The impact of combined pulmonary fibrosis and emphysema on mortality

Ch, Lee; Kim, Hyung Jun; Park, C-M; Ky, Lim; Jy, Lee; Dj, Kim; Jh, Yeon; Ss, Hwang; Dk, Kim; Lee, SM; Yim, Jae Joon; Sc, Yang; Yoo, Christopher H.; Chung, Hee Soon; Yw, Kim; Han, Shiyu; Ys, Shim

doi:10.5588/ijtld.10.0491

Cited by 41 publications

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“…11 Among these patients, high mean pulmonary arterial pressure, high pulmonary vascular resistance, high heart rate and low diffusing capacity for carbon monoxide (DLco) were significantly associated with poor outcome. In one study, CPFE patients had a fivefold higher mortality risk (adjusted HR 5.10, 95% CI 1.75 to 14.9) in non-malignant situations 19. In the present study, only 12 of 93 patients had lung cancer in contrast to the number reported in a previous study 10.…”

Section: Discussioncontrasting

confidence: 77%

A cohort study of mortality predictors and characteristics of patients with combined pulmonary fibrosis and emphysema

Kishaba

Shimaoka²,

Fukuyama³

et al. 2012

BMJ Open

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ObjectivesOur purpose was to assess the clinical data, predictors of mortality and acute exacerbation (AE) in combined pulmonary fibrosis and emphysema (CPFE) patients.DesignSingle-centre retrospective cohort study.SettingTeaching hospital in Japan.ParticipantsWe identified 93 CPFE patients with high-resolution computed tomographic (HRCT) through multidisciplinary discussion. Patients who had connective tissue disease, drug-associated interstitial lung disease and occupationally related interstitial lung disease, such as asbestosis and silicosis, were excluded.InterventionsThere were no interventions.MethodsMedical records and HRCT scans from January 2002 through December 2007 were reviewed retrospectively at our hospital. Ninety-three patients had CPFE.ResultsThe mean age of CPFE patients was 74 years. Idiopathic pulmonary fibrosis and non-specific interstitial pneumonia were observed as distinct HRCT patterns. Forty-two patients showed finger clubbing. Mean serum Krebs von den Lungen-6 (KL-6) and per cent predicted forced vital capacity (%FVC) were 1089 IU/l, 63.86%, respectively. Twenty-two patients developed AE during observation period. Baseline KL-6 was a strong predictor of AE (OR=1.0016, p=0.009). Finger clubbing (HR=2.2620, p=0.015) and per cent predicted forced expiratory volume in one second/%FVC more than 1.2 (HR=1.9259, p=0.048) were independent predictors of mortality in CPFE.ConclusionsBaseline serum KL-6 was a useful predictor of AE (cut-off =1050, receiver operator characteristic curve: 0.7720), which occurred in 24% (22/93) of the CPFE patients. Finger clubbing and per cent predicted forced expiratory volume in one second/%FVC more than 1.2 were independent predictors of mortality.

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Section: Discussioncontrasting

confidence: 77%

A cohort study of mortality predictors and characteristics of patients with combined pulmonary fibrosis and emphysema

Kishaba

Shimaoka²,

Fukuyama³

et al. 2012

BMJ Open

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“…As they were found in our SSc patients at the time of diagnosis, interstitial fibrosis with diffuse pulmonary cystic lesions may be a not so uncommon manifestation of interstitial lung disease that requires specific investigations in order to exclude infections and cancer . Our cases strengthen the hypothesis of Cottin et al .…”

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confidence: 87%

Combined pulmonary fibrosis and emphysema syndrome in systemic sclerosis

2012

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“…CPFE is characterized by a relatively well-preserved lung volume, severely impaired carbon monoxide transfer, significant decrease in PaO 2 on exercise, and a high prevalence of pulmonary hypertension leading to a poor prognosis [3, 4]. We expect that the extent of pulmonary fibrosis in CPFE patients might influence the outcome, because patients with CPFE have a worse prognosis than patients with emphysema [5]. However, it is difficult to evaluate the extent of pulmonary fibrosis in patients with CPFE.…”

Section: Introductionmentioning

confidence: 99%

The Diagnostic Value of the Interstitial Biomarkers KL-6 and SP-D for the Degree of Fibrosis in Combined Pulmonary Fibrosis and Emphysema

et al. 2012

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The combined pulmonary fibrosis and emphysema (CPFE) was reported first in 1990, but it has been comparatively underestimated until recently. Although the diagnostic findings of both emphysematous and fibrotic regions are detectable by high-resolution computed tomography (HRCT) of the chest, the degree of progressive fibrosis, which increases with emphysematous lesions, is difficult to evaluate. In this study, we hypothesized that the biomarkers for pulmonary fibrosis, surfactant protein D (SP-D), and KL-6 would serve as good indicators of fibrotic lesions in CPFE. We recruited 46 patients who had been diagnosed in our hospital with both emphysema and fibrosis by their CT scan image from April 2003 to March 2008. The correlation among their pulmonary function tests, composite physiologic index (CPI), and the serum levels of SP-D and KL-6 was evaluated. We found a correlation between KL-6 and %VC, %TLC, or CPI and between SP-D and %VC or CPI. Interestingly, the combined product of KL-6 and SP-D (KL-6xSP-D) was found to highly correlate with %VC and %TLC or CPI. These results show that both KL-6 and SP-D, and especially the product of SP-D and KL-6, are good indicators of the presence of fibrotic lesions in the lungs of CPFE patients.

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The impact of combined pulmonary fibrosis and emphysema on mortality

Abstract: CPFE is not rare and CPFE patients had a higher overall mortality risk than emphysema-only patients.

Cited by 41 publications

References 0 publications

A cohort study of mortality predictors and characteristics of patients with combined pulmonary fibrosis and emphysema

A cohort study of mortality predictors and characteristics of patients with combined pulmonary fibrosis and emphysema

Combined pulmonary fibrosis and emphysema syndrome in systemic sclerosis

The Diagnostic Value of the Interstitial Biomarkers KL-6 and SP-D for the Degree of Fibrosis in Combined Pulmonary Fibrosis and Emphysema

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