AB0395 SYSTEMIC LUPUS ASSOCIATED MACROPHAGE ACTIVATION SYNDROME – A SINGLE CENTRE EXPERIENCE FROM INDIA
Authors: N. Acharya, S. Jha, D. Mishra, A. Chattopadhyay, S. Jain, S. K. Sharma, A. Sharma, S. Jain, V. Dhir
Journal: Annals of the Rheumatic Diseases (2020)

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 (&gt;500 ng/ml, 100%) and elevated transaminases (100%, aspartate transaminase &gt; 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. Characteristic N=16 Age (years) 26 (19.5 – 30) Duration of symptoms (days) 60 (18.7 – 90) Fever 16 (100) Pancreatitis 1 (6.3) Renal 7 (43.8) Neurological 7 (43.8) Myocarditis 6 (37.5) Hepatomegaly 7 (43.8) Splenomegaly 3 (18.8) Lymphadenopathy 12 (75) Anaemia 16 (100) Leukopenia 13 (82) Thrombocytopenia 14 (88.2) Coagulopathy 7 (43.8) Concomitant infection 3 (18.8) H score 222 (193 – 254) cSLE-MAS diagnostic criteria 16 (100%) HLH 2004 (≥5) 7 (43.8) Corticosteroids 16 (100%) Cyclophosphamide 8 (50%) Intravenous immunoglobulin 2 (12.5%) Cyclosporine 6 (37.5%) Death 4 (25%) All data presented as n (%) and median (IQR) cSLE-MAS – Childhood systemic lupus erythematosus – Macrophage activation syndrome, HLH – Hemophagocytic lymphohistiocytosis Parameter N = 16 Haemoglobin (g/dL) 6.9 (5.7 – 7.8) Total leucocyte count (cells/mm3) 1400 (1025 -3175) &lt;1000 3 (18.8) 1000 – 2500 9 (56.3) 2500 - 4000 1 (6.3) Platelet (cells/mm3) 57500 (23500 – 95250) &lt;20000 3 (18.8) 20000 – 50000 3 (18.8) 50000 – 1 lac 8 (50) Serum ferritin &gt; 500 ng/ml 16 (100%) Fibrinogen 2.48 (1.6 – 4) Triglycerides (mg/dL) 375 (294 -470) AST/ALT (U/L) 153 (113 – 234) / 90 (68 – 170) Procalcitonin (pg/mL) 0.8 (0.3 – 1.4) Lactate dehydrogenase (U/L) 1674 (1081 – 2184) Erythrocyte sedimentation rate (mm in 1 st hour) 61 (44 – 69) C- reactive protein (mg/L) 36 (6.3 – 52) Bone marrow examination 14 (87.5) Increased histiocytes with hemophagocytosis 5 (32.5) All data presented as n (%) and median (IQR) ALT – Alanine transaminase, AST – Aspartate transaminase 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