Multiorgan Dysfunction in a Patient With Adult-Onset Still’s Disease Flare: A Case Report

TL;DR

A 71-year-old woman with AOSD experienced multiorgan failure, including heart, liver, and kidney dysfunction, which partially improved after treatment.

Contribution

This case report highlights a systematic approach to managing multiorgan dysfunction in a rare condition like AOSD.

Findings

Liver dysfunction was due to congestive hepatopathy, while kidney dysfunction was from a low output state.

Cardiac function remained compromised despite recovery of liver and kidney function.

Treatment included high-dose steroids and anakinra, leading to partial recovery.

Abstract

Adult-onset Still’s disease (AOSD) is a rare multisystem inflammatory disorder. A 71-year-old lady who was on treatment for AOSD presented with clinical evidence of heart failure and was subsequently found to have impaired renal and hepatic function. Following extensive workup including a liver biopsy, the cause of liver dysfunction was determined to be congestive hepatopathy, while renal dysfunction was presumed to stem from the low output state. The etiology of myocardial dysfunction, driving liver and kidney injury, was considered to be myocarditis from AOSD or global myocardial dysfunction from a systemic inflammatory state. Management involved pulse-dose glucocorticoids followed by taper and anakinra for AOSD, alongside goal-directed medical therapy for cardiac failure. At follow-up after a month, hepatic and renal function had fully recovered, whereas cardiac function remained compromised, evidenced by persistently depressed ejection fraction and global hypokinesia on a repeat echocardiogram. This report delineates a systematic approach to multiorgan dysfunction in a patient with a rare condition such as AOSD and reviews the reported causes of hepatic and cardiac involvement in AOSD.

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