Macrophage Activation Syndrome Originating from Primary Biliary Cholangitis: A Case Report

Abstract

A 49-year-old female had intermittent fever for one year, with maximum recorded temperature of 39.0℃, accompanied by itchy skin, no joint swelling and pain, ineffective in antibiotic treatment, and responding well to non-steroidal anti-inflammatory drugs (NSAIDs). Patchy rash appeared on the trunk during fever, which faded away when the fever subsided. One month ago, she developed hyperpyrexia again, complicated by anorexia. Pertinent laboratory findings were as follows: hemoglobin, 98 g/L; aspartate aminotransferase (AST), 806 U/L; alanine aminotransferase (ALT), 644 U/L; alkaline phosphatase (ALP), 279 U/L; gamma-glutamyl transferase (GGT), 115U/L; C-reactive protein, 77.04 mg/L; erythrocyte sedimentation rate (ESR), 58mm/h; ferritin, 6415 ng/mL; anti-nuclear Ab (ANA), speckled pattern, 320(+); anti-M2 Ab (AMA-M2), 41.27 units (+). Infection and tumor screening showed no obvious abnormality.