Could pulmonary arterial hypertension be an active index of systemic lupus erythematosus? A successful case of SLE-PAH cured by methylprednisolone pulse therapy

Lupus. 2014 Dec;23(14):1533-6. doi: 10.1177/0961203314552461. Epub 2014 Sep 26.


A 35-year-old female patient was diagnosed with pulmonary arterial hypertension (PAH) by right heart catheterization (RHC) in the 3rd month of systemic lupus erythematosus (SLE) duration with high disease activity. Through methylprednisolone pulse therapy, she got total recovery from PAH proven by repeat catheterization and follow-ups. This is the first simultaneously diagnosed, totally recovered, gold standard-proved SLE-PAH case ever reported in the literature. We would like to share this successful case, and at the same time, we want to highlight the relationship between presentation of PAH and active disease manifestations of SLE patients, and to discuss how immune suppressive treatment could benefit this subset of patients.

Keywords: Pulmonary arterial hypertension; corticosteroid treatment; methylprednisolone pulse therapy; systemic lupus erythematosus.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anti-Inflammatory Agents / therapeutic use
  • Arterial Pressure
  • Female
  • Humans
  • Hypertension, Pulmonary / complications
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / physiopathology*
  • Lupus Erythematosus, Systemic / complications
  • Lupus Erythematosus, Systemic / drug therapy*
  • Methylprednisolone / therapeutic use
  • Severity of Illness Index


  • Anti-Inflammatory Agents
  • Methylprednisolone