Pulmonary arterial hypertension in adult onset Still's disease: a case report of a severe complication. Issue 1 (December 2016)

• Record Type: Journal Article
• Authors: Guilleminault, L.; Laurent, S.; Foucher, A.; Poubeau, P.; Paganin, F.
• Is Part Of: BMC pulmonary medicine. Volume 16:Issue 1(2016)
• Page Start: 1
• Page End: 4
• Publication Date: 2016-12
• Subjects: Case report -- Pulmonary arterial hypertension -- Adult onset Still's disease -- Auto-inflammatory disease; Lungs -- Diseases -- Periodicals; 616.24005
• DOI: 10.1186/s12890-016-0237-x
• Languages: English
• ISSNs: 1471-2466

Abstract

Abstract Background Adult onset of Still's disease (AOSD) is a rare systemic inflammatory disease. Cardiorespiratory complications are mainly represented by pleural and pericardial disorders and are less frequent than cutaneous and articular complaints. Pulmonary arterial hypertension (PAH) occurring in AOSD is rarely described in literature. Case presentation We present the case of a young patient who developed severe PAH 2 years after diagnosis of AOSD. This is a rare and severe complication which is probably underestimated. Conclusions PAH in AOSD can be lethal, and unfortunately its occurrence is unpredictable. Echocardiographic screening of AOSD patients should be evaluated in further trials. Currently, the most suitable treatment is still unknown.