0
Original Research: POEMS SYNDROME |

Pulmonary Manifestations in Patients With POEMS Syndrome*: A Retrospective Review of 137 Patients

Joanne Shirine Allam, MD; Cassie C. Kennedy, MD; Timothy R. Aksamit, MD, FCCP; Angela Dispenzieri, MD
Author and Funding Information

Affiliations: *From the Divisions of Pulmonary and Critical Care Medicine (Drs. Allam, Kennedy, and Aksamit), and Hematology (Dr. Dispenzieri), Mayo Clinic College of Medicine, Rochester, MN.,  These authors made equal contributions as first author.

Correspondence to: Angela Dispenzieri, MD, Division of Hematology, Mayo Clinic College of Medicine, 200 First St SW, Rochester, MN 55905; e-mail: dispenzieri.angela@mayo.edu



Chest. 2008;133(4):969-974. doi:10.1378/chest.07-1800
Text Size: A A A
Published online

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 23% 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.

Figures in this Article

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Figures

Tables

References

NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
Guidelines
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543