Acute Liver Failure in Adult-Onset Still Disease: A Diagnostic Challenge

Introduction: Adult-onset still’s disease (AOSD) is a systemic inflammatory disease of unknown etiology, characterized by involvement of multiple organ systems, including the liver. Although hepatic involvement typically presents as mildly elevated transaminases, in rare cases, it can progress to acute liver failure.


Description: 48-year-old female with a new diagnosis of AOSD on high dose prednisone and Bactrim for PJP prophylaxis, presented to the ER with a 3-day history of nausea, vomiting, and abdominal pain. On examination, she was febrile and icteric. Initial laboratory investigations revealed ALT/AST: 2901/ 2918 u/L, INR of 3.0, elevated lactate levels, and an elevated procalcitonin level and Ferritin level of . Her MELD score at the time of admission was calculated to be 33. Notably, LFTs had been within normal limits two weeks prior. Imaging studies included a CT of the abdomen, which showed diffuse colitis. HIDA scan revealed the absence of radiotracer excretion, and MRCP identified gallbladder wall edema without evidence of cholelithiasis.
The differential of her acute liver injury included drug-induced liver injury (DILI) from TMP-SMX, hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS), autoimmune hepatitis, or ischemic hepatitis. Patient was started on IV N-acetylcysteine, corticosteroids, and atovaquone. Due to worsening liver failure, patient was transferred to a tertiary care facility for liver transplant. Liver biopsy was contemplated but was ultimately deferred after multidisciplinary meeting as histopathology was unlikely to distinguish between overlapping etiologies in an acute setting. Patient showed improvement over next 8 days on steroids and did not require liver transplant. After clinical stabilization, patient was discharged with a tapering course of prednisone and continued on atovaquone.


Discussion: This case highlights the challenge of managing acute liver injury in the context of AOSD, which may result from disease itself, AOSD-induced macrophage activation syndrome, or DILI. High clinical suspicion, timely escalation of care to a liver transplant center and a multidisciplinary approach are critical in ensuring a favorable clinical outcome.