SYSTEMIC LUPUS ASSOCIATED MACROPHAGE ACTIVATION SYNDROME - A SINGLE CENTRE EXPERIENCE FROM INDIA

Background: Macrophage activation syndrome (MAS) is a potentially fatal complication of systemic lupus erythematosus (SLE).(1) Overlapping clinical features of SLE activity with MA makes it a clinical diagnosis. There is limited data from developing countries like India. Objectives: To study the clinical features, treatment, and outcomes in SLE-MAS. Methods: This single-centre, retrospective study included patients fulfilling SLICC 2011 criteria for SLE, admitted from January 2017 to November 2019, and diagnosed as MAS by a team of attending physicians. Their demographic and clinical data, treatment, and outcomes were recorded. H score and SLEDAI were calculated. Patients were assessed for HLH classification criteria (2004) and preliminary diagnostic criteria for MAS in childhood SLE (cSLE). Results: Sixteen patients (median age – 26 years, 15 females) were included. Twelve patients (75%) had MAS as the initial presentation of SLE. The common clinical features were fever (100%) and cytopenias (100%). The mean duration of symptoms was 60 days. The most frequent biochemical abnormalities were high ferritin (\u003e500 ng/ml, 100%) and elevated transaminases (100%, aspartate transaminase \u003e alanine transaminase). Common complications were renal (43.8%), neurological (43.8%), and coagulopathy (43.8%). Seven and 16 patients fulfilled the HLH 2004 and cSLE – MAS preliminary criteria, respectively. The median H score was 222, giving a cumulative probability of 96%. All the patients received high-dose steroids. Cyclophosphamide pulse and cyclosporine were administered to 8 (50%) and 6 (37.5%) patients respectively. There were four (25%) in-hospital mortalities. Conclusion: Fever, cytopenia, high ferritin, and elevation of transaminases were the commonest features in this series of SLE-MAS. SLE-MAS carried a high mortality (25%) despite aggressive treatment. References: [1]Gavand P-E, Serio I, Arnaud L, Costedoat-Chalumeau N, Carvelli J, Dossier A, et al. Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: A study of 103 episodes in 89 adult patients. Autoimmun Rev. 2017;16(7):743–9. Acknowledgments: NONE Disclosure of Interests: None declared