Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients

Chest
Joanne Shirine AllamAngela Dispenzieri

Abstract

POEMS syndrome is a monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Rare reports of pulmonary manifestations of POEMS syndrome such as pulmonary hypertension exist; however, a comprehensive review of the pulmonary manifestations of POEMS syndrome is lacking. Retrospective review of patients with a diagnosis of POEMS syndrome at our institution between 1975 and 2003. Demographics, signs and symptoms, test findings (ie, radiographs, pulmonary function tests, and echocardiography), and survival data were extracted. Kaplan-Meier survival analysis was performed. In addition, categoric variables were compared using the Pearson chi(2) test or Fisher exact test, where appropriate. The study comprised 137 patients (66% male) with a mean age of 51.6 years. Respiratory symptoms were common within 2 years of diagnosis (28%). The median overall survival time was 147 months. Pulmonary manifestations of POEMS syndrome included pulmonary hypertension, restrictive lung disease, respiratory muscle weakness, and an isolated diminished diffusing capacity. Significant radiographic findings such as pleural effusions, diaphragm elevation, and increased cardiac sil...Continue Reading

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