Chest
Original ResearchInterstitial Lung DiseaseIdiopathic Pulmonary Fibrosis and Emphysema: Decreased Survival Associated With Severe Pulmonary Arterial Hypertension
Section snippets
Materials and Methods
We evaluated the clinical records of a cohort of consecutive IPF patients at the National Institute of Respiratory Diseases (INER), Mexico, from 1996 through 2006. The diagnosis of IPF was made based on established criteria and was confirmed by lung biopsy in 38% of the subjects.10 All patients in whom IPF had been diagnosed before the year 2000 were reevaluated to confirm that they met the American Thoracic Society/European Respiratory Society consensus guidelines.10 Clinical data (ie, smoking
Results
We evaluated 198 patients with a diagnosis of IPF. From them, 113 patients had available the chest HRCT scan corresponding to the first medical evaluation; 3 patients were excluded from the study because they had HRCT scan findings that were nontypical for IPF and had not undergone a biopsy. Seventy-two percent of the 110 patients were men (mean [± SD] age, 64 ± 9.5 years), and 56% had a history of cigarette smoking. Male gender and smoking history were highly associated, as follows: 56 smokers
Discussion
A growing body of evidence suggests that IPF evolves with different clinical phenotypes. Thus, for example, in 2007 we described5 an accelerated variant of the disease in which patients consulted a physician a few months after the beginning of symptoms with severe physiologic impairment and showing a significantly lower survival rate. Also, it is well known that the disease occurs more frequently in smokers9, 17, 18 and that emphysematous changes develop in a number of these patients. In this
Acknowledgment
The authors thank Dr. Fortunato Juárez for her expert evaluation of the HRCT scans in the concordance analysis.
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This study was supported by Universidad Nacional Autónoma de México, grant No. SDI.PTID.05.6.
The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.
Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).