Cryptogenic fibrosing alveolitis: clinical features and their influence on survival

Turner-Warwick, M; Burrows, Benjamin; Johnson, A. L.

doi:10.1136/thx.35.3.171

Cited by 464 publications

(306 citation statements)

References 10 publications

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“…It might be possible that cases were excluded whosurvived longer but were not examined by autopsy observations. This might affect the findings of shorter MSTin this study compared with those previously reported (8)(9)(10)(11)(12)(13)(14)(15). It might be reasonable that older patients/subjects had shorter periods of survival in IPF patients and it was necessary to correct the effects of age in this study.…”

Section: Discussioncontrasting

confidence: 56%

A Clinical Study of Idiopathic Pulmonary Fibrosis Based on Autopsy Studies in Elderly Patients

et al. 2003

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Objectives The prevalence of idiopathic pulmonary fibrosis (IPF) tends to be greater in the elderly although the precise outcome is not known. The present study investigated the outcome of IPF in elderly patients whose pathological diagnosis corresponded to usual interstitial pneumonia (UIP) on autopsy findings.Methods In a consecutive autopsy series, (n=4,554 cases), complete information was obtained in 86 cases in which the clinical and pathological diagnosis was IPF and UIP, respectively.Results Mean subject age was 80.5 years and there were 52 males and 34 females. Median survival time (MST) was 3.5 years overall. Dyspnea ranking by the MRCdyspnea scale (DR) on admission was closely related to the outcome. WhenMSTwas compared between DROand DR1, or DR2 and DR3, the former patient group survived significantly longer than the latter (p<0.05). Twenty percent of patients died of severe respiratory failure. Other causes of death were disease progression of IPF (10.5%), bacterial pneumonia (23.3%) and lung cancer (17.4%).The patients in the youngelderly group (age <75) survived for a significantly longer period than those in the old-elderly group (age^75) (MST; 5.5 vs 2.0 years; p<0.001). Both lung cancer and pneumonia were significantly more prevalent among IPF patients than among non-IPF patients; these IPF with lung cancer profoundly reflected the smoking history of IPF patients.Conclusions The poor outcome in IPF in elderly patients was characterized by more severe dyspnea, advanced age and co-morbidity. (Internal Medicine 42: 483-489, 2003)

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Section: Discussioncontrasting

confidence: 56%

A Clinical Study of Idiopathic Pulmonary Fibrosis Based on Autopsy Studies in Elderly Patients

et al. 2003

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“…There was no clear association between the TWT and lung volumes in this study. However, lung volumes are inconsistently associated with survival and other outcome measures in IPF [2,4], and changes in lung volumes are insensitive to the effect of treatment in clinical trials [10,32].…”

Section: Discussionmentioning

confidence: 99%

“…diopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease (ILD) of unknown cause, resulting in severe morbidity and death due to progressive respiratory failure [1], usually within 3-5 yrs [2][3][4][5]. 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].…”

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confidence: 99%

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

et al. 2005

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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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“…Cigarette smoking appears to enhance the development of interstitial fibrosis in workers exposed to asbestos (22)(23)(24), in idiopathic pulmonary fibrosis (25,26), and in histocytosis X (27). Cigarette smoking may also alter pulmonary immune function (28-31), reduce clearance of inhaled agents (32), and increase permeability of the respiratory epithelia (33).…”

Section: Discussionmentioning

confidence: 99%

Rheumatoid arthritis lung disease. Determinants of radiographic and physiologic abnormalities

Saag

Kolluri

Koehnke

et al. 1996

Arthritis & Rheumatism

226

112

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Objective. To determine the prevalence and important clinical predictors of radiographic and physiologic abnormalities indicative of rheumatoid arthritis interstitial lung disease (RA-ILD).Methods. An unselected cohort of patients with a confirmed diagnosis of RA and known lung disease were identified (n = 336) and evaluated for RA disease activity and seventy. Outcomes included abnormalities determined by the pulmonary function tests of forced vital capacity (FVC) and diffusion capacity for carbon monoxide (DLco), and/or chest radiographic findings of interstitial infiltrates. We used multivariable statistical modeling to determine the independent significance of cigarette smoking and other RA-specific factors on the pulmonary abnormalities of interest.Results. At least 1 of the 3 abnormal findings was identified by pulmonary tests in 32.4% of all patients. These abnormal findings included an FVC <80% of predicted in 42 patients, a DLco <80% of predicted in 64 patients, and evidence of radiographic interstitial infiltrates in 40 patients. After statistical adjustment for confounding factors, pack-years of cigarette smoking remained a significant predictor of low DLco (p = Submitted for publication January 22, 1996; accepted in revised form May 2, 1996. interstitial abnormalities on chest radiograph (odds ratio for 2 2 5 pack-years = 3.76,95% CI 1.59,8.88). The Health Assessment Questionnaire (HAQ) Disability Index (DI) was also an important risk factor for the decline in both the DLco ( p = -1.15, 95% CI -2.00, -0.30) and FVC ( p = -0.23, 95% CI -032, -0.13).-

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Cryptogenic fibrosing alveolitis: clinical features and their influence on survival

Cited by 464 publications

References 10 publications

A Clinical Study of Idiopathic Pulmonary Fibrosis Based on Autopsy Studies in Elderly Patients

A Clinical Study of Idiopathic Pulmonary Fibrosis Based on Autopsy Studies in Elderly Patients

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

Rheumatoid arthritis lung disease. Determinants of radiographic and physiologic abnormalities

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