Clinical features of adult-onset Still's disease patients with pulmonary complications

Abstract

Objective To review the clinical characteristics of adult-onset Still's disease (AOSD) patients with special pulmonary complications. Methods Medical records of 506 AOSD inpatients, who were admitted in Peking Union Medical Hospital from October 1997 through March 2015, were retrospectively reviewed. The clinical features, biochemical parameters, radiology, pulmonary function tests, ultrasound, treatments and prognosis of AOSD patients with special pulmonary complications were analyzed. Results The incidence of special pulmonary complications in AOSD inpatients was 2.2% (11/506), including 3 cases of interstitial lung disease and 8 cases of pulmonary artery hypertension. All 11 patients had common manifestations of AOSD, including fever (11/11), arthralgia/arthritis (11/11), rash (9/11), lymphadenopathy and/or splenomegaly (8/11), serositis (10/11), abnormal liver function (9/11), neutrophilia (11/11), and hyperferritinemia (11/11). The patients with interstitial lung disease had no or mild symptom, and were diagnosed by HRCT and pulmonary function test. The patients with pulmonary artery hypertension had various symptoms including dyspnea of exertion (8/8), cough (4/8), chest suppression (2/8), and chest pain (2/8). Four patients died from severe infection, including 3 cases of pulmonary artery hypertension and 1 case of interstitial lung disease. Conclusions Interstitial lung disease and pulmonary artery hypertension are life-threatening complications of AOSD patients. Earlier recognition and prompt treatment should be made to improve patient outcomes. Key words: Still's disease, adult-onset; Lung disease; Signs and symptoms