Introduction Macrophage activation syndrome (MAS) is a rare life threatening complication seen with various infectious disorders, hematologic malignancies as well as in connective tissue diseases including adult Still's disease (ASD), juvenile rheumatoid arthritis, systemic lupus erythematosus, Kawasaki's disease and dermatomyositis. We describe a case of Still's disease complicated by MAS.
Case A 29 year old African American female was admitted with a one month history of daily fevers, pruritic rash, fatigue and arthralgia. Physical examination was notable for fever of 101.6°C, generalized lymphadenopathy, diffuse maculopapular rash, splenomegaly and synovitis of the right wrist, proximal interphalangeal and ankle joints. Pertinent laboratory evaluation on admission included: hemoglobin (Hb) 8.6 g/dL, white blood cells (WBC) 18.7 k/μL, platelet (PLT) 396 k/μL, ferritin 7500 ng/mL, erythrocyte sedimentation rate (ESR) 78 mm/hr, LDH 3136 U/L and AST 117 U/L. Antinuclear antibodies (ANA) were positive (1:320), however anti-Smith, anti-double stranded-DNA, and rheumatoid factor were absent. Nine months prior to this admission, she was admitted to another facility with similar symptoms. At that time, an extensive workup including lymph node, liver and bone marrow biopsies revealed no infectious disorder or malignancy. She was treated with NSAIDs and steroids with improvement in her symptoms. During the current admission, a diagnosis of Still's disease was made after excluding bacterial, mycobacterial, viral and fungal infections. She was started on intravenous (IV) Solu-Medrol one gram daily. Over the next four days, she developed confusion and her laboratory values dropped (Hb - 6.6 g/dL, WBC - 4 k/μL, PLT - 139 k/μL, ESR - 6 mm/hr). Ferritin increased to 13,100 ng/mL accompanied by an increase in liver enzymes, mild coagulopathy and normal fibrinogen. Bone marrow biopsy revealed features of hemophagocytic syndrome. In addition to IV Solu-Medrol, plasma exchange was initiated. Her confusion resolved, and all laboratory values improved after a few days.
Discussion MAS can rarely be associated with ASD and has a high mortality rate. The presence of unremitting fever, mental status changes, rising liver enzymes, falling ESR, normal or low fibrinogen, and relative pancytopenia with prolongation of the PT/PTT should raise suspicion of MAS complicating Still's disease. Steroid refractory cases have been treated with plasmapheresis, cyclosporine, etoposide and IV immunoglobulin. Response to treatment is manifested by resolution of fever and hematological abnormalities in a few days.
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