Forster R, Ruschoff J, Kleinsorge F: Retroperitoneal xanthofibrogranulomatosis. Rofo. 1989, 151: 688-691.PubMedGoogle Scholar
- Dauch WA, Rossberg C, Luttges J: Xanthofibrogranulomatosis with meningocortical involvement. Zentralbl Allg Pathol. 1986, 132: 33-36.PubMedGoogle Scholar
- Malhotra R, Porter RG, Selva D: Adult orbital xanthogranuloma with periosteal infiltration. Br J Ophthalmol. 2003, 87: 120-121.PubMedPubMed CentralGoogle Scholar
- van der PR, Nieuwenhuis MG, Mourits MP: Multifocal fibrosclerosis presenting as Grave's orbitopathy. Bilateral exophthalmos associated with retroperitoneal and sellar fibrosis. Graefes Arch Clin Exp Ophthalmol. 1999, 237: 256-258.Google Scholar
- Schnitzler L, Verret JL, Caron C, Tuchais E, Soret JY, Salaun Y: [Multifocal fibrosclerosis (retroperitoneal, mediastinal, mesenteric and pelvic involvement) associated with vasculitis and revealed by a livedo: clinical, histopathological and ultrastructural study in a case] (author's translation). Ann Dermatol Venereol. 1978, 105: 943-956.PubMedGoogle Scholar
- Brazier DJ, Sanders MD: Multifocal fibrosclerosis associated with suprasellar and macular lesions. Br J Ophthalmol. 1983, 67: 292-296.PubMedPubMed CentralGoogle Scholar
- Tutuncu NB, Erbas T, Bayraktar M, Gedik O: Multifocal idiopathic fibrosclerosis manifesting with Riedel's thyroiditis. Endocr Pract. 2000, 6: 447-449.PubMedGoogle Scholar
- Laitt RD, Hubscher SG, Buckels JA, Darby S, Elias E: Sclerosing cholangitis associated with multifocal fibrosis: a case report. Gut. 1992, 33: 1430-1432.PubMedPubMed CentralGoogle Scholar
- Manassero A, Cracco C, Terrone C, Rossetti SR: Coexistence of orbital and retroperitoneal involvement in multifocal fibrosclerosis: case report. Arch Ital Urol Androl. 1998, 70: 11-14.PubMedGoogle Scholar
- Richards AB, Shalka HW, Roberts FJ, Flint A: Pseudotumor of the orbit and retroperitoneal fibrosis. A form of multifocal fibrosclerosis. Arch Ophthalmol. 1980, 98: 1617-1620.PubMedGoogle Scholar
- Levine MR, Kaye L, Mair S, Bates J: Multifocal fibrosclerosis. Report of a case of bilateral idiopathic sclerosing pseudotumor and retroperitoneal fibrosis. Arch Ophthalmol. 1993, 111: 841-843.PubMedGoogle Scholar
- Meijer S, Hoitsma HF, Scholtmeijer R: Idiopathic retroperitoneal fibrosis in multifocal fibrosclerosis. Eur Urol. 1976, 2: 258-260.PubMedGoogle Scholar
- Nielson HK: Multifocal idiopathic fibrosclerosis. Two cases with simultaneous occurrence of retroperitoneal fibrosis and Riedel's thyroiditis. Acta Med Scand. 1980, 208: 119-123.PubMedGoogle Scholar
- Aylward GW, Sullivan TJ, Garner A, Moseley I, Wright JE: Orbital involvement in multifocal fibrosclerosis. Br J Ophthalmol. 1995, 79: 246-249.PubMedPubMed CentralGoogle Scholar
- Bonnet C, Arnaud M, Bertin P, Treves R, Gotteron RD: Idiopathic retroperitoneal fibrosis with systemic manifestations. J Rheumatol. 1994, 21: 360-362.PubMedGoogle Scholar
- Hashimoto S, Fujii W, Takahashi T, Shiroshita K, Sakurai T, Ueda T, Kawata T: Pulmonary hyalinizing granuloma with hydronephrosis. Intern Med. 2002, 41: 463-466.PubMedGoogle Scholar
- Dent RG, Godden DJ, Stovin PG, Stark JE: Pulmonary hyalinising granuloma in association with retroperitoneal fibrosis. Thorax. 1983, 38: 955-956.PubMedPubMed CentralGoogle Scholar
- Dejaco C, Ferenci P, Schober E, Kaserer K, Fugger R, Novacek G, Gangl A: Stenosis of the common bile duct due to Ormond's disease: case report and review of the literature. J Hepatol. 1999, 31: 156-159.PubMedGoogle Scholar
- Kamisawa T, Matsukawa M, Ohkawa M: Autoimmune pancreatitis associated with retroperitoneal fibrosis. JOP. 2005, 6: 260-263.PubMedGoogle Scholar
- Pereira-Lima JC, Kromer MU, Adamek HE, Riemann JF: Cholestatic jaundice due to Ormond's disease (primary retroperitoneal fibrosis). Hepatogastroenterology. 1996, 43: 992-994.PubMedGoogle Scholar
- Cappell MS: Obstructive jaundice due to retroperitoneal fibrosis involving the head of the pancreas. J Clin Gastroenterol. 1994, 18: 53-56.PubMedGoogle Scholar
- Fruh D, Jaeger W, Kafer O: Orbital involvement in retroperitoneal fibrosis (morbus Ormond). Mod Probl Ophthalmol. 1975, 14 (651–6): 651-656.PubMedGoogle Scholar
- Lenhard V, Bommer J, Andrassy K, Krampien B, Orth H, Ritz E: Retro-orbital fibrosis in Ormond's disease (author's transl). Dtsch Med Wochenschr. 1974, 99: 2289-2286.Google Scholar
- Schonder AA, Clift RC, Brophy JW, Dane LW: Bilateral recurrent orbital inflammation associated with retroperitoneal fibrosclerosis. Br J Ophthalmol. 1985, 69: 783-787.PubMedPubMed CentralGoogle Scholar
- Reittner P, Riepl T, Goritschnig T, Preidler KW, Koele W, Szolar DH: Bilateral orbital pseudotumour due to Ormond's disease: MR imaging and CT findings. Neuroradiology. 2002, 44: 272-274.PubMedGoogle Scholar
- Kruit WH, den Ottolander GJ: Riedel's thyroiditis in a patient with retroperitoneal fibrosis. Neth J Med. 1991, 39: 17-19.PubMedGoogle Scholar
- Miro I, Bakir R, Chanu B, Brocheriou C, Brenot F, Rouffy J: Riedel's thyroiditis and retroperitoneal fibrosis. Apropos of a case of multiple fibrosing disease. Ann Med Interne (Paris). 1984, 135: 212-216.Google Scholar
- Rao CR, Ferguson GC, Kyle VN: Retroperitoneal fibrosis associated with Riedel's struma. Can Med Assoc J. 1973, 108: 1019-1021.PubMedPubMed CentralGoogle Scholar
- Meyer S, Hausman R: Occlusive phlebitis in multifocal fibrosclerosis. Am J Clin Pathol. 1976, 65: 274-283.PubMedGoogle Scholar
- Wich M, Steegmuller KW, Junginger T, Teifke A: Riedel's thyroiditis and idiopathic multifocal fibrosclerosis. A case report. Chirurg. 1991, 62: 211-213.PubMedGoogle Scholar
- Kitagawa S, Zen Y, Harada K, Sasaki M, Sato Y, Minato H, Watanabe K, Kurumaya H, Katayanagi K, Masuda S, Niwa H, Tsuneyama K, Saito K, Haratake J, Takagawa K, Nakanuma Y: Abundant IgG4-Positive plasma cell infiltration characterizes chronic sclerosing sialadenitis (Kuttner's Tumor). Am J Surg Pathol. 2005, 29: 783-791.PubMedGoogle Scholar
- Leger L, Roberti A, Deloche dN, Lemaigre G: Mesenteric panniculitis, a variety of retroperitoneal fibrosclerosis. Presse Med. 1967, 75: 75-79.PubMedGoogle Scholar
- Godquin B, Saout R, Loupias P, Garipuy A, Blanchard J: Inflammatory and necrotic pseudotumor of the mesentery. Classification of mesenteric panniculitis. J Chir (Paris). 1972, 104: 167-175.Google Scholar
- Paraf A, Menager C, Texier J: Mesenteric panniculitis, retroperitoneal lipodistrophy and pancreatic diseases. Apropos of a case. Presse Med. 1968, 76: 2263-2265.PubMedGoogle Scholar
- Decker GA, Gaylis H: Mesenteric panniculitis simulating an abdominal aortic aneurysm. S Afr Med J. 1972, 46: 750.PubMedGoogle Scholar
- Herr W, Lohse AW, Spahn TW, Dienes HP, Trautmann F, Meyer zum Buschenfelde KH, Marker-Hermann E: Nodular nonsuppurative panniculitis in association with primary biliary cirrhosis and Hashimoto's thyroiditis. Z Rheumatol. 1996, 55: 122-126.PubMedGoogle Scholar
- Proenca NG, Frucchi H: Weber-Christian febrile recurrent nodular nonsuppurative panniculitis and panniculitis caused by pancreopathy: differential diagnosis and enzymatic mechanisms. Rev Paul Med. 1978, 91: 82-83.PubMedGoogle Scholar
- Motoo Y, Minamoto T, Watanabe H, Sakai J, Okai T, Sawabu N: Sclerosing pancreatitis showing rapidly progressive changes with recurrent mass formation. Int J Pancreatol. 1997, 21: 85-90.PubMedGoogle Scholar
- Sarles H, Sarles J, Muratore R, Guien C: Chronic inflammatory sclerosis of the pancreas – an autonomous pancreatic disease?. Am J Dig Dis. 1961, 6: 688-698.PubMedGoogle Scholar
- Kawaguchi K, Koike M, Tsuruta K, Okamoto A, Tabata I, Fujita N: Lymphoplasmacytic sclerosing pancreatitis with cholangitis: a variant of primary sclerosing cholangitis extensively involving pancreas. Hum Pathol. 1991, 22: 387-395.PubMedGoogle Scholar
- Notohara K, Burgart LJ, Yadav D, Chari S, Smyrk TC: Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases. Am J Surg Pathol. 2003, 27: 1119-1127.PubMedGoogle Scholar
- Ito T, Nakano I, Koyanagi S, Miyahara T, Migita Y, Ogoshi K, Sakai H, Matsunaga S, Yasuda O, Sumii T, Nawata H: Autoimmune pancreatitis as a new clinical entity. Three cases of autoimmune pancreatitis with effective steroid therapy. Dig Dis Sci. 1997, 42: 1458-1468.PubMedGoogle Scholar
- Erkelens GW, Vleggaar FP, Lesterhuis W, van Buuren HR, van der Werf SD: Sclerosing pancreato-cholangitis responsive to steroid therapy. Lancet. 1999, 354: 43-44.PubMedGoogle Scholar
- Fukui T, Okazaki K, Yoshizawa H, Ohashi S, Tamaki H, Kawasaki K, Matsuura M, Asada M, Nakase H, Nakashima Y, Nishio A, Chiba T: A case of autoimmune pancreatitis associated with sclerosing cholangitis, retroperitoneal fibrosis and Sjogren's syndrome. Pancreatology. 2005, 5: 86-91.PubMedGoogle Scholar
- Fukukura Y, Fujiyoshi F, Nakamura F, Hamada H, Nakajo M: Autoimmune pancreatitis associated with idiopathic retroperitoneal fibrosis. AJR Am J Roentgenol. 2003, 181: 993-995.PubMedGoogle Scholar
- Ichimura T, Kondo S, Ambo Y, Hirano S, Ohmi M, Okushiba S, Morikawa T, Shimizu M, Katoh H: Primary sclerosing cholangitis associated with autoimmune pancreatitis. Hepatogastroenterology. 2002, 49: 1221-1224.PubMedGoogle Scholar
- Nishino T, Toki F, Oyama H, Oi I, Kobayashi M, Takasaki K, Shiratori K: Biliary tract involvement in autoimmune pancreatitis. Pancreas. 2005, 30: 76-82.PubMedGoogle Scholar
- Kamisawa T: IgG4-positive plasma cells specifically infiltrate various organs in autoimmune pancreatitis. Pancreas. 2004, 29: 167-168.PubMedGoogle Scholar
- Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K, Okamoto A, Egawa N, Nakajima H: A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003, 38: 982-984.PubMedGoogle Scholar
- Uchida K, Okazaki K, Konishi Y, Ohana M, Takakuwa H, Hajiro K, Chiba T: Clinical analysis of autoimmune-related pancreatitis. Am J Gastroenterol. 2000, 95: 2788-2794.PubMedGoogle Scholar
- Pickartz T, Pickartz H, Lochs H, Ockenga J: Overlap syndrome of autoimmune pancreatitis and cholangitis associated with secondary Sjogren's syndrome. Eur J Gastroenterol Hepatol. 2004, 16: 1295-1299.PubMedGoogle Scholar
- Shinji A, Sano K, Hamano H, Unno H, Fukushima M, Nakamura N, Akamatsu T, Kawa S, Kiyosawa K: Autoimmune pancreatitis is closely associated with gastric ulcer presenting with abundant IgG4-bearing plasma cell infiltration. Gastrointest Endosc. 2004, 59: 506-511.PubMedGoogle Scholar
- Taniguchi T, Ko M, Seko S, Nishida O, Inoue F, Kobayashi H, Saiga T, Okamoto M, Fukuse T: Interstitial pneumonia associated with autoimmune pancreatitis. Gut. 2004, 53: 770-771.PubMedPubMed CentralGoogle Scholar
- Arai K, Kawamura O, Naruse I, Tsunekawa K, Hayashi A, Yonezu M, Oya N, Takagi H, Mori M, Kon Y: A case of chronic pancreatitis with diffuse irregular narrowing of the pancreatic duct complicated by Sjogren's syndrome and interstitial pneumonia. Nippon Shokakibyo Gakkai Zasshi. 2001, 98: 847-852.PubMedGoogle Scholar
- Phillips RH, Carr RA, Preston R, Pereira SP, Wilkinson ML, O'Donnell PJ, Thompson RP: Sclerosing mesenteritis involving the pancreas: two cases of a rare cause of abdominal mass mimicking malignancy. Eur J Gastroenterol Hepatol. 1999, 11: 1323-1329.PubMedGoogle Scholar
- Harada M, Hashimoto O, Kumemura H, Taniguchi E, Shiratsuchi M, Harada R, Sakamoto M, Yoshida H, Fukuda T, Sakisaka S, Sata M: Bronchiolitis obliterans organizing pneumonia in a patient with primary biliary cirrhosis and rheumatoid arthritis treated with prednisolone. Hepatol Res. 2002, 23: 301.PubMedGoogle Scholar
- Takahashi K, Sakamoto N, Okada M, Tamechika Y, Ishibashi H: [Interstitial pneumonia observed during acute exacerbation of autoimmune hepatitis]. Nippon Shokakibyo Gakkai Zasshi. 2000, 97: 719-722.PubMedGoogle Scholar
- Yousem SA, Hochholzer L: Pulmonary hyalinizing granuloma. Am J Clin Pathol. 1987, 87: 1-6.PubMedGoogle Scholar
- Gossot D, Fromont G, Galetta D, Debrosse D, Grunenwald D: Pulmonary hyalinising granuloma with mediastinal fibrosis: a rare cause of dysphagia. Ann Chir. 2003, 128: 622-625.PubMedGoogle Scholar
- Burrig KF, Kastendieck H: Ultrastructural observations on the histogenesis of localized fibrous tumours of the pleura (benign mesothelioma). Virchows Arch A Pathol Anat Histopathol. 1984, 403: 413-424.PubMedGoogle Scholar
- Burrig KF, Kastendieck H, Husselmann H: Localized fibrous pleural tumor (benign mesothelioma). Clinico-pathological study of 24 cases for classification, morphogenesis and prognosis. Pathologe. 1983, 4: 120-129.PubMedGoogle Scholar
- Lee HY, Chuah KL, Tan PH: Test and teach. Number fifty-three. Diagnosis: Calcifying fibrous pseudotumour. Pathology. 2003, 35: 166-169.PubMedGoogle Scholar
- Dargent JL, Delplace J, Roufosse C, Laget JP, Lespagnard L: Development of a calcifying fibrous pseudotumour within a lesion of Castleman disease, hyaline-vascular subtype. J Clin Pathol. 1999, 52: 547-549.PubMedPubMed CentralGoogle Scholar
- Zamecnik M, Boudova L, Sulc M, Michal M: Location of calcifying fibrous pseudotumour: peritoneal predominance. Histopathology. 2005, 46: 346.PubMedGoogle Scholar
- Weynand B, Draguet AP, Bernard P, Marbaix E, Galant C: Calcifying fibrous pseudotumour: first case report in the peritoneum with immunostaining for CD34. Histopathology. 1999, 34: 86-87.PubMedGoogle Scholar
- Peachell M, Mayo J, Kalloger S, Flint J, English J: Calcifying fibrous pseudotumour of the lung. Thorax. 2003, 58: 1018-1019.PubMedPubMed CentralGoogle Scholar
- Tsuneyama K, Saito K, Ruebner BH, Konishi I, Nakanuma Y, Gershwin ME: Immunological similarities between primary sclerosing cholangitis and chronic sclerosing sialadenitis: report of the overlapping of these two autoimmune diseases. Dig Dis Sci. 2000, 45: 366-372.PubMedGoogle Scholar
- Sjogren I, Wengle B, Korsgren M: Primary sclerosing cholangitis associated with fibrosis of the submandibular glands and the pancreas. Acta Med Scand. 1979, 205: 139-141.PubMedGoogle Scholar
- Monteil RA, Saint-Paul MC, Hofman P, Jehl-Pietri C, Michiels JF, Raspaldo H: Oral inflammatory pseudotumour: immunohistochemical investigation of a case involving the submandibular gland and review of the literature. Oral Oncol. 1997, 33: 215-219.PubMedGoogle Scholar
- Zamboni G, Luttges J, Capelli P, Frulloni L, Cavallini G, Pederzoli P, Leins A, Longnecker D, Kloppel G: Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens. Virchows Arch. 2004, 445: 552-563.PubMedGoogle Scholar
- Imasaki T, Yoshii A, Tanaka S, Ogura T, Ishikawa A, Takahashi T: Polymyositis and Sjogren's syndrome associated with bronchiolitis obliterans organizing pneumonia. Intern Med. 1996, 35: 231-235.PubMedGoogle Scholar
- Doi M, Uji Y: A case of uveitis associated with idiopathic retroperitoneal fibrosis. Am J Ophthalmol. 1994, 117: 358-362.PubMedGoogle Scholar
- Armigliato M, Paolini R, Bianchini E, Monesi G, Zamboni S, D'Andrea E: Hashimoto's thyroiditis and Graves' disease associated with retroperitoneal fibrosis. Thyroid. 2002, 12: 829-831.PubMedGoogle Scholar
- Hanley PC, Shub C, Lie JT: Constrictive pericarditis associated with combined idiopathic retroperitoneal and mediastinal fibrosis. Mayo Clin Proc. 1984, 59: 300-304.PubMedGoogle Scholar
- van Bommel EF, Bouvy ND, Liem E, Boeve ER, Weimar W: Coexistent idiopathic retroperitoneal and mediastinal fibrosis presenting with portal hypertension. Neth J Med. 1994, 44: 174-177.PubMedGoogle Scholar
- Morgan AD, Loughridge LW, Calne RY: Combined mediastinal and retroperitoneal fibrosis. Lancet. 1966, 1: 67-70.PubMedGoogle Scholar
- Mitchinson MJ, Wight DG, Arno J, Milstein BB: Chronic coronary periarteritis in two patients with chronic periaortitis. J Clin Pathol. 1984, 37: 32-36.PubMedPubMed CentralGoogle Scholar
- Keith DS, Larson TS: Idiopathic retroperitoneal fibrosis. J Am Soc Nephrol. 1993, 3: 1748-1752.PubMedGoogle Scholar
- Debrand-Passard A, Wilhelm H: Ormond's disease or aortic aneurysm? Case reports. Int Urol Nephrol. 1996, 28: 295-304.PubMedGoogle Scholar
- Vaideeswar P, Shenoy AS, Sivaraman A, Deshpande JR, Pandit AA: Idiopathic retroperitoneal fibrosis: a report of 3 cases. J Postgrad Med. 1993, 39: 95-98.PubMedGoogle Scholar
- Remedios D, Coppen M, Bradbeer J, Theodossi A: Chronic periaortitis presenting as common bile duct obstruction. Gut. 1991, 32: 713-714.PubMedPubMed CentralGoogle Scholar
- Zdrojewski Z: Retroperitoneal fibrosis and chronic peri-aortitis – new hypothesis. Pol Merkuriusz Lek. 1998, 4: 50-53.Google Scholar
- Turo AJ, Torres LJ, Peiro MF, Cabrera Cabrera JA: Obstructive uropathy caused by retroperitoneal fibrosis secondary to an aneurysm of the abdominal aorta. Actas Urol Esp. 2000, 24: 764-766.Google Scholar
- Breems DA, Haye H, van der MJ: The role of advanced atherosclerosis in idiopathic retroperitoneal fibrosis. Analysis of nine cases. Neth J Med. 2000, 56: 38-44.PubMedGoogle Scholar
- Serra RM, Engle JE, Jones RE, Schoolwerth AC: Perianeurysmal retroperitoneal fibrosis. An unusual cause of renal failure. Am J Med. 1980, 68: 149-153.PubMedGoogle Scholar
- Hautekeete ML, Babany G, Marcellin P, Gayno S, Palazzo E, Erlinger S, Benhamou JP: Retroperitoneal fibrosis after surgery for aortic aneurysm in a patient with periarteritis nodosa: successful treatment with corticosteroids. J Intern Med. 1990, 228: 533-536.PubMedGoogle Scholar
- Izzedine H, Servais A, Launay-Vacher V, Deray G: Retroperitoneal fibrosis due to Wegener's granulomatosis: a misdiagnosis as tuberculosis. Am J Med. 2002, 113: 164-166.PubMedGoogle Scholar
- Regan F, Tran T, Crofton ME: Case report: idiopathic retroperitoneal fibrosis: an unusual cause of an ovarian mass. Br J Radiol. 1993, 66: 558-560.PubMedGoogle Scholar
- Tsai TC, Chang PY, Chen BF, Huang FY, Shih SL: Retroperitoneal fibrosis and juvenile rheumatoid arthritis. Pediatr Nephrol. 1996, 10: 208-209.PubMedGoogle Scholar
- Stey C, Truninger K, Marti D, Vogt P, Medici TC: Bronchiolitis obliterans organizing pneumonia associated with polymyalgia rheumatica. Eur Respir J. 1999, 13: 926-929.PubMedGoogle Scholar
- Bezza A, Maghraoui AE, Ghadouane M, Tabache F, Abouzahir A, Abbar M, Ghafir D, Ohayon V, Archane MI: Idiopathic retroperitoneal fibrosis and ankylosing spondylitis. A new case report. Joint Bone Spine. 2002, 69: 502-505.PubMedGoogle Scholar
- Ewald EA, Gikas PW, Castor CW: Periarticular fibrosis associated with idiopathic retroperitoneal fibrosis. J Rheumatol. 1988, 15: 1443-1446.PubMedGoogle Scholar
- Sozay S, Bayramoglu M, Karatas M, Ozker R: Diffuse idiopathic skeletal hyperostosis in a patient with idiopathic retroperitoneal fibrosis: a case report. Rheumatol Int. 2002, 22: 249-252.PubMedGoogle Scholar
- Knoell KA, Hook M, Grice DP, Hendrix JD: Dermatomyositis associated with bronchiolitis obliterans organizing pneumonia (BOOP). J Am Acad Dermatol. 1999, 40: 328-330.PubMedGoogle Scholar
- Fata F, Rathore R, Schiff C, Herzlich BC: Bronchiolitis obliterans organizing pneumonia as the first manifestation of polymyositis. South Med J. 1997, 90: 227-230.PubMedGoogle Scholar
- Mansell MA, Watts RW: Retroperitoneal fibrosis and scleroderma. Postgrad Med J. 1980, 56: 730-733.PubMedPubMed CentralGoogle Scholar
- Okada H, Takahira S, Sugahara S, Nakamoto H, Suzuki H: Retroperitoneal fibrosis and systemic lupus erythematosus. Nephrol Dial Transplant. 1999, 14: 1300-1302.PubMedGoogle Scholar
- Wallach PM, Flannery MT, Adelman HM, PowSang J, Saba H, Altus P, Espinoza LR: Retroperitoneal fibrosis accompanying immune thrombocytopenia. Am J Hematol. 1991, 37: 204-205.PubMedGoogle Scholar
- Nakamura A, Funatomi H, Katagiri A, Katayose K, Kitamura K, Seki T, Yamamura F, Aoyagi Y, Nishida H, Mitamura K: A case of autoimmune pancreatitis complicated with immune thrombocytopenia during maintenance therapy with prednisolone. Dig Dis Sci. 2003, 48: 1968-1971.PubMedGoogle Scholar
- Iwami S, Ryu T, Kasai S, Tokuda H, Saitoh T: Idiopathic interstitial pneumonia with autoimmune hemolytic anemia. Nippon Naika Gakkai Zasshi. 2003, 92: 1079-1081.PubMedGoogle Scholar
- Pineau BC, Pattee LP, McGuire S, Sekar A, Scully LJ: Unusual presentation of primary sclerosing cholangitis. Can J Gastroenterol. 1997, 11: 45-48.PubMedGoogle Scholar
- Briffod M, Berlie J, Clavel B, Hebert H: Retroperitoneal malignant xanthogranuloma (author's transl). Sem Hop. 1982, 58: 113-119.PubMedGoogle Scholar
- de Sa J, Pimentel J, Carvalho M, Evangelista P, Martins P: Spinal cord compression secondary to idiopathic retroperitoneal fibrosis. Neurosurgery. 1990, 26: 678-681.PubMedGoogle Scholar
- Sevenet F, Capron-Chivrac D, Delcenserie R, Lelarge C, Delamarre J, Capron JP: Idiopathic retroperitoneal fibrosis and primary biliary cirrhosis. A new association?. Arch Intern Med. 1985, 145: 2124-2125.PubMedGoogle Scholar
- Rozynek M: Mesenteric lipodystrophy as a variant of Ormond's disease. Patol Pol. 1975, 26: 525-528.PubMedGoogle Scholar
- Verit A, Yeni E, Unal D, Kafali H, Ozturk A, Ozardali I: Idiopathic retroperitoneal fibrosis mimicking a pelvic tumor: a case of pericystitis plastica. Yonsei Med J. 2003, 44: 548-550.PubMedGoogle Scholar
- Haciyanli M, Erkan N, Elverdi B, Avci G, Fuzun M, Dicle O: Retroperitoneal fibrosis mimicking a rectal tumor: report of a case. Dis Colon Rectum. 1998, 41: 664-666.PubMedGoogle Scholar
- Yamada H, Komatsu R, Nagae H, Fujioka Y, Fujita M: Idiopathic retroperitoneal fibrosis with duodenal obstruction successfully treated with corticosteroids. Intern Med. 1998, 37: 592-598.PubMedGoogle Scholar
- Jun BM, Lee EY, Yoon YJ, Kim EK, Ahn MS, Lee CK, Cho YS, Yoo B, Moon HB: Retroperitoneal fibrosis with duodenal stenosis. J Korean Med Sci. 2001, 16: 371-374.PubMedPubMed CentralGoogle Scholar
- Realini S, Reiner M, Bernasconi S, Pedrinis E: Retroperitoneal fibrosis and portal hypertension. Schweiz Med Wochenschr. 1993, 123: 26-28.PubMedGoogle Scholar
- Zabetakis PM, Novich RK, Matarese RA, Michelis MF: Idiopathic retroperitoneal fibrosis: a systemic connective tissue disease?. J Urol. 1979, 122: 100-102.PubMedGoogle Scholar
- Moroni G, Farricciotti A, Cappelletti M, Ponticelli C: Retroperitoneal fibrosis and membranous nephropathy. Improvement of both diseases after treatment with steroids and immunosuppressive agents. Nephrol Dial Transplant. 1999, 14: 1303-1305.PubMedGoogle Scholar
- Uchiyama-Tanaka Y, Mori Y, Kimura T, Sonomura K, Umemura S, Kishimoto N, Nose A, Tokoro T, Kijima Y, Yamahara H, Nagata T, Masaki H, Umeda Y, Okazaki K, Iwasaka T: Acute tubulointerstitial nephritis associated with autoimmune-related pancreatitis. Am J Kidney Dis. 2004, 43: e18-e25.PubMedGoogle Scholar
- Nistal M, Paniagua R, Torres A, Hidalgo L, Regadera J: Idiopathic peritesticular fibrosis associated with retroperitoneal fibrosis. Eur Urol. 1986, 12: 64-68.PubMedGoogle Scholar
- The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1741-7015/4/23/prepub
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.