title: Pulmonary arterial hypertension in adult onset Still's disease: a case report of a severe complication.
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.
  
  
  METHODS
  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.
authors.affiliation: Service de Pneumologie, CHU Reunion/GHSR, Saint-Pierre, F-97410, France. laurent.guilleminault@chu-reunion.fr.|Inserm, UMR 1188 Diabète athérothrombose Thérapies Réunion Océan Indien (DéTROI), plateforme CYROI, Sainte-Clotilde, F-97490, France. laurent.guilleminault@chu-reunion.fr.|Université de La Réunion, UMR 1188, Sainte-Clotilde, F-97490, France. laurent.guilleminault@chu-reunion.fr.|Service de Pneumologie, Groupe Hospitalier Sud Réunion, BP 350, 97448, Saint-Pierre cedex, France. laurent.guilleminault@chu-reunion.fr.
authors.forename: L
authors.initials: L
authors.lastname: Guilleminault
authors.affiliation: Service de Pneumologie, CHU Reunion/GHSR, Saint-Pierre, F-97410, France.
authors.forename: S
authors.initials: S
authors.lastname: Laurent
authors.affiliation: Service de maladies infectieuses, CHU Reunion/GHSR, Saint-Pierre, F-97410, France.
authors.forename: A
authors.initials: A
authors.lastname: Foucher
authors.affiliation: Service de maladies infectieuses, CHU Reunion/GHSR, Saint-Pierre, F-97410, France.
authors.forename: P
authors.initials: P
authors.lastname: Poubeau
authors.affiliation: Service de Pneumologie, CHU Reunion/GHSR, Saint-Pierre, F-97410, France.|Université de La Réunion, UMR 1188, Sainte-Clotilde, F-97490, France.
authors.forename: F
authors.initials: F
authors.lastname: Paganin
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use; D018501:Antirheumatic Agents / Q000627:therapeutic use; D004452:Echocardiography; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000209:etiology; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use; D010666:Phenylpropionates / Q000627:therapeutic use; D011724:Pyridazines / Q000627:therapeutic use; D016706:Still's Disease, Adult-Onset / Q000150:complications* / Q000188:drug therapy*; D000068581:Tadalafil / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D000959:Antihypertensive Agents; D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein; D010666:Phenylpropionates; D011724:Pyridazines; D000068581:Tadalafil; C467894:ambrisentan
keywords: Adult onset Still’s disease; Auto-inflammatory disease; Case report; Pulmonary arterial hypertension