Macrophage Activation Syndrome Secondary to Mixed Connective Tissue Disease: A Rare Case in a Tertiary Care Hospital of Eastern India

Authors : Jyoti Prakash; Rajdeep Porel; Neeraj Kumar; Ratnadeep Biswas; Vishnu S. Ojha

Volume/Issue : Volume 8 - 2023, Issue 8 - August

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Macrophage activation syndrome (MAS) is considered a rare, potentially fatal type of secondary hemophagocytic lymphohistiocytosis (HLH) that occurs especially in cases of rheumatic diseases. 18-year- old female presented with a history of rheumatoid arthritis who presented with fever, headache, vomiting, altered sensorium, and abnormal limb movements. She also had joint pains, alopecia, and oral ulcers. Examination revealed neurological signs, elevated inflammatory markers, and abnormal liver function. Cerebrospinal fluid analysis showed increased protein levels. Urine analysis showed proteinuria. Anti- nuclear antibody profile indicated possible systemic lupus erythematosus (SLE) and rheumatoid arthritis. She developed aspiration pneumonitis during her hospital stay with a culture positive for Acinetobacter baumannii. A diagnosis of macrophage activation syndrome (MAS) secondary to mixed connective tissue disorder (MCTD) was made based on clinical and laboratory parameters. She was started on intravenous colistin and methylprednisolone, but unfortunately, she eventually succumbed to the overwhelming infection.Thus, in cases of MCTD, MAS can manifest as a life- threatening condition. Therefore, it is crucial to maintain a high level of clinical suspicion for MAS in rheumatological diseases to enable early initiation of treatment with steroids or other immunomodulatory agents. Categories: Internal Medicine, Infectious Disease, Rheumatology.

Keywords : Immune dysregulation syndrome, systemic lupus erythematosus, hemophagocytic lymphohistiocytosis, mixed connective tissue disease, macrophage activation syndrome.


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