Prognosis of cryptogenic fibrosing alveolitis.

Tukiainen, P; Taskinen, Eero; Holsti, Paul; Korhola, O; Valle, M

doi:10.1136/thx.38.5.349

Cited by 137 publications

(98 citation statements)

References 13 publications

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“…The prognostic role of DLCO in pulmonary fibrosis had been previously suggested by TUKIANEN et al [17]. However, these investigators included patients with associated connective diseases, and there is increasing evidence that the natural history of pulmonary fibrosis…”

Section: Discussionmentioning

confidence: 99%

“…The lack of a predictive role was also observed for the BAL differential cell count. Discrepancies among studies using gallium-67 lung scanning and BAL fluid differential cell count can be explained by different factors [5,6,8,[15][16][17]. Frequently, different types of interstitial lung diseases have been analysed together.…”

Section: Follow-up Studymentioning

confidence: 99%

“…To this end, we have prospectively studied a homogeneous population of 27 patients with lone IPF; 19 of them being followed for 3 yrs after diagnosis, a period of time longer than that used in most of similar studies [5,6,8,[15][16][17]. Previous studies by our group [18] prompted the hypothesis that the assessment of lung volumes alone may not be sufficient for an adequate clinical evaluation of this type of patients.…”

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confidence: 99%

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

Agustı́¹,

Xaubet²,

Agustí³

et al. 1994

Eur Respir J

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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)

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Section: Discussionmentioning

confidence: 99%

Section: Follow-up Studymentioning

confidence: 99%

mentioning

confidence: 99%

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

Agustı́¹,

Xaubet²,

Agustí³

et al. 1994

Eur Respir J

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“…Prognostic factors that have been variably associated with survival include age [6], smoking status [7], sex [8], resting pulmonary function [9], histopathology score [7], fibrotic score based on high-resolution computed tomography [10,11], and initial response to treatment with corticosteroids [10]. A composite score of clinical, radiographical and physiological variables has been associated with survival in IPF [12].…”

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confidence: 99%

The timed walk test as a measure of severity and survival in idiopathic pulmonary fibrosis

Hallstrand

Boitano

Johnson

et al. 2005

European Respiratory Journal

163

112

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Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive disease with a median survival of ,3 yrs. Measurements of airflow and lung volumes at rest are generally used to monitor the clinical course in this disorder. This study was designed to determine if a modified version of the 6-min walk test, called the timed walk test, accurately characterises disease severity and survival in IPF.The study population consisted of 28 patients with well-characterised progressive IPF. The timed walk test and concurrent measures of disease severity were assessed at baseline. Participants were prospectively followed for o4 yrs to determine the relationship between parameters of the timed walk test and survival.There were strong correlations between the end-exercise saturation and walk-velocity parameters of the timed walk test and diffusing capacity, and arterial oxygen tension at rest. In univariate Cox proportional-hazards models, end-exercise saturation, change in saturation with exercise, walk distance and walk velocity were associated with survival. In unadjusted logistic regression models, odds of death at 2 yrs were associated with the same parameters.In conclusion, the timed walk test relates to disease severity and long-term outcome in progressive idiopathic pulmonary fibrosis.

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“…A bronchoalveolar lavage neutrophilia and/or eosinophilia has been advocated by some as a marker of progressive disease [11], but is insufficiently consistent to be applied with confidence in routine clinical practice. In a number of studies of outcome, a wide variety of functional indices have been predictive of mortality, including lung volumes, arterial gases and measures of gas exchange [1,3,12]. However, the derivation of markers of disease progression from survival data suffers from one important drawback; mortality is heavily influenced by the baseline severity of disease as well as by the rate of progression.…”

mentioning

confidence: 99%

Prediction of disease progression in idiopathic pulmonary fibrosis

Wells¹,

Bois²

1994

Eur Respir J

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Prognosis of cryptogenic fibrosing alveolitis.

Abstract: We have analysed retrospectively 100 consecutive patients with cryptogenic fibrosing alveolitis, who were treated with corticosteroids and followed for at least three years. At

Cited by 137 publications

References 13 publications

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

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

The timed walk test as a measure of severity and survival in idiopathic pulmonary fibrosis

Prediction of disease progression in idiopathic pulmonary fibrosis

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