POEMS syndrome-related pulmonary hypertension is steroid-responsive.

Abstract

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.

• Authors: Pascale Jouve, Marc Humbert, Marie-Paule Chauveheid, Xavier Jaïs, Thomas Papo
• Journal: Respiratory medicine (Respir Med) Vol. 101 Issue 2 Pg. 353-5 (Feb 2007) ISSN: 0954-6111 [Print] England
• PMID: 16774822 (Publication Type: Case Reports, Journal Article)

Chemical References

• Glucocorticoids
• Prednisone

Topics

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