Progressive fibrosing interstitial lung disease: prevalence and clinical outcome

Kwon, Byoung Soo; Choe, Jooae; Chae, Eun Jin; Hwang, Hyeon-Shik; Song, Jin Woo

doi:10.1186/s12931-021-01879-6

Cited by 38 publications

(46 citation statements)

References 34 publications

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“…33 Other real-world retrospective studies also reported the prevalence and clinical significance of a progressive phenotype. [34][35][36][37] Our data showed a prevalence of FILD with a progressive phenotype consistent with that predicted by a questionnaire survey, 38 and showed that patients with non-IPF FILD and clinically progressive disease have a poor prognosis. Moreover, we show that patients with non-IPF FILD and a progressive phenotype have a prognosis similar to those with IPF.…”

Section: Discussionsupporting

confidence: 85%

“…Moreover, in a recent study including 168 non‐IPF patients who had a progressive phenotype without antifibrotic agents in a real‐world setting, relative FVC decline of ≥10% in 24 months was a poor prognostic factor in patients with non‐IPF FILD 33 . Other real‐world retrospective studies also reported the prevalence and clinical significance of a progressive phenotype 34–37 . Our data showed a prevalence of FILD with a progressive phenotype consistent with that predicted by a questionnaire survey, 38 and showed that patients with non‐IPF FILD and clinically progressive disease have a poor prognosis.…”

Section: Discussionsupporting

confidence: 82%

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Prevalence and prognosis of chronic fibrosing interstitial lung diseases with a progressive phenotype

et al. 2022

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Background and objective: The development of clinically progressive fibrosis complicates a wide array of interstitial lung diseases (ILDs). However, there are limited data regarding its prevalence and prognosis. Methods: We analysed consecutive patients seen for initial evaluation of a fibrosing form of ILD (FILD). Patients were evaluated for evidence of progressive fibrosis over the first 24 months of follow-up. We defined a progressive phenotype as the presence of at least one of the following: a relative decline in forced vital capacity (FVC) of ≥10%; a relative decline in FVC of ≥5%-<10% with a relative decline in diffusing capacity of the lung for carbon monoxide of ≥15%, increased fibrosis on HRCT or progressive symptoms. Results: Eight hundred and forty-four patients (397 with idiopathic pulmonary fibrosis [IPF] and 447 non-IPF FILD) made up the final analysis cohort. Three hundred and fifty-five patients (42.1%) met the progressive phenotype criteria (59.4% of IPF patients and 26.6% of non-IPF FILD patients, p <0.01). In both IPF and non-IPF FILD, transplantation-free survival differed between patients with a progressive phenotype and those without (p <0.01). Multivariable analysis showed that a progressive phenotype was an independent predictor of transplantation-free survival (hazard ratio [HR]: 3.36, 95% CI: 2.68-4.23, p <0.01). Transplantation-free survival did not differ between non-IPF FILD with a progressive phenotype and IPF (HR: 1.12, 95% CI: 0.85-1.48, p = 0.42). Conclusion: Over one-fourth of non-IPF FILD patients develop a progressive phenotype compared to approximately 60% of IPF patients. The survival of non-IPF FILD patients with a progressive phenotype is similar to IPF.

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Section: Discussionsupporting

confidence: 85%

Section: Discussionsupporting

confidence: 82%

Prevalence and prognosis of chronic fibrosing interstitial lung diseases with a progressive phenotype

et al. 2022

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“…In an IPF cluster analysis, progressive patients were divided into three different clusters based on three unique expression patterns of proteins, which were pondered to refer different progressive profiles [ 17 ]. In addition, among patients with non-IPF ILDs the survival was shorter in patients with disease progression than in patients with a stable disease course [ 8 , 10 ].…”

Section: Introductionmentioning

confidence: 99%

Reticulation pattern without honeycombing on high-resolution CT is associated with the risk of disease progression in interstitial lung diseases

et al. 2022

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Background The disease course of idiopathic pulmonary fibrosis (IPF) is progressive and occasionally, other types of interstitial lung disease (ILD) may progress similarly to IPF. This study aimed to evaluate risk factors for disease progression within 24 months in patients with various ILDs. Methods This prospective study obtained 97 patients with a suspected ILD who underwent a transbronchial lung cryobiopsy. The extent of several high-resolution computed tomography (HRCT) patterns was assessed. Due to the inclusion criteria the study population presented a low extent of honeycombing and definite usual interstitial pneumonia (UIP) pattern on HRCT suggesting an early stage of ILD. Disease progression within 24 months despite treatment was defined as a relative decline of ≥ 10% in forced vital capacity (FVC), or a relative decline in FVC of ≥ 5% and one of the three additional criteria: (1) a decline in diffusion capacity to carbon monoxide (DLCO) ≥ 15%; (2) increased fibrosis on HRCT; (3) progressive symptoms, or progressive symptoms and increased fibrosis on HRCT. The same definition was utilized in patients with IPF and other ILDs. Risk factors for disease progression were evaluated in a multivariable logistic regression model. Results Disease progression was revealed in 52% of the patients with ILD, 51% of the patients with IPF, and 53% of the patients with other types of ILD. A high extent of reticulation on HRCT (Odds ratio [OR] 3.11, 95% Confidence interval [CI] 1.21–7.98, P = 0.019) and never smoking (OR 3.11, CI 1.12–8.63, P = 0.029) were associated with disease progression whereas platelet count (OR 2.06 per 100 units increase, CI 0.96–4.45, P = 0.065) did not quite reach statistical significance. Conclusion Higher extent of reticulation on HRCT and never smoking appeared to associate with the risk of disease progression within 24 months in ILD patients without honeycombing. Approximately half of the patients with ILD revealed disease progression, and similar proportions were observed in patients with IPF and in other types of ILD.

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“…A large-scale survey estimated that 18.0%-32.0% of patients diagnosed with non-IPF ILDs developed progressive fibrosis with overall survival of 61-80 months in the US, France, Germany, Italy, Spain, the United Kingdom, and Japan (Wijsenbeek et al, 2019). PF-ILD was identified in 135 of 396 (34.1%) patients with non-IPF ILDs in a cohort of Korean patients (Kwon et al, 2021). In another Chinese single-center cohort of 608 patients, 132 patients (21.7%) with ILD met the criteria for PF-ILD (Chen et al, 2021).…”

Section: Discussionmentioning

confidence: 99%

Prognostic Predictive Characteristics in Patients With Fibrosing Interstitial Lung Disease: A Retrospective Cohort Study

Wang

Guo

et al. 2022

Front. Pharmacol.

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Background: Limited data are available regarding the entire spectrum of interstitial lung disease with a progressive fibrosing feature. We investigated the prevalence and prognostic predictive characteristics in patients with PF-ILD.Methods: This retrospective cohort study included patients with fibrosing ILD who were investigated between 1 January 2015 and 30 April 2021. We recorded clinical features and outcomes to identify the possible risk factors for fibrosing progression as well as mortality.Results: Of the 579 patients with fibrosing ILD, 227 (39.21%) met the criteria for progression. Clubbing of fingers [odds ratio (OR) 1.52, 95% confidence interval (CI) 1.03 to 2.24, p = 0.035] and a high-resolution computed tomography (HRCT)-documented usual interstitial pneumonia (UIP)-like fibrotic pattern (OR 1.95, 95% CI 1.33 to 2.86, p = 0.001) were risk factors for fibrosis progression. The mortality was worse in patients with PF with hypoxemia [hazard ratio (HR) 2.08, 95% CI 1.31 to 3.32, p = 0.002], in those with baseline diffusion capacity of the lung for carbon monoxide (DLCO) % predicted <50% (HR 2.25, 95% CI 1.45 to 3.50, p < 0.001), or in those with UIP-like fibrotic pattern (HR 1.68, 95% CI 1.04 to 2.71, p < 0.001).Conclusion: Clubbing of fingers and an HRCT-documented UIP-like fibrotic pattern were more likely to be associated with progressive fibrosing with varied prevalence based on the specific diagnosis. Among patients with progressive fibrosing, those with hypoxemia, lower baseline DLCO% predicted, or UIP-like fibrotic pattern showed poor mortality.

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Progressive fibrosing interstitial lung disease: prevalence and clinical outcome

Cited by 38 publications

References 34 publications

Prevalence and prognosis of chronic fibrosing interstitial lung diseases with a progressive phenotype

Prevalence and prognosis of chronic fibrosing interstitial lung diseases with a progressive phenotype

Reticulation pattern without honeycombing on high-resolution CT is associated with the risk of disease progression in interstitial lung diseases

Prognostic Predictive Characteristics in Patients With Fibrosing Interstitial Lung Disease: A Retrospective Cohort Study

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