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.
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: E D Austin; M T Rock; C A Mosse; C L Vnencak-Jones; S M Yoder; I M Robbins; J E Loyd; B O Meyrick Journal: Respir Med Date: 2009-10-31 Impact factor: 3.415
Authors: Melanie J Brewis; Alistair C Church; Andrew J Peacock; Stephen Thomson; Jane Tighe; Martin K Johnson Journal: Pulm Circ Date: 2014-12 Impact factor: 3.017
Authors: Laura C Price; Dongmin Shao; Chao Meng; Frederic Perros; Benjamin E Garfield; Jie Zhu; David Montani; Peter Dorfmuller; Marc Humbert; Ian M Adcock; Stephen J Wort Journal: Respir Res Date: 2015-09-18