Pulmonary arterial hypertension in adult onset Still's disease: a case report of a severe complication

被引：13

作者：

Guilleminault, L. ^{[1
,2
,3
,5
]}

Laurent, S. ^{[1
]}

Foucher, A. ^{[4
]}

Poubeau, P. ^{[4
]}

Paganin, F. ^{[1
,3
]}

机构：

[1] CHU Reunion GHSR, Serv Pneumol, F-97410 St Pierre, Reunion, France

[2] INSERM, UMR Diabet Atherothrombose Therapies Reunion Ocea, Plateforme CYROI, F-97490 St Clotilde, Reunion, France

[3] Univ La Reunion, UMR 1188, F-97490 St Clotilde, Reunion, France

[4] CHU Reunion GHSR, Serv Malad Infect, F-97410 St Pierre, Reunion, France

[5] Grp Hosp Sud Reunion, Serv Pneumol, BP 350, F-97448 St Pierre, Reunion, France

来源：

BMC PULMONARY MEDICINE | 2016年 / 16卷

关键词：

Case report; Pulmonary arterial hypertension; Adult onset Still's disease; Auto-inflammatory disease; THROMBOTIC MICROANGIOPATHY; MANIFESTATIONS; CLASSIFICATION; CRITERIA;

D O I：

10.1186/s12890-016-0237-x

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

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.

引用

页数：4

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