An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management

Raghu, Ganesh; Collard, Harold R.; Egan, Jim; Martínez, Fernando J.; Behr, Jüergen; Brown, Kevin K.; Colby, Thomas V.; Cordier, J.-F.; Flaherty, Kevin R.; Lasky, Joseph A.; Lynch, David A.; Ryu, Jay H.; Swigris, Jeffrey J.; Wells, Athol U.; Ancochea, Julio; Bouros, Demosthenes; Carvalho, Carlos Roberto Ribeiro; Costabel, Ulrich; Ebina, Masahito; Hansell, David M.; Johkoh, Takeshi; Kim, Dong Soon; King, Talmadge E.; Kondoh, Yasuhiro; Myers, Jeffrey L.; Müller, Néstor L.; Nicholson, Andrew G.; Richeldi, Luca; Selman, Moisés; Dudden, Rosalind F.; Griss, Barbara S.; Protzko, Shandra; Schünemann, Holger J.

doi:10.1164/rccm.2009-040gl

Cited by 6,273 publications

(7,982 citation statements)

References 313 publications

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“…The diagnosis was based on predetermined clinical, radiological, and histological criteria based on current international guidelines [1]. Consecutive patients with a definitive diagnosis of IPF were included if they had a consultation from October 2015 to April 2016 and had completed CAT questionnaire at the time of consultation.…”

Section: Methodsmentioning

confidence: 99%

“…Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive disease that is associated with increased morbidity and mortality [1], and is the most common type of idiopathic interstitial pneumonia (IIP). It accounts for approximately 55% of all new cases [2,3].…”

Section: Introductionmentioning

confidence: 99%

“…It accounts for approximately 55% of all new cases [2,3]. It is characterized by fibrotic scarring in the lung tissue subsequent to lung injury that remains poorly understood [1]. The incidence of IPF has been shown to be between 6.8 and 7.4 pr.…”

Section: Introductionmentioning

confidence: 99%

“…The cardinal symptoms are increased exertional dyspnoea and dry cough. Progression of fibrosis leads to respiratory insufficiency and death [1,7,9–11]. Disease progress is often slow and occurs gradually over a few years.…”

Section: Introductionmentioning

confidence: 99%

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Validation of the COPD Assessment Test (CAT) in patients with idiopathic pulmonary fibrosis

Grufstedt

Shaker

Konradsen

2018

European Clinical Respiratory Journal

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Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic disease with high morbidity and mortality. A reliable and manageable questionnaire that assesses patients’ quality of life is needed to help to improve the content of clinical consultations. The chronic obstructive pulmonary disease (COPD) Assessment Test (CAT) is used in clinical practice; therefore, the aim of this study was to investigate correlations among the CAT, St. George’s Respiratory Questionnaire (SGRQ) and clinical parameters among patients diagnosed with IPF.Methods: A retrospective cohort design was employed in which 87 patients diagnosed with IPF who were receiving treatment at an outpatient clinic were included.Results: The CAT was found to be significantly correlated with SGRQ (p < 0.001). Furthermore, the CAT was significantly correlated with all of the included physiological variables.Conclusions: This add to support the validity of the COPD Assessment test (CAT) as a measure of symptoms in patients with IPF which in clinical practice in combination with dialogue between healthcare professionals and the patient, can help to improve the content of a clinical consultation.

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Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Validation of the COPD Assessment Test (CAT) in patients with idiopathic pulmonary fibrosis

Grufstedt

Shaker

Konradsen

2018

European Clinical Respiratory Journal

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“…The median survival of WHWTs with IPF has been reported in a retrospective study to be 16 months from onset of clinical signs and 7 months from diagnosis, but speculated not to shorten life expectancy as the disease affects middle‐aged to older WHWTs 2. In humans, median survival of IPF patients from diagnosis is only 2–3 years,5 whereas fibrotic NSIP has a better prognosis of 6–13.5 years 6, 7…”

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confidence: 99%

Long‐Term Outcome and Use of 6‐Minute Walk Test in West Highland White Terriers with Idiopathic Pulmonary Fibrosis

Lilja‐Maula

Laurila

Syrjä

et al. 2014

Veterinary Internal Medicne

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BackgroundIdiopathic pulmonary fibrosis (IPF) is an incurable interstitial lung disease occurring mainly in West Highland White Terriers (WHWTs). The effects of IPF on survival and on exercise tolerance in WHWTs are unknown.ObjectivesTo evaluate survival, prognostic factors, and exercise tolerance in WHWTs with IPF.AnimalsPrivately owned WHWTs; 15 with IPF and 11 healthy controls.MethodsProspective case‐control study conducted in 2007–2012. For survival, descriptive statistics and Kaplan–Meier (KM) survival curves with Cox proportional hazard ratios were performed. For the prognostic factor study, KM curves, Cox regression analysis, and logistic regression models were used. The 6‐minute walk test (6MWT) was used for measurement of exercise tolerance.ResultsThe median IPF‐specific survival of deceased WHWTs (7/15) with IPF was 32 (range 2–51) months from onset of clinical signs. The risk of death from birth in WHWTs with IPF in age‐adjusted Cox model was significantly higher (hazard ratio 4.6; 95% confidence interval 1.05–19.74, P = .04) than in control WHWTs. No significant prognostic factors were identified. In 6MWT, WHWTs with IPF walked a shorter distance, median 398 m (range 273–519 m), than healthy controls, median 492 m (420–568 m), P = .05, and the partial pressure of oxygen in arterial blood in diseased dogs had a moderate positive correlation with walking distance (Kendall′s tau‐b = 0.69, P = .06).Conclusion and Clinical Importance IPF had a negative impact on life expectancy, but individual survival varied considerably. 6MWT proved to be a well‐tolerated, noninvasive test to evaluate exercise tolerance.

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Association Between Interstitial Lung Abnormalities and All-Cause Mortality

Putman

Hatabu

Araki

et al. 2016

JAMA

364

392

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An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management

Cited by 6,273 publications

References 313 publications

Validation of the COPD Assessment Test (CAT) in patients with idiopathic pulmonary fibrosis

Validation of the COPD Assessment Test (CAT) in patients with idiopathic pulmonary fibrosis

Long‐Term Outcome and Use of 6‐Minute Walk Test in West Highland White Terriers with Idiopathic Pulmonary Fibrosis

Association Between Interstitial Lung Abnormalities and All-Cause Mortality

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