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

Research output: Contribution to journalArticle

72 Citations (Scopus)

Abstract

Background: 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. Methods: 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 χ2 test or Fisher exact test, where appropriate. Results: 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 silhouette were seen in 28% of patients. When separated by the presence or absence of respiratory muscle weakness, the median survival time was 87 vs 139 months, respectively (p < 0.05). The presence of cough was associated with reduced survival time. Conclusions: Pulmonary manifestations of POEMS are common, and both symptomatic and asymptomatic respiratory involvements are frequent on presentation in patients with POEMS syndrome. Respiratory muscle weakness and cough portend a poorer prognosis. These results suggest the need for increased awareness of the association between POEMS syndrome and pulmonary disease to guide appropriate screening and supportive therapy.

Original languageEnglish (US)
Pages (from-to)969-974
Number of pages6
JournalChest
Volume133
Issue number4
DOIs
StatePublished - Apr 2008

Fingerprint

POEMS Syndrome
Lung
Respiratory Muscles
Muscle Weakness
Survival
Cough
Pulmonary Hypertension
Lung Diseases
Paraproteinemias
Respiratory Function Tests
Kaplan-Meier Estimate
Pleural Effusion
Survival Analysis
Diaphragm
Plasma Cells
Signs and Symptoms
Echocardiography
Demography
Skin

Keywords

  • Lung disease
  • Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin manifestations (POEMS) syndrome
  • Pulmonary hypertension
  • Respiratory muscle weakness

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Pulmonary manifestations in patients with POEMS syndrome : A retrospective review of 137 patients. / Allam, Joanne Shirine; Kennedy, Cassie; Aksamit, Timothy; Dispenzieri, Angela.

In: Chest, Vol. 133, No. 4, 04.2008, p. 969-974.

Research output: Contribution to journalArticle

@article{4a531f1b27e4442c8b2fef397827dc22,
title = "Pulmonary manifestations in patients with POEMS syndrome: A retrospective review of 137 patients",
abstract = "Background: 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. Methods: 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 χ2 test or Fisher exact test, where appropriate. Results: 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 silhouette were seen in 28{\%} of patients. When separated by the presence or absence of respiratory muscle weakness, the median survival time was 87 vs 139 months, respectively (p < 0.05). The presence of cough was associated with reduced survival time. Conclusions: Pulmonary manifestations of POEMS are common, and both symptomatic and asymptomatic respiratory involvements are frequent on presentation in patients with POEMS syndrome. Respiratory muscle weakness and cough portend a poorer prognosis. These results suggest the need for increased awareness of the association between POEMS syndrome and pulmonary disease to guide appropriate screening and supportive therapy.",
keywords = "Lung disease, Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin manifestations (POEMS) syndrome, Pulmonary hypertension, Respiratory muscle weakness",
author = "Allam, {Joanne Shirine} and Cassie Kennedy and Timothy Aksamit and Angela Dispenzieri",
year = "2008",
month = "4",
doi = "10.1378/chest.07-1800",
language = "English (US)",
volume = "133",
pages = "969--974",
journal = "Chest",
issn = "0012-3692",
publisher = "American College of Chest Physicians",
number = "4",

}

TY - JOUR

T1 - Pulmonary manifestations in patients with POEMS syndrome

T2 - A retrospective review of 137 patients

AU - Allam, Joanne Shirine

AU - Kennedy, Cassie

AU - Aksamit, Timothy

AU - Dispenzieri, Angela

PY - 2008/4

Y1 - 2008/4

N2 - Background: 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. Methods: 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 χ2 test or Fisher exact test, where appropriate. Results: 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 silhouette were seen in 28% of patients. When separated by the presence or absence of respiratory muscle weakness, the median survival time was 87 vs 139 months, respectively (p < 0.05). The presence of cough was associated with reduced survival time. Conclusions: Pulmonary manifestations of POEMS are common, and both symptomatic and asymptomatic respiratory involvements are frequent on presentation in patients with POEMS syndrome. Respiratory muscle weakness and cough portend a poorer prognosis. These results suggest the need for increased awareness of the association between POEMS syndrome and pulmonary disease to guide appropriate screening and supportive therapy.

AB - Background: 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. Methods: 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 χ2 test or Fisher exact test, where appropriate. Results: 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 silhouette were seen in 28% of patients. When separated by the presence or absence of respiratory muscle weakness, the median survival time was 87 vs 139 months, respectively (p < 0.05). The presence of cough was associated with reduced survival time. Conclusions: Pulmonary manifestations of POEMS are common, and both symptomatic and asymptomatic respiratory involvements are frequent on presentation in patients with POEMS syndrome. Respiratory muscle weakness and cough portend a poorer prognosis. These results suggest the need for increased awareness of the association between POEMS syndrome and pulmonary disease to guide appropriate screening and supportive therapy.

KW - Lung disease

KW - Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin manifestations (POEMS) syndrome

KW - Pulmonary hypertension

KW - Respiratory muscle weakness

UR - http://www.scopus.com/inward/record.url?scp=42149156009&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=42149156009&partnerID=8YFLogxK

U2 - 10.1378/chest.07-1800

DO - 10.1378/chest.07-1800

M3 - Article

C2 - 18198255

AN - SCOPUS:42149156009

VL - 133

SP - 969

EP - 974

JO - Chest

JF - Chest

SN - 0012-3692

IS - 4

ER -