Adult-onset Still's disease (AOSD) is an inflammatory disorder affecting multiple systems characterized by polyarthritis, rash, fever, and an elevated erythrocyte sedimentation rate. Macrophage activating syndrome is a severe complication of AOSD leading to pancytopenia, hypofibrinogenemia, elevated triglycerides, lactate dehydrogenase, soluble IL-2 receptor alpha chain (CD25), and ferritin. AOSD is a very rare cause of markedly elevated liver chemistries. A 50-year-old female with a past medical history of asthma developed sore throat, diffuse joint aches, muscle weakness, and a fever of 104° F. She was evaluated at an outside facility and noted to have positive Parvovirus IgM/IgG. She was started on Prednisone 30 mg and with a taper, she experienced a reticular erythematous rash, arthralgias involving the wrist and ankle, myalgias, and cyclic fevers with a temperature max of 100.8° F. She had an aspartate aminotransferase (AST) of 853, alanine transaminase (ALT) of 866, and total bilirubin of 1.5 from prior normal baseline. Complete evaluation for viral hepatitis and hereditary liver disease was negative. Ultrasound with doppler showed parenchymal liver disease with patent vascular flow. Within 48 hours, her AST increased to 2,452, ALT 2,213, and total bilirubin of 3.3. Ferritin was elevated >30,000 ng/mL. Transjugular liver biopsy showed hepatic parenchyma with acidophil bodies, Kupffer aggregates, and preserved architecture with patchy collections of acute and chronic inflammatory cells. No bridging necrosis or iron deposition was appreciated. She was treated with high dose Methylprednisolone 250 mg IV daily with downtrend in transaminases. As steroids were tapered, AST rose from 71 to 198 and ALT from 390 to 900. Percutaneous liver biopsy showed mildly increased inflammation and confluent necrosis. She was discharged on anakinra 100 mg, a recombinant interleukin 1 receptor antagonist, and prednisolone 60 mg twice daily with continued improvement in transaminases to AST of 59 and ALT of 380. The patient developed AOSD likely induced by parvovirus which was complicated by MAS and transaminase elevation >5 upper limit of normal. The pathogenesis of liver involvement of AOSD is unknown but it is presumed that sustained macrophage activation with cytokine production play a role. This is evidenced in this case as transaminases decreased with steroid dosing and increased with the initial taper.
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