POEMS syndrome-related pulmonary hypertension is steroid-responsive.
BACKGROUND
The POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal immunoglobulin, Skin changes) syndrome is a rare disease that entails a specific risk for pulmonary arterial hypertension.


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.


CONCLUSIONS
First-line therapy should include corticosteroids in POEMS syndrome-related PH.
Jouve
Pascale
P
Service de Médecine Interne, Hôpital Bichat, 46 rue Henri Huchard, 75877 Paris cedex 18, France.
Humbert
Marc
M
Chauveheid
Marie-Paule
MP
Jaïs
Xavier
X
Papo
Thomas
T
D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000150:complications / Q000503:physiopathology*; D011241:Prednisone / Q000627:therapeutic use*; D016896:Treatment Outcome
D005938:Glucocorticoids; D011241:Prednisone