Table 2 Studies including patients affected with Sjögren’s syndrome (SS) treated with rituximab

Somer et al., 2003 [109]

1

Case report

Marginal zone lymphoma, xerophthalmia, xerostomia

Improvement in corneal staining, Schirmer’s test, salivary flow rate, tear production, salivary pooling, diminished parotid enlargement

No AE reported

Voulgarelis et al., 2004 [110]

4

Case reports

Marginal zone lymphoma, parotid gland enlargement, lymphadenopathy, cryoglobulinemia, purpura, peripheral neuropathy, arthralgia

Improvement in lymphoma, arthralgia, cryoglobulinemia, purpura, peripheral neuropathy (50%)

No AE reported

Gottenberg et al., 2005 [111]

6

Retrospective

2 MALT lymphomas, 2 vasculitis with cryoglobulinemia, 2 parotid gland enlargement and articular involvement

Improvement in parotid swelling, subjective dryness, fatigue and vasculitis in 5 out of 6 patients

1 serum sickness, 1 infusion reaction

Pijpe et al., 2005 [112]

15

Open label

8 early primary SS and 7 MALT lymphoma: 8 parotid gland swelling, 8 Raynaud’s phenomenon, 13 fatigue, 11 arthralgia, 2 pulmonary involvement, 2 vasculitis

Remission of lymphoma in 3 of 7 patients, disease stability in 3 of 7, progression in 1. Increased salivary secretion, improvement in the rose Bengal score and tear, BUT, mouth dryness, arthralgia. No improvement on Schirmer test.

2 infusion reactions, 1 Herpes zoster

Ring et al., 2006 [113]

1

Case report

Distal renal tubular acidosis, xerostomia with mouth ulcerations

Xerostomia improvement

No AE reported

Seror et al., 2007 [114]

16

Retrospective

5 lymphoma, 2 pulmonary involvement, 2 polysynovitis, 5 mixed cryoglobulinemia, 1 thrombocytopenia, and 1 mononeuritis multiplex

Remission of lymphoma in 4 of 5 patients; improvement of systemic involvements in 9 of 11, subjective dryness in 5 of 16, and regression of keratitis in 2 of 11. No response on salivary flow and Schirmer test.

2 infusion reactions, 1 serum sickness

Devauchelle-Pensec et al., 2007 [115]

16

Open label

Sicca symptoms, pain, fatigue; 1 pulmonary involvement

Improvement in subjective fatigue, pain, dryness, and pulmonary involvement. No changes in unstimulated salivary flow, salivary gland score and ophthalmologist evaluation.

3 infusion reactions, 1 serum sickness

Dass et al., 2008 [116]

8 (PL 9)

RCT

Fatigue, ocular and mouth dryness; no systemic involvement

Improvement in fatigue, general health and SF-36. No improvement on Schirmer test and salivary flow rate.

2 infusion reactions, 1 delayed reaction with meningism, 1 gastroenteritis and palpitation

Galarza et al., 2008 [117]

8

Open label

Severe glandular and musculoskeletal involvement, cutaneous vasculitis

Improvement in parotid swelling, articular involvement, fatigue, and subjective dryness in 4 of 7 patients

3 AE: 2 infusion reactions

Ramos-Casals et al., 2010 [118]

15

Registry

6 lymphoma, 4 neurological involvement, 2 hematological involvement, 1 refractory glomerulonephritis, arthritis, and protein-losing enteropathy

Complete response in 67% of patients, partial response in 20%, no response in 13%

1 urinary tract infection, 1 interstitial pneumonitis

Meijer et al., 2010 [119]

20 (PL 10)

RCT

15 arthralgia, 6 arthritis, 2 renal involvement, 1 peripheral neuropathy, 11 Raynaud’s phenomenon, 17 tendomyalgia, 6 vasculitis, thyroid dysfunction

Improvement in saliva flow rate, stimulated lacrimal gland function; but not in BUT and Schirmer test. Improvement in SF-36 and MFI. Improvement in extraglandular manifestations.

1 serum sickness, 12 infections in 11 patients on rituximab vs 7 infections in 4 patients on placebo

Mekinian et al., 2012 [120]

17

Registry

All peripheral nervous system involvement: 10 patients with cryoglobulinemia and/or vasculitis, 7 patients without cryoglobulinemia and/or vasculitis

Response in 9 of 10 patients with cryoglobulinemia and/or vasculitis, and in 2 of 7 without cryoglobulinemia and/or vasculitis

6 (35%) AE: 2 mild arterial hypertension, 1 infusion reaction, 1 cutaneous infection, CMV infection, hypogammaglobulinemia