Open AccessEffectiveness of pulmonary vasodilators on pulmonary hypertension associated with POEMS syndrome
Author(s) -
Ohashi Kana,
Nishimura Rintaro,
Sugimoto Shunsuke,
Sakao Seiichiro,
Tanabe Nobuhiro,
Tatsumi Koichiro
Publication year - 2019
Publication title -
respirology case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.304
H-Index - 9
ISSN - 2051-3380
DOI - 10.1002/rcr2.411
Subject(s) - poems syndrome , medicine , organomegaly , pulmonary hypertension , polyneuropathy , sildenafil , pulmonary artery , cardiology
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare plasma cell disease. Patients with POEMS syndrome are considered to be at a high risk of developing pulmonary hypertension (PH). We report a 51‐year‐old woman diagnosed with PH associated with POEMS syndrome. She was started on dexamethasone and thalidomide. Although, the plasma vascular endothelial growth factor (VEGF) level decreased, systolic pulmonary artery pressure (sPAP) remained high. Auto‐peripheral blood stem cell transplantation improved the plasma VEGF and sPAP levels. Four years later, she presented with dyspnoea on exertion, and elevated plasma VEGF and sPAP levels. Subsequently, on administering sildenafil and macitentan, the plasma VEGF and PH levels improved. Pulmonary vasodilators can be considered when PH remains after treatment of POEMS syndrome.