Ab1010 macrophage Activation Syndrome: A Case Series of 16 Patients

Abstract

BackgroundMacrophage activation syndrome (MAS) is a severe complication of several rheumatologic diseases, being of special relevance systemic lupus erythematosus (SLE) and systemic juvenile idiopathic arthritis (sJIA). Its characterized by an excessive activation of the immune system due to various mechanisms, including hyperactivation of macrophages and a failure in downregulation activity by NK and cytotoxic lymphocytes. There are various criteria for its diagnosis, highlighting secondary lymphohistiocytosis syndrome (HLH) criteria from 2004 and provisional secondary MAS criteria for JIA proposed by Ravelli in 20161.ObjectivesTo describe a case series of patients with MAS.MethodsThis is a retrospective case series of 16 patients with MAS secondary to systemic autoimmune diseases diagnosed in Ramón y Cajal Universitary Hospital between April 2009 and September 2018.ResultsThe baseline pathology was sJIA in 8 patients (2 cases with 2 episodes) and SLE in the other 6 patients. Mean age at diagnosis was 17.44 years for sJIA and 37.5 years for SLE. Mean time from diagnosis of the baseline disease to MAS episode was 11.31 years, with 3 cases being the initial manifestation of their systemic disease. 43.8% of patients were treated with corticosteroids previously to MAS episode, and 50% were being treated with DMARDS/biologic agents (SLE: 3 patients with hydroxychloroquine and 1 patient with mycophenolate and hydroxychloroquine; sJIA: 2 patients with Anakinra, 1 patient with tocilizumab and 1 patient with etanercept). Clinical and analytical characteristics of the patients are presented in table 1 and table 2, respectively. In SLE group, only 2 patients (33.3%) had high anti-DNA titers during the MAS episode, 5 patients (83.3%) had increased C3 consumption and 4 patients (66.6%) had increased C4 consumption. As severe manifestations, 4 SLE patients presented neurologic abnormalities and 3 patients presented external hemorrhage. Infection was confirmed as a trigger in 3 patients with SLE (50%) and 4 patients with JIA (40%). Prednisone at high doses was prescribed to all patients, cyclosporine in 4 patients (66.6%) with SLE and 9 patients (90%) with sJIA. Additionally, anakinra was prescribed in 4 patients (40%) with sJIA. 4 patients (66.6%) with SLE met secondary HLH criteria and 9 patients (90%) with sJIA met secondary MAS criteria. Bone marrow biopsy was performed in all patients with SLE and in 9 patients with sJIA, demonstrating hemophagocytosis in 5 patients (83.3%) with LES and 5 (50%) patients with sJIA. Only 2 patients in the SLE group died because of MAS.Table 2 Analytical measures. Hb=haemoglobin(g/dl), Nt=neutrophils (103/mm2), Pla=platelets (103/mm2), Cr=creatinine (mg/dl), Brb=bilirubin (mg/dl), AST (UI/L), ALT (UI/L), Fe=ferritine (ng/ml), Tg=triglycerides (mg/dl), Fb=fibrinogen (mg/dl), INR (International Normalizated Ratio). Hypotension Fever Rash Adenopathy Splenomegaly Hepatomegaly Haemorrhage sJIA 1 (10%)7 (70%)7 (70%)3 (30%)4 (40%)1 (10%)0 SLE 04 (66.7%)1 (16.7%)3 (50%)5 (83.3%)4 (66.7%)3 (50%...