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The article presents a study which is focused on analyzing the effect of using the nintedanib drug in the treatment of idiopathic pulmonary fibrosis (IPF). The study was conducted on 515 patients of mean age 67 years in which level of carbon monoxide was measured. According to the results, nintedanib helps in reducing the forced vital capacity (FVC) of the IPF.

Some patients with interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis exhibit a progressive clinical phenotype. These chronic progressive fibrosing ILDs have a variety of underlying diseases, and their prevalence is currently unknown. Here we carry out the first systematic review of literature on the prevalence of fibrosing ILDs and progressive fibrosing ILDs using data from physician surveys to estimate frequency of progression among different ILDs. We searched MEDLINE and Embase for studies assessing prevalence of ILD, individual ILDs associated with fibrosis and progressive fibrosing ILDs. These were combined with data from previously published physician surveys to obtain prevalence estimates of each chronic fibrosing ILD with a progressive phenotype and of progressive fibrosing ILDs overall. We identified 16 publications, including five reporting overall ILD prevalence, estimated at 6.3-76.0 per 100,000 people in Europe (four studies) and 74.3 per 100,000 in the USA (one study). In total, 13-40% of ILDs were estimated to develop a progressive fibrosing phenotype, with overall prevalence estimates for progressive fibrosing ILDs of 2.2-20.0 per 100,000 in Europe and 28.0 per 100,000 in the USA. Prevalence estimates for individual progressive fibrosing ILDs varied up to 16.7 per 100,000 people. These conditions represent a sizeable fraction of chronic respiratory disorders and have a high unmet need.

Background: This document provides clinical recommendations for the diagnosis of idiopathic pulmonary fibrosis (IPF). It represents a collaborative effort between the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society., Methods: The evidence syntheses were discussed and recommendations formulated by a multidisciplinary committee of IPF experts. The evidence was appraised and recommendations were formulated, written, and graded using the Grading of Recommendations, Assessment, Development, and Evaluation approach., Results: The guideline panel updated the diagnostic criteria for IPF. Previously defined patterns of usual interstitial pneumonia (UIP) were refined to patterns of UIP, probable UIP, indeterminate, and alternate diagnosis. For patients with newly detected interstitial lung disease (ILD) who have a high-resolution computed tomography scan pattern of probable UIP, indeterminate, or an alternative diagnosis, conditional recommendations were made for performing BAL and surgical lung biopsy; because of lack of evidence, no recommendation was made for or against performing transbronchial lung biopsy or lung cryobiopsy. In contrast, for patients with newly detected ILD who have a high-resolution computed tomography scan pattern of UIP, strong recommendations were made against performing surgical lung biopsy, transbronchial lung biopsy, and lung cryobiopsy, and a conditional recommendation was made against performing BAL. Additional recommendations included a conditional recommendation for multidisciplinary discussion and a strong recommendation against measurement of serum biomarkers for the sole purpose of distinguishing IPF from other ILDs., Conclusions: The guideline panel provided recommendations related to the diagnosis of IPF.

Experimental models are critical for the understanding of lung health and disease and are indispensable for drug development. However, the pathogenetic and clinical relevance of the models is often unclear. Further, the use of animals in biomedical research is controversial from an ethical perspective.The objective of this task force was to issue a statement with research recommendations about lung disease models by facilitating in-depth discussions between respiratory scientists, and to provide an overview of the literature on the available models. Focus was put on their specific benefits and limitations. This will result in more efficient use of resources and greater reduction in the numbers of animals employed, thereby enhancing the ethical standards and translational capacity of experimental research.The task force statement addresses general issues of experimental research (ethics, species, sex, age, ex vivo and in vitro models, gene editing). The statement also includes research recommendations on modelling asthma, chronic obstructive pulmonary disease, pulmonary fibrosis, lung infections, acute lung injury and pulmonary hypertension.The task force stressed the importance of using multiple models to strengthen validity of results, the need to increase the availability of human tissues and the importance of standard operating procedures and data quality., Competing Interests: Conflict of interest: P. Bonniaud reports personal fees (board/advice) from Roche, Boehringer, Novartis, TEVA and AstraZeneca, and travel reimbursement (ERS meetings) from Chiesi, outside the submitted work. Conflict of interest: A. Fabre reports grants from Pfizer, reagents from Roche, and personal fees from MSD (Merck Sharp & Dohme Corp.), outside the submitted work. Conflict of interest: T. Maes reports grants from Belgian Science Policy (Federal Government Belgium-Interuniversity attraction Poles), Ghent University (Concerted Research Action and Spearhead Immunology) and Novartis, and personal fees (GSK Clinical Science award: prize for best abstract presentation at Belgian Pulmonology Society Meeting 2015) and non-financial support (travel/accommodation/meeting expenses) from GlaxoSmithKline, outside the submitted work; and is shareholder of Oryzon Genomics. Conflict of interest: W. Shi reports grants from National Institute of Health and Department of Defense, outside the submitted work. Conflict of interest: M. Stampfli reports grants from RespiVert and MedImmune, and personal fees from AstraZeneca and Boehringer Ingelheim, outside the submitted work. Conflict of interest: S. Uhlig reports grants from Germany Research Foundation (DFG), during the conduct of the study. Conflict of interest: E. White reports grants and other support from NIH, personal fees for consulting from Akcea Therapeutics and Kadmon Pharmaceuticals, and research grants and personal fees for consulting from Boehringer-Ingelheim, outside the submitted work. Conflict of interest: M. Witzenrath reports grants and personal fees from Bayer Health Care, Boehringer Ingelheim, Biotest and Vaxxilon, and personal fees from Actelion, Berlin Chemie, AstraZeneca, GlaxoSmithKline and Novartis, outside the submitted work. Conflict of interest: B. Crestani has receieved an honorarium for speaking from Aventis, honoraria for speaking, grants for research and congress travel support grants from Boehringer Ingelheim and Roche, grants for research from CARDIF and LVL, an honorarium for speaking and congress travel support from AstraZeneca, and a grant for research and congress travel support from MedImmune, outside the submitted work. Conflict of interest: O. Eickelberg reports grants from the Helmholtz Association, the German Center of Lung Research, Roche, and Bayer and consultancy/lecture fees from BMS, Novartis, Bayer, Intermune, McKinsey, and MorphoSys. Conflict of interest: A. Guenther reports research funding, and compensation for lectures and consulting from Roche, compensation for lectures and consulting from Boehringer Ingelheim and Teva, and research funding from Sanofi, outside the submitted work. Conflict of interest: G. Jenkins reports grants from GlaxoSmithKline (institutional funding for the PROFILE study) and the Medical Research Council (co-funder of the PROFILE study with GSK through MICA award), during the conduct of the study; grants from Biogen (SRA for work on integrins in IPF), personal fees (consulting and data monitoring committees on lung fibrosis) from Boehringer Ingelheim, grants (PhD studentship) from Galecto, personal fees (advisory board on IPF) from GlaxoSmithKline and Intermune, grants and personal fees (consulting on IPF, PhD studentship) from MedImmune, personal fees (consulting on IPF) from PharmAkea, personal fees (advisory board on IPF and lecture fees) from Roche, consulting on IPF (with no payment received to date) for Pliant Therapeutics, participating in scientific advisory boards (with no payment received to date) for NuMedii, and personal fees from Pulmatrix, outside the submitted work; and is a trustee for the charities Action for Pulmonary Fibrosis and the British Thoracic Society. Conflict of interest: G. Joos reports grants, personal fees and non-financial support from AstraZeneca, grants and personal fees from Boehringer Ingelheim, Chiesi, GlaxoSmithKline and Novartis, and personal fees from Mundipharma, Sandoz and Teva, outside the submitted work. Conflict of interest: U.A. Maus reports grants from Federal Ministry of Education and Research, German Research Foundation and Lower Saxony Society for the Control of Tuberculosis and Bronchial Diseases, outside the submitted work. Conflict of interest: L. Richeldi reports grants and personal fees (for advisory board membership) from InterMune, personal fees (for advisory board membership) from Medimmune, Roche and FibroGen, personal fees (for consulting activity) from Biogen, Sanofi-Aventis, ImmuneWorks, Celgene and Nitto, personal fees (for speaking) from Shionogi, and personal fees (for membership of steering committee) from Boehringer Ingelheim, outside the submitted work. Conflict of interest: M. Kolb reports grants and personal fees from Roche, Boehringer Ingelheim, GSK, Gilead, Prometic and Alkermes, grants from Actelion, Respivert and Synairgen, and personal fees from AstraZeneca and Genoa, outside the submitted work., (Copyright ©ERS 2018.)

Many patients with an idiopathic interstitial pneumonia (IIP) have clinical features that suggest an underlying autoimmune process but do not meet established criteria for a connective tissue disease (CTD). Researchers have proposed differing criteria and terms to describe these patients, and lack of consensus over nomenclature and classification limits the ability to conduct prospective studies of a uniform cohort.The "European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease" was formed to create consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity.The task force proposes the term "interstitial pneumonia with autoimmune features" (IPAF) and offers classification criteria organised around the presence of a combination of features from three domains: a clinical domain consisting of specific extra-thoracic features, a serologic domain consisting of specific autoantibodies, and a morphologic domain consisting of specific chest imaging, histopathologic or pulmonary physiologic features.A designation of IPAF should be used to identify individuals with IIP and features suggestive of, but not definitive for, a CTD. With IPAF, a sound platform has been provided from which to launch the requisite future research investigations of a more uniform cohort., (Copyright ©ERS 2015.)

Background: In 2002 the American Thoracic Society/European Respiratory Society (ATS/ERS) classification of idiopathic interstitial pneumonias (IIPs) defined seven specific entities, and provided standardized terminology and diagnostic criteria. In addition, the historical "gold standard" of histologic diagnosis was replaced by a multidisciplinary approach. Since 2002 many publications have provided new information about IIPs., Purpose: The objective of this statement is to update the 2002 ATS/ERS classification of IIPs., Methods: An international multidisciplinary panel was formed and developed key questions that were addressed through a review of the literature published between 2000 and 2011., Results: Substantial progress has been made in IIPs since the previous classification. Nonspecific interstitial pneumonia is now better defined. Respiratory bronchiolitis-interstitial lung disease is now commonly diagnosed without surgical biopsy. The clinical course of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia is recognized to be heterogeneous. Acute exacerbation of IIPs is now well defined. A substantial percentage of patients with IIP are difficult to classify, often due to mixed patterns of lung injury. A classification based on observed disease behavior is proposed for patients who are difficult to classify or for entities with heterogeneity in clinical course. A group of rare entities, including pleuroparenchymal fibroelastosis and rare histologic patterns, is introduced. The rapidly evolving field of molecular markers is reviewed with the intent of promoting additional investigations that may help in determining diagnosis, and potentially prognosis and treatment., Conclusions: This update is a supplement to the previous 2002 IIP classification document. It outlines advances in the past decade and potential areas for future investigation.

Idiopathic pulmonary fibrosis is the most lethal form of diffuse lung fibrosis, killing approximately half of those affected within 2-3 years of diagnosis. Until recently, no therapies had been shown to have an impact on disease progression. The Clinical Studies Assessing Pirfenidone (Esbriet(®)) in IPF: Research of Efficacy and Safety Outcomes (CAPACITY) program comprised two almost identical double-blind placebo-controlled studies assessing the effects of pirfenidone on change in forced vital capacity, the primary end point, over a 72-week period. One of these studies was positive, matching in magnitude the benefit seen in two previous positive Japanese studies. The other study did not meet its primary end point but positive trends were consistent in this and a number of secondary end point indices. Safety was acceptable, comprising mainly problems of tolerability rather than toxicity. It is likely that pirfenidone will be utilised in many countries as first-line therapy and will also be included in studies of combination therapy for this attritional disease.

This document is an international evidence-based guideline on the diagnosis and management of idiopathic pulmonary fibrosis, and is a collaborative effort of the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association. It represents the current state of knowledge regarding idiopathic pulmonary fibrosis (IPF), and contains sections on definition and epidemiology, risk factors, diagnosis, natural history, staging and prognosis, treatment, and monitoring disease course. For the diagnosis and treatment sections, pragmatic GRADE evidence-based methodology was applied in a question-based format. For each diagnosis and treatment question, the committee graded the quality of the evidence available (high, moderate, low, or very low), and made a recommendation (yes or no, strong or weak). Recommendations were based on majority vote. It is emphasized that clinicians must spend adequate time with patients to discuss patients' values and preferences and decide on the appropriate course of action.

Rationale: The rapid diagnosis of pulmonary tuberculosis (TB) is difficult when acid fast bacilli (AFB) cannot be detected in sputum smears., Objectives: Following a proof of principle study, we examined in routine clinical practice whether individuals with sputum AFB smear-negative TB can be discriminated from those with latent TB infection by local immunodiagnosis with a Mycobacterium tuberculosis-specific enzyme-linked immunospot (ELISpot) assay., Methods: Subjects suspected of having active TB who were unable to produce sputum or with AFB-negative sputum smears were prospectively enrolled at Tuberculosis Network European Trialsgroup centers in Europe. ELISpot with early-secretory-antigenic-target-6 and culture-filtrate-protein-10 peptides was performed on peripheral blood mononuclear cells (PBMCs) and bronchoalveolar lavage mononuclear cells (BALMCs). M. tuberculosis-specific nucleic acid amplification (NAAT) was performed on bronchoalveolar lavage fluid., Measurements and Main Results: Seventy-one of 347 (20.4%) patients had active TB. Out of 276 patients who had an alternative diagnosis, 127 (46.0%) were considered to be latently infected with M. tuberculosis by a positive PBMC ELISpot result. The sensitivity and specificity of BALMC ELISpot for the diagnosis of active pulmonary TB were 91 and 80%, respectively. The BALMC ELISpot (diagnostic odds ratio [OR], 40.4) was superior to PBMC ELISpot (OR, 10.0), tuberculin skin test (OR, 7.8), and M. tuberculosis specific NAAT (OR, 12.4) to diagnose sputum AFB smear-negative TB. In contrast to PBMC ELISpot and tuberculin skin test, the BALMC ELISpot was not influenced by previous history of TB., Conclusions: Bronchoalveolar lavage ELISpot is an important advancement to rapidly distinguish sputum AFB smear-negative TB from latent TB infection in routine clinical practice.