CLINICAL PROFILE AND OUTCOMES OF MACROPHAGE ACTIVATION SYNDROME IN SYSTEMIC LUPUS ERYTHEMATOSUS: A RETROSPECTIVE STUDY FROM A SOUTH INDIAN TERTIARY CARE CENTER

Abstract

Background:  Macrophage Activation Syndrome (MAS) represents a severe and potentially life-threatening complication of Systemic Lupus Erythematosus (SLE), characterized by excessive activation and proliferation of macrophages, leading to a cytokine storm and multi-organ dysfunction. While MAS is relatively rare, its occurrence in SLE patients poses significant diagnostic and therapeutic challenges due to its overlapping clinical features with other disease entities and the potential for rapid deterioration.

Methods: We identified patients afflicted with Systemic Lupus Erythematosus, presenting with an acute flare or concomitant infection, which led to Macrophage Activation Syndrome. Clinical data was obtained through review of medical records. The data reported here are those available from January 2016 to March 2024. The objective of this study is to study the clinical profile of SLE patients presenting with MAS in our tertiary care hospital.

Results: We identified 10 patients of SLE with confirmed Macrophage Activation Syndrome. The mean (±SD) age of the patients was 36 ± 11.85 years, women were afflicted more than men in the ratio 4:1, and the symptoms began 7±4 days before admission. The most common symptoms were fever (n=10, 100%) and skin rashes (n=5, 50%). All patients were admitted in the ICU out of which 30% died. Patients were managed with IV steroids, cyclophosphamide and antibiotics (if indicated). Statistical analysis revealed disease severity (SLEDAI score) as the most significant predictor of mortality in this patient population, highlighting the importance of managing disease activity in SLE patients with MAS.

Conclusion:  SLE patients with high disease activity and concomitant MAS are more likely to have a poor prognosis. The study also reinforces the necessity for heightened clinical suspicion, early diagnosis, and prompt, aggressive treatment of MAS to improve patient outcomes. Comparisons with larger, multi-center studies highlight the need for standardized diagnostic criteria and treatment protocols, as well as further research to for better understanding of MAS in SLE.