Review: Macrophage activation syndrome in juvenile systemic lupus erythematosus: an under-recognized complication?

Abstract

Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic diseases that is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread haemophagocytosis and cytokine overproduction. It is seen most commonly in systemic juvenile idiopathic arthritis, but is increasingly recognized also in juvenile systemic lupus erythematosus (J-SLE). Recognition of MAS in patients with J-SLE is often challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication. This review summarizes the characteristics of patients with J-SLE-associated MAS reported in the literature or seen by the authors and analyses the distinctive clinical, diagnostic and therapeutic issues that the occurrence of MAS may raise in patients with J-SLE.

A 21‐year‐old man with Still's disease with fever, rash, and pancytopenia

“…Infectious triggers can be bacterial, viral, fungal, mycobacterial, or parasitic (7), the most common being Epstein-Barr virus (17,18). The associated rheumatic conditions can be systemic JIA, systemic lupus erythematosus, sarcoidosis, or rheumatoid arthritis (7,19). Reports of patients treated with drugs associated with cases of MAS include MTX (20), gold injections (especially the second injection), and sulfasalazine (9), although TNF inhibitors such as etanercept (21) have been described.…”

Etanercept-Induced Lupus Accompanied by Hemophagocytic Syndrome

“…Some authors have insisted that hemophagocytosis is not essential for the diagnosis of HPS nor should it be overestimated in the absence of any signs of massive histiocytic activation (19). The present patient fulfilled both the 2004 and 2009 diagnostic criteria proposed by the Histiocyte Society, based on fever, bicytopenia, hypofibrinogenemia, hyperferritinemia, elevated sIL-2R, liver dysfunction, and hyponatremia, despite which, it was challenging to differentiate HPS from active flare of lupus since some clinical features may overlap (20). Recently, Parodi et al published a paper on this point (21).…”

Macrophage activation syndrome

“…Może również wystąpić w przebiegu choroby Stilla, TRU (0,9-4,6% przypadków) [15,16], w reumatoidalnym zapaleniu stawów (RZS), zespole Sjögrena, zapaleniu skórno-mięśniowym, chorobie Kawasaki, mieszanej chorobie tkanki łącznej oraz w twardzinie układo-wej [5,7,12,17].…”

“…Diagnostyka MAS w przebiegu TRU, w którego obrazie klinicznym mieszczą się leuko-i trombocytopenia, też jest trudna. Pomocna może być hiperferrytynemia i zwiększone stężenie LDH [13][14][15]18] Oprócz diagnostyki zmierzającej do rozpoznania MAS, zaleca się badania w kierunku zakażeń wirusowych, spośród których badania metodą reakcji łańcuchowej polimerazy (polymerase chain reaction -PCR) mają większą wartość niż testy serologiczne [4]. W 2010 r. Instytut "Pomnik -Centrum Zdrowia Dziecka" (IP-CZD) w Warszawie przedstawił propozycję standardu diagnostyczno-terapeutycznego dla dzieci z podejrzeniem pierwotnego lub wtórne-go HLH [3].…”