Pulmonary Arterial Hypertension in Adult-Onset Still's Disease: A Rare but Severe Complication.

Abstract

BACKGROUND Adult-onset Still's disease (AOSD) is a rare multisystem inflammatory disorder with a highly variable clinical presentation. Pulmonary complications of AOSD most commonly include pleural effusion and transient pulmonary infiltrates. In extremely rare cases, pulmonary arterial hypertension (PAH) develops as a complication. We present the case of a 49-year-old woman with adult-onset Still's disease presenting with fever, dyspnea, and pleuritic chest pain who was diagnosed with PAH. CASE REPORT A 49-year-old woman with a history of adult-onset Still's disease presented to the Emergency Department due to 1 week of fever, dyspnea, and pleuritic chest pain. Imaging, cardiac, immunologic, and infectious workups were performed and detected elevated inflammatory markers. She then underwent right-heart catheterization, which revealed high pulmonary artery pressure (PAP) and mean PAP at 43/18 mmHg and 27 mmHg, respectively. The patient was stabilized and discharged for further management of heart failure with preserved ejection fraction, and group 1 pulmonary arterial hypertension secondary to Still's disease. CONCLUSIONS Pulmonary complications of adult-onset Still's disease, such as PAH, are rare but potentially life-threatening. The treatment of PAH in adult-onset Still's disease involves the use of pulmonary vasodilators, immunosuppressive therapy, and regular monitoring to assess the prognosis of PAH. Our case report highlights the importance of considering PAH in patients with adult-onset Still's disease who present with dyspnea, fatigue, and chest pain. Increased clinician awareness of this extremely rare complication of AOSD can assist with rapid identification and improved patient outcomes.