Clinical and functional assessment of patients with idiopathic pulmonary fibrosis: results of a 3 year follow-up

The purpose of this study was to analyse the information provided by different techniques used in the assessment of patients with idiopathic pulmonary fibrosis (IPF) and their role in the prediction of lung function decline with the decline. Twenty seven subjects with IPF (55 +/- 14 (mean +/- SD) yrs) were studied at the initial staging. Nineteen of them (70%) were included in a follow-up over 3 yrs (32 +/- 6 months), whilst the remaining 8 patients were lost to follow-up. During the period of the study, 6 of the 19 patients died. A significant correlation between diffusing capacity of the lungs for carbon monoxide (DLCO) (and carbon monoxide transfer coefficient (KCO) = DLCO/alveolar volume (VA)) and the increase in alveolar-arterial oxygen tension difference (A-aPO2) during exercise (delta A-aPO2) was observed at diagnosis (r = -0.58). Despite the treatment with prednisone (1 mg.kg-1 daily during 4 weeks, tapered to an individualized maintenance daily dose of 15-30 mg), the 13 patients controlled throughout the whole period of the study showed a marked impairment in lung volumes; forced vital capacity (FVC) -0.46 +/- 0.09 l, from 69 +/- 16 to 52 +/- 11% of predicted, and total lung capacity (TLC) -0.39 +/- 0.11 l, from 75 +/- 16 to 62 +/- 14%, and in DLCO -0.6 +/- 0.2 mmol.min-1.kPa-1, from 56 +/- 15 to 47 +/- 18%, predicted. By contrast, both mean arterial oxygen tension (PaO2) and A-aPO2 at rest remained unchanged throughout the 3 yrs follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

This article has been cited by other articles:

				M. Molina-Molina, A. Xaubet, X. Li, A. Abdul-Hafez, K. Friderici, K. Jernigan, W. Fu, Q. Ding, J. Pereda, A. Serrano-Mollar, et al. Angiotensinogen gene G-6A polymorphism influences idiopathic pulmonary fibrosis disease progression Eur. Respir. J., October 1, 2008; 32(4): 1004 - 1008. [Abstract] [Full Text] [PDF]

				A U Wells, N Hirani, and on behalf of the BTS Interstitial Lung Disease Gui Interstitial lung disease guideline Thorax, September 1, 2008; 63(Suppl_5): v1 - v58. [Full Text] [PDF]

				F. J. Martinez and K. Flaherty Pulmonary function testing in idiopathic interstitial pneumonias. Proceedings of the ATS, January 1, 2006; 3(4): 315 - 321. [Abstract] [Full Text] [PDF]

				V. N. Lama, K. R. Flaherty, G. B. Toews, T. V. Colby, W. D. Travis, Q. Long, S. Murray, E. A. Kazerooni, B. H. Gross, J. P. Lynch III, et al. Prognostic Value of Desaturation during a 6-Minute Walk Test in Idiopathic Interstitial Pneumonia Am. J. Respir. Crit. Care Med., November 1, 2003; 168(9): 1084 - 1090. [Abstract] [Full Text] [PDF]

				A. Xaubet, A. Marin-Arguedas, S. Lario, J. Ancochea, F. Morell, J. Ruiz-Manzano, E. Rodriguez-Becerra, J. M. Rodriguez-Arias, P. Inigo, S. Sanz, et al. Transforming Growth Factor-{beta}1 Gene Polymorphisms Are Associated with Disease Progression in Idiopathic Pulmonary Fibrosis Am. J. Respir. Crit. Care Med., August 15, 2003; 168(4): 431 - 435. [Abstract] [Full Text] [PDF]

				Y C G Lee, B Singh, S C Pang, N H de Klerk, D R Hillman, and A W Musk Radiographic (ILO) readings predict arterial oxygen desaturation during exercise in subjects with asbestosis Occup. Environ. Med., March 1, 2003; 60(3): 201 - 206. [Abstract] [Full Text] [PDF]

				ATS/ACCP Statement on Cardiopulmonary Exercise Testing Am. J. Respir. Crit. Care Med., January 15, 2003; 167(2): 211 - 277. [Full Text] [PDF]

				A. XAUBET, C. AGUSTI, P. LUBURICH, J. ROCA, C. MONTON, M. C. AYUSO, J. A. BARBERA, and R. RODRIGUEZ-ROISIN Pulmonary Function Tests and CT Scan in the Management of Idiopathic Pulmonary Fibrosis Am. J. Respir. Crit. Care Med., August 1, 1998; 158(2): 431 - 436. [Abstract] [Full Text] [PDF]

				Corrigenda for vol. 155, p. 1367 Am. J. Respir. Crit. Care Med., July 1, 1997; 156(2): 676 - 677. [Full Text]

				B. T. SOCIETY and S. O. C. COMMITTEE The Diagnosis, Assessment and Treatment of Diffuse Parenchymal Lung Disease in Adults---British Thoracic Society recommendations Thorax, April 1, 1990; 54(90001): 1S - 28. [Full Text]