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BMC Pulm Med. 2016 May 10;16(1):72. doi: 10.1186/s12890-016-0237-x.

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

Author information

1
Service de Pneumologie, CHU Reunion/GHSR, Saint-Pierre, F-97410, France. laurent.guilleminault@chu-reunion.fr.
2
Inserm, UMR 1188 Diabète athérothrombose Thérapies Réunion Océan Indien (DéTROI), plateforme CYROI, Sainte-Clotilde, F-97490, France. laurent.guilleminault@chu-reunion.fr.
3
Université de La Réunion, UMR 1188, Sainte-Clotilde, F-97490, France. laurent.guilleminault@chu-reunion.fr.
4
Service de Pneumologie, Groupe Hospitalier Sud Réunion, BP 350, 97448, Saint-Pierre cedex, France. laurent.guilleminault@chu-reunion.fr.
5
Service de Pneumologie, CHU Reunion/GHSR, Saint-Pierre, F-97410, France.
6
Service de maladies infectieuses, CHU Reunion/GHSR, Saint-Pierre, F-97410, France.
7
Université de La Réunion, UMR 1188, Sainte-Clotilde, F-97490, France.

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.

KEYWORDS:

Adult onset Still’s disease; Auto-inflammatory disease; Case report; Pulmonary arterial hypertension

PMID:
27160441
PMCID:
PMC4862120
DOI:
10.1186/s12890-016-0237-x
[Indexed for MEDLINE]
Free PMC Article

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