Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report

Collard, HR; Cj, Ryerson; Tj, Corte; Jenkins, Gisli; Kondoh, Yasuhiro; Lederer, David J.; Lee, Joyce; Maher, Toby M.; Au, Wells; Km, Antoniou; Behr, Jüergen; Brown, KK; Cottin,; Kr, Flaherty; Fukuoka, Junya; Dm, Hansell; Johkoh, Takeshi; Kaminski, Naftali; Ds, Kim; Kolb, Martin; Lynch, DA; Jl, Myers; Raghu, Ganesh; Richeldi, Luca; Taniguchi, Hiroyuki; Fj, Martínez

doi:10.1164/rccm.201604-0801ci

Cited by 1,115 publications

(1,241 citation statements)

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“…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%

“…However, some patients experience an accelerated decline, with acute respiratory worsening and exacerbations [12,13]. It is the type of lung fibrosis with the highest mortality rate, as the median length of survival is 3–5 years [8–11,14], which means that IPF has a poorer prognosis than a number of malignant diseases [7,15]. In 2006, Gribbin and colleagues reported a 43% 5-year survival rate among patients with IPF, and Navaratnam later estimated a 5-year survival rate of 37% in 2011 [4,16].…”

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: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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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“…However, acute exacerbations were not associated with increased visits to a physician. This may mean that acute exacerbations influence primarily hospitalisation-related costs, which is consistent with the often-severe nature of this complication and its strong association with morbidity and mortality [11][12][13]. Our analysis covered unscheduled visits to the hospital or physicians.…”

Section: Discussionmentioning

confidence: 69%

“…Acute exacerbations are associated with high morbidity and mortality [11][12][13]. They usually require a visit to a specialist or a general practitioner (GP) in less severe cases or hospitalisation in more severe cases.…”

Section: Introductionmentioning

confidence: 99%

Influence of Idiopathic Pulmonary Fibrosis Progression on Healthcare Resource Use

Διαμαντόπουλος¹,

Maher

Schoof

et al. 2018

PharmacoEconomics Open

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Background Disease progression and acute exacerbations in patients with idiopathic pulmonary fibrosis (IPF) are associated with high morbidity and mortality. They usually require a visit to a specialist or a general practitioner (GP) in less severe cases or hospitalisation in more severe cases. Objective The objective of this study was to identify factors that influence resource use in IPF. Methods Clinical and healthcare resource use data were collected in two large, international, multi-centre, randomised controlled trials (RCTs) that studied nintedanib for the treatment of IPF (INPULSIS-1 and -2). The pooled data of nintedanib and placebo included 1014 patients followed for 12 months. The trial data were analysed in 3-month intervals. We studied two dependent variables: the occurrence of allcause hospitalisation and visits to a physician (GP or specialist). The independent variables included the change in forced vital capacity percent predicted (FVC%pred), investigator-reported acute exacerbation events, age, time since diagnosis, smoking status, and sex. Results Hospitalisation during a 3-month interval was significantly associated with a drop of at least 5 or 10 points in FVC%pred (odds ratios [ORs] 1.58 [p = 0.009] and 2.62[p \ 0.001]) and associated with the occurrence of at least one acute exacerbation (OR 14.44; p \ 0.001) during the same interval. The above factors remained significant when repeating the analysis for hospitalisation based on change in FVC%pred or events occurring during the previous 3 months interval. Smoker status and a unit change in FVC%pred during the previous interval were added to the significant factors. Physician visits during a 3-month interval were significantly associated with a lower FVC%pred at the start of the interval (per 10-point decrement, OR 1.05; p = 0.040) and with the change in FVC%pred during the same interval (per 10-point loss, OR 1.13; p = 0.042). Visits were also associated with a 5-point drop in FVC%pred (OR 1.23; p = 0.020), age (per 5-year increments OR 1.07; p = 0.028), and female sex (OR 1.32; p = 0.017). Nevertheless, the predictive power of the models was considered poor for both outcomes (hospitalisation and physician visits). Conclusions Disease progression and acute exacerbation events are significantly associated with hospitalisation of patients with IPF. Outpatient visits to physicians are associated with disease progression, baseline FVC%pred, age and sex.

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“…Acute exacerbation is known to develop during IPF clinical course, and, in 2011 evidence-based IPF guidelines, it is clearly stated as "acute exacerbation" [3]. In 2016, "An International Working Group Report" on acute exacerbation has been published [4]. In this report, new definitions and diagnostic criteria of acute exacerbation were proposed.…”

Section: Introductionmentioning

confidence: 99%

Thrombomodulin for Acute Exacerbations of Idiopathic Pulmonary Fibrosis

Ichimura¹,

Tsushima²,

Matsumura³

et al. 2017

Chron Obstruct Pulmon Dis

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Background: Acute exacerbation (AE) is known as a leading cause of morbidity and mortality in chronic fibrosing group includes idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP). There is no standardizes drug therapy with comparative studies. The clinical application of recombinant human soluble thrombomodulin (rhTM) has recently been expected against AE-IPF/NSIP, however, the impact of rhTM remains unestablished. Thus, this review assessed the impact of rhTM on mortality in patients with AE-IPF/NSIP.

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Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report

Cited by 1,115 publications

References 89 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

Influence of Idiopathic Pulmonary Fibrosis Progression on Healthcare Resource Use

Thrombomodulin for Acute Exacerbations of Idiopathic Pulmonary Fibrosis

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