Document Detail

Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension.
MedLine Citation:
PMID:  19225068     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: It has been suggested that the presence of emphysema modifies the outcome of patients with idiopathic pulmonary fibrosis (IPF). In this article we compare clinical features, smoking history, pulmonary function, estimated systolic pulmonary artery pressure (eSPAP), and mortality in IPF with emphysema vs IPF without emphysematous changes. METHODS: A cohort of 110 IPF patients was evaluated. Clinical data were collected from clinical charts. High-resolution CT (HRCT) scans were examined by an expert blinded to clinical data, and patients were classified into the following two groups: patients with IPF with emphysema; and patients with IPF without emphysema. The Kaplan-Meier method, log-rank test, and Cox regression model were used for statistical analyses. RESULTS: The prevalence of emphysema in the IPF cohort was 28% (31 of 110 patients). IPF with emphysema was significantly associated with male gender (odds ratio [OR], 18; 95% confidence interval [CI], 2.7 to 773.7; p = 0.0003), and smoking (OR, 3.8; 95% CI, 1.36 to 11.6; p = 0.004). Patients with IPF and emphysema had a higher mean (+/- SD) decrease in oxygen saturation during rest and exercise (16.3 +/- 6.7% vs 13.5 +/- 4.6%, respectively; p = 0.04), a higher mean fibrosis HRCT scan score (1.75 +/- 0.36 vs 1.55 +/- 0.38, respectively; p = 0.015), a higher eSPAP (82 +/- 20 vs 57 +/- 15 mm Hg, respectively; p < 0.0001), and lower median survival time (25 vs 34 months, respectively; p = 0.01) than patients with IPF without emphysema. The Cox regression model showed that the two most important variables associated with mortality were FVC < 50% predicted (hazard ratio [HR], 2.6; 95% CI, 1.19 to 5.68; p = 0.016) and eSPAP >or= 75 mm Hg (HR, 2.25; 95% CI, 1.12 to 4.54; p = 0.022). CONCLUSIONS: IPF patients with emphysema exhibited higher mortality compared with those with IPF without emphysema. This dire prognosis seems to be at least partially associated with the development of severe pulmonary arterial hypertension.
Mayra Mejía; Guillermo Carrillo; Jorge Rojas-Serrano; Andrea Estrada; Teresa Suárez; Delfino Alonso; Emilio Barrientos; Miguel Gaxiola; Carmen Navarro; Moisés Selman
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2009-02-18
Journal Detail:
Title:  Chest     Volume:  136     ISSN:  1931-3543     ISO Abbreviation:  Chest     Publication Date:  2009 Jul 
Date Detail:
Created Date:  2009-07-08     Completed Date:  2009-08-05     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0231335     Medline TA:  Chest     Country:  United States    
Other Details:
Languages:  eng     Pagination:  10-5     Citation Subset:  AIM; IM    
Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico.
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MeSH Terms
Case-Control Studies
Cohort Studies
Hypertension, Pulmonary / complications*,  mortality,  physiopathology
Idiopathic Pulmonary Fibrosis / complications*,  mortality*,  physiopathology
Middle Aged
Pulmonary Artery
Pulmonary Emphysema / epidemiology*,  mortality*,  physiopathology
Pulmonary Ventilation
Risk Factors
Survival Rate
Comment In:
Chest. 2009 Jul;136(1):1-2   [PMID:  19584202 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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