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

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.

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