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POEMS syndrome-related pulmonary hypertension is steroid-responsive
Pascale Jouve1, Marc Humbert, Marie-Paule Chauveheid
1Service de Médecine Interne, Hôpital Bichat, 46 rue Henri Huchard, 75877 Paris cedex 18, France.
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.
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