Abstract: Objective To describe the clinical characteristics of POEMS syndrome associated with pulmonary arterial hypertension. Methods Medical records of patients with POEMS syndrome who were identified in Peking Union Medical College Hospital were reviewed. The clinical manifestations of patients complicated with PAH (pulmonary arterial hypertension, PAH) diagnosed by echocardiography (uCG) was compared with those without PAH and those without echocradiography results. Results One hundred and seventeen patients with POEMS syndrome were recruited. Forty-nine patients have PAH，thirty-three patients have no PAH and thirty-five patients didn’t undergo echocradiography. The prevalence of PAH was 41.9%(49/117). Chest distress is the main manifestation in group of POEMS syndrome with PAH, and the frequency is 42.9%（21/49），higher than the other two groups. These patients with PAH are more likely to have pleural effusion, ascites, or pericardial effusion. Conclusion The prevalence of PAH in POEMS syndrome patients was more than 40%, and over half of them don’t have any symptoms like chest distress. We suggest patients with POEMS syndrome should have echocradiography to screen PAH.

Key words: POEMS syndrome, Pulmonary arterial hypertension, echocardiography