Table 3

The effectiveness of common treatment modalities: MAS, AOSD, septic shock and cAPS
Corticosteroids IVIG Blood purification/Plasma exchange Others
MAS +++ [55] ++ [55] ++ [16,73-75] 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] ++ [85-88] 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].

+++ 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.

Rosário et al.

Rosário et al. BMC Medicine 2013 11:185   doi:10.1186/1741-7015-11-185

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