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Original Research: Interstitial Lung DiseaseSignificance of Pulmonary Arterial Pressure and Diffusion Capacity of the Lung as Prognosticator in Patients With Idiopathic Pulmonary Fibrosis
Section snippets
Study Population
Seventy-eight patients with IPF (67 men, 11 women; diagnosis by pathology, n = 59; clinical diagnosis, n = 19) underwent RHC and PFT at their initial workup under normal conditions while breathing room air. None of the patients were receiving corticosteroids or immunosuppressive agents at the time of the initial workup. Three patients who died within a month of open-lung biopsy were excluded from the longitudinal study because the biopsy surgery was judged to have significantly influenced the
Initial Workup
Data on 70 patients were available for analysis of initial workups. Among them, PAH (mPAP > 25 mm Hg) was detected in six patients (8.1%). The mPAP was significantly correlated with both Pao2and %Dlco(r= − 0.470, p < 0.0001, andr= − 0.460, p < 0.001, respectively) [Table 1;Fig 1].
Five-Year Survival
In addition to the three patients who died within a month after open-lung biopsy and the eight patients with diseases other than IPF, six patients were unavailable during the follow-up period. This left data on 61
Discussion
The main focus in this study was to evaluate the influence of elevated PAP on the prognosis of IPF. Among the 70 patients who underwent RHC during their initial workup, the prevalence of PAH based on standard criterion (mPAP > 25 mm Hg) was 8.1% (6 patients). Lettieri et al26reported a markedly higher prevalence of 31.6% among IPF patients who underwent the evaluation while waiting for lung transplantation. In estimations of PAP by Nadrous et al25using the USCG method, systolic PAP was > 50 mm
Acknowledgments
The authors thank their colleagues from the Division of Respiratory Medicine of Kyoto University Hospital for cooperating and collecting patient data, especially Dr. Michio Hayashi, Kobe Central Hospital, who helped with RHC. We also thank Mr. Simon Johnson for working with the language of this article.
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This work was performed at Kyoto University. Original data from this study were presented at the 100th International Conference of the American Thoracic Society, San Diego, CA, May 22, 2005. This study was funded by a grant from the Ministry of Science and Education in Japan.