The progressive fibrotic phenotype in current clinical practice

Wells, Athol U.; Brown, Kevin K.; Cottin, Vincent

doi:10.1097/mcp.0000000000000805

Cited by 9 publications

(9 citation statements)

References 37 publications

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“…5 Institute of Clinical Radiology, School of Medicine, Faculty of Health Sciences, University of Eastern Finland, POB 1627, 70211 Kuopio, Finland. 6 Acute Services, Kuopio University Hospital, POB 100, 70029 Kuopio, Finland. 7 Science Services Center, Kuopio University Hospital, POB 100, 70029 Kuopio, Finland.…”

Section: Supplementary Informationmentioning

confidence: 99%

“…Occasionally, other types of interstitial lung disease (ILD), such as rheumatoid arthritis associated ILD (RA-ILD), systemic scleroderma associated ILD (SSc-ILD), fibrotic hypersensitivity pneumonia (fHP), and acute fibrinous and organizing pneumonia, may progress similarly to IPF [5]. It has been suggested that rather than a specific ILD diagnosis, the disease course should determine the management of ILDs [6] since patients with a progressive disease course have revealed similar survival rates than patients with IPF [7,8]. Moreover, an antifibrotic drug nintedanib has been found to be effective also in non-IPF ILD patients with disease progression [9].…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Supplementary Informationmentioning

confidence: 99%

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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“…auch die Karenz gegenüber der auslösenden Noxe empfohlen. Erst wenn trotz dieser Maßnahmen nach den in der Präambel definierten Progressionskriterien der Krankheitsverlauf als PPF zu klassifizieren ist, sollte mit einer antifibrotischen Therapie unabhängig vom HRCT-Muster begonnen werden [16,170]. Hierbei ist auch eine retrospektive Analyse des Krankheitsverlaufs zulässig.…”

Section: Sollen Patienten Mit Fibrosierender Ild (Inkl Ipf) Und Mit P...unclassified

Pharmakotherapie der idiopathischen Lungenfibrose (ein Update) und anderer progredienter pulmonaler Fibrosen

et al. 2023

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“…Moreover, it is also important to note that our IPAF progressed during the first years of disease, as 50% of them developed functional deterioration at 16 months after IPAF diagnosis. To date, there are no studies that analyze long-term pulmonary functional progression, although as in other ILDs, this is a determining factor in the definition of a progressive phenotype with prognostic and therapeutic implications [ 30 , 31 ]. Our results are similar to those shown in patients with rheumatoid arthritis-related ILD from the NEREA registry [ 32 ].…”

Section: Discussionmentioning

confidence: 99%

Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry

Nieto

Sánchez‐Pernaute²,

Rio-Ramirez³

et al. 2023

Respir Res

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Background The objective of the present study is to describe the characteristics of interstitial pneumonia with autoimmune features (IPAF) patients, to assess the incidence rate of functional respiratory impairment over time and to evaluate the influence of therapeutic alternatives on the prognosis of these patients. Methods A longitudinal observational multicenter study was performed (NEREA registry). It was carried out by a multidisciplinary team in seven Hospitals of Madrid. Patients were included from IPAF diagnosis. Main outcome: poor prognosis as functional respiratory impairment (relative decline in FVC % defined as ≥ 5% every 6 months). Covariates: therapy, sociodemographic, clinical, radiological patterns, laboratory and functional tests. Statistics: Survival techniques were used to estimate IR per 100 patients-semester with their 95% confidence interval [CI]. The influence of covariates in prognosis were analyzed through cox multivariate regression models (hazard ratio (HR) and [CI]). Results 79 IPAF were included, with a mean and a maximum follow-up of 3.17 and 12 years respectively. Along the study, 77.2% received treatment (52 glucocorticoids, 25 mycophenolate, 21 azathioprine, 15 rituximab and 11 antifibrotics). IR was 23.9 [19.9–28.8], and 50% of IPAF developed functional respiratory impairment after 16 months from its diagnosis. Multivariate analysis: usual interstitial pneumonia (UIP) had poorer prognosis compared to non-specific interstitial pneumonia (NSIP) (p = 0.001). In NSIP, positive ANA, increased the risk of poor prognosis. In UIP, glucocorticoids (HR: 0.53 [0.34–0.83]), age (HR: 1.04 [1.01–1.07]), and Ro-antibodies (HR: 0.36 [0.19–0.65]) influenced the prognosis. Conclusions IPAF have functional impairment during the first years of disease. Factors predicting deterioration differ between radiographic patterns. Our real-life study suggests the potential benefit of particular therapies in IPAF.

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The progressive fibrotic phenotype in current clinical practice

Cited by 9 publications

References 37 publications

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

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

Pharmakotherapie der idiopathischen Lungenfibrose (ein Update) und anderer progredienter pulmonaler Fibrosen

Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry

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