Adult-Onset Still's Disease With Macrophage Activation Syndrome During Pregnancy: A Unique Cause of Hepatic Dysfunction

Abstract

We present a rare case of adult-onset Still's disease (AoSD) with macrophage activation syndrome (MAS) marked by profound hepatic dysfunction during pregnancy. A 41-year-old woman with a history of hepatic steatosis was initially admitted at 28-week gestation for nausea, vomiting, proteinuria, and mildly elevated transaminases concerning for preeclampsia. Two weeks after admission, she developed daily fevers, an evanescent rash, and rising transaminases (Figure 1) necessitating delivery through caesarean section at 33-week gestation.After delivery, she had persistent fevers and underwent an extensive negative workup for fever of unknown origin. She was treated empirically with antibiotics and underwent a cholecystectomy for possible cholecystitis. Intraoperatively, her liver appeared purplish and friable, so a wedge liver biopsy was performed showing hepatic necrosis and hemophagocytes (Figure 2). Computed tomography abdomen showed hepatomegaly with patent hepatic veins and portal veins. She then developed acute encephalopathy along with worsening coagulopathy and was transferred for expedited liver transplant evaluation. DISCLOSURESAuthor contributions: TA Mai and D. Ayyala-Somayajula wrote the manuscript. J. Singh, L. Wise, and L. Yuan revised the manuscript. D. Braxton provided the pathology images. L. Yuan revised the manuscript and is the article guarantor. All authors discussed the results and contributed to the final manuscript.