POEMS syndrome-related pulmonary hypertension is steroid-responsive

Respir Med. 2007 Feb;101(2):353-5. doi: 10.1016/j.rmed.2006.04.026. Epub 2006 Jun 13.


Background: The POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal immunoglobulin, Skin changes) syndrome is a rare disease that entails a specific risk for pulmonary arterial hypertension.

Methods and results: We report on 2 patients who suffered from POEMS syndrome and pulmonary arterial hypertension for whom a detailed hemodynamic pulmonary study was obtained before and after steroid treatment. Patient 1 had defined post-capillary pulmonary hypertension (PH) ascribed to an abnormally high cardiac output. Patient 2 suffered from severe pre-capillary PH. Under high-dose steroids treatment, clinical symptoms disappeared and PH assessment by sequential hemodynamic study showed a clear-cut improvement in both patients.

Conclusion: First-line therapy should include corticosteroids in POEMS syndrome-related PH.

Publication types

  • Case Reports

MeSH terms

  • Female
  • Glucocorticoids / therapeutic use*
  • Humans
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / physiopathology
  • Male
  • Middle Aged
  • POEMS Syndrome / complications
  • POEMS Syndrome / physiopathology*
  • Prednisone / therapeutic use*
  • Treatment Outcome


  • Glucocorticoids
  • Prednisone