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Table 3 The effectiveness of common treatment modalities: MAS, AOSD, septic shock and cAPS

From: The Hyperferritinemic Syndrome: macrophage activation syndrome, Still’s disease, septic shock and catastrophic antiphospholipid syndrome

  Corticosteroids IVIG Blood purification/Plasma exchange Others
MAS +++ [55] ++ [55] ++ [16, 7375] Cyclosporine A [55]
AOSD +++ [41, 65] ++ [41, 76] + [59, 77, 78] DMARDs [41, 65]; Anti-IL-6 [41, 48]; Anti-IL-1 [41, 48]
cAPS +++ [46] +++ [46, 79, 80] +++ [46, 81] Anticoagulation [46, 70]
Septic shock +/− [54, 82, 83] +/− [84] ++ [8588] Antibiotics [54]
Rationale Anti-inflammatory effects of corticosteroids rely on their ability to repress the activity of immunomodulatory transcriptor factors like NF-κB and activator protein-1 [89]. Direct antitoxic effects, as well as indirect immunomodulatory mechanisms of IVIG has been described in the literature [84]. The overall concept of blood purification is to attenuate the overwhelming systemic overflow of pro- and anti-inflammatory mediators and to restore a broad-based humoral homeostasis [90].  
IVIG probably acts by cytokine- and pathogen-specific antibodies [55, 91].
They are cytotoxic for lymphocytes and inhibit expression of cytokines and differentiation of dendritic cells [55]. IVIG prevents release of pro-inflammatory cytokines in human monocytic cells stimulated with procalcitonin [92]. It is an extracorporeal blood purification technique designed to remove various toxic and inflammatory mediators and to replenish essential compounds via the replacement plasma [16].
  1. +++ first line treatment recommended in international literature, ++ recommended treatment based in series cases reported in the literature, + treatment used in clinical practice described in case reports, +/− controversial use in clinical practice. AOSD adult onset Still’s disease, cAPS catastrophic antiphospholipid syndrome, DMARDs disease-modifying antirheumatic drugs, IVIG intravenous immunoglobulin, MAS macrophage activation syndrome, NF-κB nuclear factor kappaB, NR not reported.
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