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.