The added value of comorbidities in predicting survival in idiopathic pulmonary fibrosis: a multicentre observational study

Torrisi, Sebastiano Emanuele; Ley, Brett; Kreuter, Michael; Wijsenbeek, Marlies; Vittinghoff, Eric; Collard, Harold R.; Vancheri, Carlo

doi:10.1183/13993003.01587-2018

Cited by 59 publications

(55 citation statements)

References 35 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…When we adjusted the effects from the GAP stages for pulmonary co-morbidity, we noted that effect of the stage III group on mortality was reduced compared with stage I. This corroborates the recent trend to introduce comorbidity in prognostic indices [36]. Our cubic spline analyses of the continuous predictors age at diagnosis, Fig.…”

Section: Discussionsupporting

confidence: 82%

The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

Tran¹,

Šterclová²,

Moğulkoç³

et al. 2020

Respir Res

View full text Add to dashboard Cite

Background: Several registries of idiopathic pulmonary fibrosis (IPF) have been established to better understand its natural history, though their size and duration of follow-up are limited. Here, we describe the large European MultiPartner IPF Registry (EMPIRE) and validate predictors of long-term survival in IPF. Methods: The multinational prospective EMPIRE registry enrolled IPF patients from 48 sites in 10 Central and Eastern European countries since 2014. Survival from IPF diagnosis until death was estimated, accounting for lefttruncation. The Cox proportional hazards regression model was used to estimate adjusted hazard ratios (HR) of death for prognostic factors, using restricted cubic splines to fit continuous factors. Results: The cohort included 1620 patients (mean age at diagnosis 67.6 years, 71% male, 63% smoking history), including 75% enrolled within 6 months of diagnosis. Median survival was 4.5 years, with 45% surviving 5 years post-diagnosis. Compared with GAP stage I, mortality was higher with GAP stages II (HR 2.9; 95% CI: 2.3-3.7) and III (HR 4.0; 95% CI: 2.8-5.7) while, with redefined cutoffs , the corresponding HRs were 2.7 (95% CI: 1.8-4.0) and 5.8 (95% CI: 4.0-8.3) respectively. Mortality was higher with concurrent pulmonary hypertension (HR 2.0; 95% CI: 1.5-2.9) and lung cancer (HR 2.6; 95% CI: 1.3-4.9). Conclusions: EMPIRE, one of the largest long-term registries of patients with IPF, provides a more accurate confirmation of prognostic factors and co-morbidities on longer term five-year mortality. It also suggests that some fine-tuning of the indices for mortality may provide a more accurate long-term prognostic profile for these patients.

show abstract

Section: Discussionsupporting

confidence: 82%

The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

Tran¹,

Šterclová²,

Moğulkoç³

et al. 2020

Respir Res

View full text Add to dashboard Cite

show abstract

“…However, baseline FVC does only have limited prognostic value and longitudinal changes in FVC have some weaknesses [63]. Therefore, composite scoring systems incorporating lung physiology, sex and age are deemed more accurate at predicting mortality [64,65]. It is recognised that biomarkers may augment these models and improve prognostication in IPF.…”

Section: Prognostic Biomarkersmentioning

confidence: 99%

“…The German INSIGHTS IPF registry has determined that an increased number of comorbidities decreases HRQL [191]. Recently, the novel comorbidity assessment TORVAN model has shown that comorbidities may refer to a lower survival rate based on clinical and physiological parameters as in the GAP index [192]. While no effects of antifibrotic drugs on comorbidities have been reported so far, several reports focused on the effects of comedications or treatments of comorbidities in IPF.…”

Section: Treatment Of Comorbiditiesmentioning

confidence: 99%

The therapy of idiopathic pulmonary fibrosis: what is next?

et al. 2019

Self Cite

View full text Add to dashboard Cite

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial lung disease, characterised by progressive scarring of the lung and associated with a high burden of disease and early death. The pathophysiological understanding, clinical diagnostics and therapy of IPF have significantly evolved in recent years. While the recent introduction of the two antifibrotic drugs pirfenidone and nintedanib led to a significant reduction in lung function decline, there is still no cure for IPF; thus, new therapeutic approaches are needed. Currently, several clinical phase I–III trials are focusing on novel therapeutic targets. Furthermore, new approaches in nonpharmacological treatments in palliative care, pulmonary rehabilitation, lung transplantation, management of comorbidities and acute exacerbations aim to improve symptom control and quality of life. Here we summarise new therapeutic attempts and potential future approaches to treat this devastating disease.

show abstract

“…Die Auswertungen von großen randomisierten Studien [11 -13] Ein früheres Krankheitsauftreten ist bei den familiären Formen der IPF möglich und mehrfach beschrieben [17,19]. Dennoch sollte man bei jüngerem Alter der betroffenen Patienten immer auch an die Möglichkeit einer pulmonalen Manifestation einer Autoimmunerkrankung denken [20].…”

Section: Epidemiologieunclassified

S2K-Leitlinie zur Diagnostik der idiopathischen Lungenfibrose

Behr,

Günther,

Bonella

et al. 2020

Pneumologie

View full text Add to dashboard Cite

ZusammenfassungDie idiopathische Lungenfibrose (IPF) ist eine schwerwiegende, chronische Lungenerkrankung, deren Diagnosestellung eine profunde fachliche Expertise verlangt. Seit Veröffentlichung der internationalen IPF-Leitlinie im Jahr 2011 mit Update 2018 haben zahlreiche Studien und technische Fortschritte eine Neubewertung des diagnostischen Vorgehens notwendig gemacht. Angesichts der Indikation, symptomatischen Patienten eine antifibrotische Therapie sofort nach Diagnosestellung zu empfehlen, hat die aktuelle IPF-Diagnostik-Leitlinie zum Ziel, die Diagnose der IPF möglichst ohne Verzögerung, wenig invasiv und zuverlässig zu ermöglichen. Sie beschreibt den typischen klinischen Kontext der IPF sowie die Untersuchungen, die zum Ausschluss bekannter Ursachen einer Lungenfibrose erforderlich sind, einschließlich standardisierter Anamneseerhebung, serologischer Testungen und zellulärer Analyse der bronchoalveolären Lavage. Zentrale Bedeutung kommt dem hochauflösenden Computertomogramm in Dünnschichttechnik zu. Sofern eine Histologie zur Diagnoseeingrenzung erforderlich ist, soll diese in erster Linie mittels der transbronchialen Lungen-Kryobiopsie gewonnen werden. Die chirurgische Lungenbiopsie soll Patienten vorbehalten bleiben, die in ausreichender Verfassung für diesen Eingriff sind und bei denen die endoskopische Diagnostik keine Klärung der Diagnose ergab. Goldstandard ist auch weiterhin die interdisziplinäre Diskussion der erhobenen Befunde, um nach Ausschluss anderer Formen chronisch progredienter Lungenfibrosen die Diagnose der IPF zu stellen.

show abstract

The added value of comorbidities in predicting survival in idiopathic pulmonary fibrosis: a multicentre observational study

Abstract: This is the first ever validated clinical prediction model and point score index for all-cause mortality in IPF to include comorbidity variables. Their inclusion significantly improved prediction of survival beyond demographic and physiological parameters.

Cited by 59 publications

References 35 publications

The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

The therapy of idiopathic pulmonary fibrosis: what is next?

S2K-Leitlinie zur Diagnostik der idiopathischen Lungenfibrose

Contact Info

Product

Resources

About