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
Categories: Internal Medicine, Infectious Disease,
Keywords : Immune dysregulation syndrome, systemic lupus erythematosus, hemophagocytic lymphohistiocytosis, mixed connective tissue disease, macrophage activation syndrome.