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

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


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.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Antihypertensive Agents / therapeutic use
  • Antirheumatic Agents / therapeutic use
  • Echocardiography
  • Female
  • Humans
  • Hypertension, Pulmonary / diagnostic imaging*
  • Hypertension, Pulmonary / etiology
  • Interleukin 1 Receptor Antagonist Protein / therapeutic use
  • Phenylpropionates / therapeutic use
  • Pyridazines / therapeutic use
  • Still's Disease, Adult-Onset / complications*
  • Still's Disease, Adult-Onset / drug therapy*
  • Tadalafil / therapeutic use
  • Tomography, X-Ray Computed
  • Young Adult


  • Adrenal Cortex Hormones
  • Antihypertensive Agents
  • Antirheumatic Agents
  • Interleukin 1 Receptor Antagonist Protein
  • Phenylpropionates
  • Pyridazines
  • Tadalafil
  • ambrisentan