Probable Idiopathic Pulmonary Fibrosis Research Articles

Survival and lung function decline in patients with definite, probable and possible idiopathic pulmonary fibrosis treated with pirfenidone.

There is no clear evidence whether pirfenidone has a benefit in patients with probable or possible UIP, i.e. when idiopathic pulmonary fibrosis (IPF) is diagnosed with a lower degree of diagnostic certainty. We report on outcomes of treatment with pirfenidone in IPF patients diagnosed with various degrees of certainty. We followed patients in the multi-national European MultiPartner IPF Registry (EMPIRE) first seen between 2015 and 2018. Patients were assessed with HRCT, histopathology and received a multi-disciplinary team (MDT) IPF diagnosis. Endpoints of interest were overall survival (OS), progression-free survival (PFS) and lung function decline. A total of 1626 patients were analysed, treated with either pirfenidone (N = 808) or receiving no antifibrotic treatment (N = 818). When patients treated with pirfenidone were compared to patients not receiving antifibrotic treatment, OS (one-, two- and three-year probability of survival 0.871 vs 0.798; 0.728 vs 0.632; 0.579 vs 0.556, P = 0.002), and PFS (one-, two- and three-year probability of survival 0.597 vs 0.536; 0.309 vs 0.281; 0.158 vs 0.148, P = 0.043) was higher, and FVC decline smaller (-0.073 l/yr vs -0.169 l/yr, P = 0.017). The benefit of pirfenidone on OS and PFS was also seen in patients with probable or possible IPF. This EMPIRE analysis confirms the favourable outcomes observed for pirfenidone treatment in patients with definitive IPF and indicates benefits also for patients with probable or possible IPF.

IPF diagnosis: flexibility is a virtue

In 2011, the joint guidelines 1 Raghu G Collard HR Egan JJ et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011; 183: 788-824 Crossref PubMed Scopus (5323) Google Scholar of the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Thoracic Association of Latin America provided the first truly international evidence-based statement on the diagnosis of idiopathic pulmonary fibrosis (IPF). Although this statement undoubtedly underpinned international collaboration in science and interventional trials, the rigour of the 2011 diagnostic criteria effectively disenfranchised a large subgroup of patients with IPF, in whom a surgical lung biopsy (SLB) could not be done and no definite diagnosis could be made. The concept of probable IPF was not explored in the 2011 guidelines, and the document was misinterpreted by some as proscribing IPF-specific therapies unless formal diagnostic guideline criteria were met. CorrectionsWells A U. IPF: flexibility is a virtue. Lancet Respir Med 2018; published online Sept 14. http://dx.doi.org/10.1016/S2213-2600(18)30374-6—In this Comment, the title should be “IPF diagnosis: flexibility is a virtue”. This correction has been made to the online version as of Sept 26, 2018, and the printed version is correct. Full-Text PDF

Definitions of disease: Should possible and probable idiopathic pulmonary fibrosis be enrolled in treatment trials?

Idiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing interstitial lung disease of unknown etiology characterized by progressive lung scarring and a median survival of 3-5 years from the time of diagnosis. The most recent consensus guidelines adopt a diagnostic process that characterizes patients as having a final diagnosis of IPF, probable IPF, or possible IPF determined from a combination of the clinical context and specific chest imaging and histologic disease patterns. Based on currently available data, the enrollment criteria for treatment trials could be expanded to include not only patients with IPF but also those with probable and possible IPF without adversely affecting trial design or outcomes.

P9 Cough Is Prevalent In Higher Proportion Of Older Patients With Both Idiopathic Pulmonary Fibrosis And Non-specific Interstitial Lung Disease

Introduction Cough is a major symptom of Idiopathic Pulmonary fibrosis (IPF), a progressive, fatal lung disease with median survival of 3 years. The first study to suggest cough as an independent predictor of disease progression, reported it in 84% of the population. 1 The only study to quantify cough in 19 IPF patients, reported strong correlation between objective cough counts and cough related quality of life 2 There is a lack of studies investigating the prevalence, pathogenesis or treatment of cough in IPF. We set up a study to evaluate the prevalence of cough in an incident IPF and Non specific Interstitial Pneumonia (NSIP) population. Methods A prospective, multi-centre, observational, cohort study, PROFILE-Central England (September 2009 to June 2014) was set up. Patients had a diagnosis of definite or probable IPF or NSIP based on the ATS consensus. Leicester cough questionnaire (LCQ) was used to assess presence or absence of cough at baseline. Furthermore,the cohort was divided into 3 groups to assess severity of cough: Mild (17–20), Moderate (11–16.9) and Severe ( Results 312 incident patients with IPF or NSIP were enrolled. 261/312(83.6%) patients had incident IPF whilst 51/312(16.4%) had NSIP. The mean age of the cohort was 73.5 years (35–90 years). 235/312(76%) were males with mean age 73.7 years (47–90 years); 74/312(24%) were females with mean age of 72.9 years (35.8–88.8 years). 261/312(83.6%) reported cough compared with 51/312(16.4%) who reported no cough. Of the patients who reported cough, 45/261(17.2%) had severe cough, 112/261(42.9%) had moderate cough and 104/261(39.8%) had mild cough. There was no effect of gender, however, older cohort reported more cough (age >55 years; p = 0.014). Smoking may be a confounder, however the number of current smokers in the cohort is too small (p = 0.05). Interestingly both NSIP and IPF cohort reported cough; however, proportionally NSIP patients have less cough (14/51,27.4%) compared with IPF (37/261, 14%). Conclusions Cough occurs in a huge majority of patients with both IPF and NSIP. Cough appears to be a greater problem in older patients. References Ryerson CJ et al . Cough predicts prognosis in idiopathic pulmonary fibrosis. Respirology . 2011 Aug;16(6):969–75 Key AL et al . Objective cough frequency in Idiopathic Pulmonary Fibrosis. Cough . 2010;6:4

Treatment strategies for idiopathic interstitial pneumonias.

With recent changes in diagnostic algorithms in idiopathic pulmonary fibrosis (IPF) guidelines and treatment advances in IPF, it is now necessary to reappraise the way in which clinicians should formulate treatment strategies in the idiopathic interstitial pneumonias. The idiopathic interstitial pneumonias can usefully be subdivided into the following categories: first, definite IPF, second, probable IPF with major differential diagnoses of fibrotic nonspecific interstitial pneumonia and chronic hypersensitivity pneumonitis and third, apparently idiopathic interstitial pneumonias other than IPF. In definite IPF, the therapeutic landscape has irrevocably changed with the identification of robust treatment effects with pirfenidone and nintedanib, consisting of the prevention of approximately 50% of disease progression (as judged by serial forced vital capacity trends). In probable IPF, generally equating with high resolution computed tomography findings of 'possible usual interstitial pneumonia' and the nonperformance of a diagnostic surgical biopsy, management is based on multidisciplinary evaluation, integrating all available information, with a final 'working diagnosis' made for treatment purposes. In other idiopathic interstitial pneumonias and their major differentials, removal of potential triggers and immunomodulation remain the cornerstones of therapy, with treatment goals usefully designated using a disease behaviour classification. In mild disease, an initial policy of observation is often appropriate. The striking recent treatment effects reported in IPF will have major management implications in the idiopathic interstitial pneumonia in general, whenever IPF is in the differential diagnosis.

Effects of smoking, gender and occupational exposure on the risk of severe pulmonary fibrosis: a population-based case-control study

ObjectivesTo estimate the effects of smoking, gender and occupational exposure on the risk of developing severe pulmonary fibrosis (PF), including dose-response and interaction effects.MethodsNational case–control study of 171 patients (cases)...

S17 A prothrombotic state is associated with increased mortality in idiopathic pulmonary fibrosis

BackgroundOur previous case-control study demonstrated that a prothrombotic state was almost five times more common in people with idiopathic pulmonary fibrosis (IPF) than general population controls. We followed up the...

The revised ATS/ERS/JRS/ALAT diagnostic criteria for idiopathic pulmonary fibrosis (IPF) - practical implications

Idiopathic pulmonary fibrosis (IPF), the most prevalent idiopathic interstitial pneumonia, is associated with a poor prognosis. An accurate diagnosis of IPF is essential for optimal management. The recent ATS/ERS/JRS/ALAT recommendations on the diagnosis and management of IPF were developed from a systematic review of the published literature. High-resolution computed tomography (HRCT) scanning has a central role in the IPF diagnostic pathway with formal designation of criteria for an HRCT pattern of UIP. In the correct clinical context, a UIP pattern on HRCT is indicative of a definite diagnosis of IPF without the need for a surgical lung biopsy. However, although the 2011 ATS/ERS/JRS/ALAT statement is a major advance, the application of guideline recommendations by clinicians has identified limitations that need to be addressed in future statements. Key problems include: 1) the lack of management recommendations for the highly prevalent clinical scenarios of probable and possible IPF; 2) the ongoing confusion about the diagnostic role of bronchoalveolar lavage (reflecting ambiguity in the current recommendation); 3) HRCT misdiagnosis by less experienced radiologists, increasingly recognised as a major problem; and 4) the lack of integration of clinical data, including the treated course of disease, in the designation of the diagnostic likelihood of IPF.

Semiinvasive Aspergillosis: A Diagnosis to Consider in a Seemingly Immunocompetent Patient with a Pulmonary Nodule, Haemoptysis and Structural Lung Disease.

Semiinvasive pulmonary aspergillosis is an indolent form of pulmonary aspergillosis, which is seen mainly in patients who are mildly immunocompromised with underlying chronic lung diseases. This syndrome is rare, and the available literature is based on case reports and small case series. We describe here a patient with a semiinvasive aspergillosis associated with a probable idiopathic pulmonary fibrosis.

Clinical Course of Probable Idiopathic Pulmonary Fibrosis

Background : According to the 2002 consensus report of the American Thoracic Society/European Respiratory Society (ATS/ERS), idiopathic pulmonary fibrosis (IPF) was classified as biopsy proven or probable IPF. Probable IPF is defined as those with distinctive features that allow for a confident diagnosis of IPF/usual interstitial pneumonia (UIP) within an appropriate clinical setting. The determination of the clinical course of probable IPF, as diagnosed by the ATS/ERS criteria, was studied. Methods : Between March 1995 and August 2002, 36 patients with probable IPF, from two tertiary referral hospitals, were enrolled in this study. The clinical characteristics, prognostic factors and treatment efficacy of these patients were retrospectively evaluated. Results : The mean age of the subjects was years. The one and 3 year survival rates were 82.4 and 50.3%, respectively, and a median survival period of 42.0 months. The total cell count of bronchoalveolar lavage was higher in the death than the survival group (p