|The effectiveness of common treatment modalities: MAS, AOSD, septic shock and cAPS|
|Corticosteroids||IVIG||Blood purification/Plasma exchange||Others|
|MAS||+++ ||++ ||++ [16,73-75]||Cyclosporine A |
|AOSD||+++ [41,65]||++ [41,76]||+ [59,77,78]||DMARDs [41,65]; Anti-IL-6 [41,48]; Anti-IL-1 [41,48]|
|cAPS||+++ ||+++ [46,79,80]||+++ [46,81]||Anticoagulation [46,70]|
|Septic shock||+/− [54,82,83]||+/− ||++ [85-88]||Antibiotics |
|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 .||Direct antitoxic effects, as well as indirect immunomodulatory mechanisms of IVIG has been described in the literature .||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 .|
|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 .||IVIG prevents release of pro-inflammatory cytokines in human monocytic cells stimulated with procalcitonin .||It is an extracorporeal blood purification technique designed to remove various toxic and inflammatory mediators and to replenish essential compounds via the replacement plasma .|
+++ 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