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.

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

ALAT
: alanine aminotransferase
ASAT
: aspartate aminotransferase
APAH
: associated pulmonary arterial hypertension
BAS
: balloon atrial septostomy
BMPR2
: bone morphogenetic protein receptor 2
BNP
: brain natriuretic peptide
BPA
: balloon pulmonary angioplasty
BREATHE
: Bosentan

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2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).

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Each year, the American Heart Association (AHA), in conjunction with the Centers for Disease Control and Prevention, the National Institutes of Health, and other government agencies, brings together in a single document the most up-to-date statistics related to heart disease, stroke, and the cardiovascular risk factors listed in the AHA’s My Life Check - Life’s Simple 7 (Figure1), which include core health behaviors (smoking, physical activity, diet, and weight) and health factors (cholesterol, blood pressure [BP], and glucose control) that contribute to cardiovascular health. The Statistical Update represents …

Heart Disease and Stroke Statistics-2018 Update: A Report From the American Heart Association.

Summary Background Since December, 2019, Wuhan, China, has experienced an outbreak of coronavirus disease 2019 (COVID-19), caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Epidemiological and clinical characteristics of patients with COVID-19 have been reported but risk factors for mortality and a detailed clinical course of illness, including viral shedding, have not been well described. Methods In this retrospective, multicentre cohort study, we included all adult inpatients (≥18 years old) with laboratory-confirmed COVID-19 from Jinyintan Hospital and Wuhan Pulmonary Hospital (Wuhan, China) who had been discharged or had died by Jan 31, 2020. Demographic, clinical, treatment, and laboratory data, including serial samples for viral RNA detection, were extracted from electronic medical records and compared between survivors and non-survivors. We used univariable and multivariable logistic regression methods to explore the risk factors associated with in-hospital death. Findings 191 patients (135 from Jinyintan Hospital and 56 from Wuhan Pulmonary Hospital) were included in this study, of whom 137 were discharged and 54 died in hospital. 91 (48%) patients had a comorbidity, with hypertension being the most common (58 [30%] patients), followed by diabetes (36 [19%] patients) and coronary heart disease (15 [8%] patients). Multivariable regression showed increasing odds of in-hospital death associated with older age (odds ratio 1·10, 95% CI 1·03–1·17, per year increase; p=0·0043), higher Sequential Organ Failure Assessment (SOFA) score (5·65, 2·61–12·23; p Interpretation The potential risk factors of older age, high SOFA score, and d-dimer greater than 1 μg/mL could help clinicians to identify patients with poor prognosis at an early stage. Prolonged viral shedding provides the rationale for a strategy of isolation of infected patients and optimal antiviral interventions in the future. Funding Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences; National Science Grant for Distinguished Young Scholars; National Key Research and Development Program of China; The Beijing Science and Technology Project; and Major Projects of National Science and Technology on New Drug Creation and Development.

Clinical course and risk factors for mortality of adult inpatients with COVID-19 in Wuhan, China: a retrospective cohort study

Updated clinical classification of pulmonary hypertension.

Nader Chebib, Vincent Cottin, Martine Taharo-Ag-Ralissoum, Michel Chuzeville and Jean-François Mornex Hospices Civils de Lyon, National Reference Center for Rare Pulmonary Diseases, Competence Center for Pulmonary Hypertension, Lyon, France; Claude Bernard Lyon 1 University, University of Lyon, Lyon, France; Hospices Civils de Lyon, Hôpital Edouard Herriot, Cardiogeriatrics Department, Lyon, France

https://journals.sagepub.com/doi/pdf/10.1177/2045893217753352

Epoprostenol discontinuation in patients with pulmonary arterial hypertension: a complex medical and social problem.

Pirfenidone post-authorization safety registry (PASSPORT) update

Heterogeneity in the extent and progression of lung fibrosis probably reflects differences in the underlying pathogenic mechanisms. A growing understanding of the key pathogenic drivers of lung fibrosis and of CTDs (including the role of tobacco smoking) might lead to the development of more effective targeted therapies. The key goals of the clinical assessment of patients with both ILD and CTD are the detection of ILD and prognostic evaluation to determine which patients should be treated and how. Recent developments have moved the field forward. Several studies have provided significant advances, especially regarding HRCT-pathologic correlations, prediction of the outcome based on imaging and/or pathology, and the relevance of subtle clinical, serologic and morphologic imaging/pathological findings not fulfilling the criteria for CTDs (a condition now called ‘interstitial pneumonia with autoimmune features’). Demonstration of the efficacy of antifibrotic drugs to slow disease progression in idiopathic pulmonary fibrosis has raised hope that these or other drugs might be effective in fibrotic ILD associated with CTD and foster clinical research. This thematic series sets the scene of CTD principles for a series of papers that will focus on the investigation of lung involvement in rheumatologic disease, the investigation of CTD in ILD, biomarkers, treatment considerations, acute exacerbation and monitoring of lung involvement in rheumatologic disease from authorities in the field. Lung involvement in autoimmune rheumatic diseases is a common manifestation and a major cause of mortality and morbidity. Involvement may include lung parenchyma, airways, pleura, vessels and respiratory muscles. Main differential diagnoses include rheumatic disease manifestation, drug-induced manifestation or infection. Interstitial lung disease (ILD) in connective tissue disease (CTD) can be characterized using the classification of the idiopathic interstitial pneumonias. CTDs as a whole may develop all histologic patterns observed in idiopathic interstitial pneumonias. Systemic sclerosis is most frequently associated with ILD and, in most of these patients, ILD manifests as a histological pattern of nonspecific interstitial pneumonia. Conversely, in rheumatoid arthritis, the pattern of ILD seems to be most often usual interstitial pneumonia. Histologic patterns may be combined, as exemplified by the frequent association of ILD with lymphocytic bronchiolitis in rheumatoid arthritis. Occasionally, lung involvement can present before the diagnosis of CTD, especially in idiopathic inflammatory myopathy and rheumatoid arthritis. In order to decrease mortality, there is a need for early, exact diagnosis and effective treatment. Cooperation of experienced physicians for possible complications, including infections, drug-induced adverse effects, direct or indirect pulmonary or cardiovascular complications, is of paramount importance for the best management. Published online: June 24, 2015

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Lung Involvement in Rheumatic Disease: Introduction.

Lymphangioleiomyomatosis (LAM) is a rare (orphan) lung disease, in which the lung parenchyma is progressively replaced by cysts associated with a proliferation of immature smooth muscle cells and perivascular epithelioid cells (so-called LAM cells) around lymphatic vessels [1, 2]. LAM can be sporadic, almost exclusively affecting females, or can be associated with tuberous sclerosis complex (TSC) where it affects 30–50% of adult females, and less frequently males [3–6].

Patients with LAM most commonly present with relapsing pneumothorax and dyspnoea on exertion, and may have angiomyolipomas (benign tumours) of the kidneys and, occasionally, chylothorax or chylous ascites. Computed tomography of the chest shows characteristic multiple, round thin-walled cysts evenly distributed throughout the lung parenchyma [7], possibly associated with pneumothorax, pleural effusion, lymphadenopathies or lymphangiomas. Over time the disease may progress to chronic obstructive respiratory failure that may require lung transplantation. Pulmonary hypertension is very rare [8]. Diagnostic criteria and management guidelines have been proposed by a task force of the European Respiratory Society. Furthermore, the serum level of vascular endothelial growth factor (VEGF)-D, a major growth factor for lymphatic vessels, may further contribute to the noninvasive diagnosis of LAM if elevated [9].

For many years, treatment of LAM has been mostly supportive, including bronchodilators, supplemental oxygen (if needed), management of complications (especially pneumothorax) and lung transplantation. With a high variability of disease progression [10], many patients with LAM do not need a treatment intervention, whereas a minority of patients do progress to respiratory failure. In 2000, a major breakthrough in the understanding of the pathophysiology of the disease led to a novel treatment approach 10 years later [11]. Based on the observation that pulmonary LAM is present in patients with TSC [4], …

https://erj.ersjournals.com/content/erj/43/4/966.full.pdf

Treatment of lymphangioleiomyomatosis: building evidence in orphan diseases.

Resume La fibrose pulmonaire idiopathique (FPI) caracterisee par un aspect histopathologique de pneumopathie interstitielle commune est la plus frequente et la plus grave des PID idiopathiques. Aucun traitement n'a montre a ce jour une amelioration de la survie au cours de la FPI. Les voies therapeutiques a l'etude reposent sur le role essentiel des interactions entre l'epithelium alveolaire pulmonaire et les fibroblastes. L'endotheline-1 favorise la fibrogenese in vitro. Le bosentan, antagoniste de l'endotheline-1, presente des proprietes antifibrosantes in vitro et chez l'animal. Si l'essai clinique BUILD-1 n'a pas montre d'amelioration du perimetre de marche en 6 minutes par le bosentan au cours de la FPI, il indiquait une tendance non significative en faveur d'une amelioration de la survie sans progression, et surtout une amelioration de la survie chez les patients dont le diagnostic avait ete confirme par biopsie pulmonaire chirurgicale et (ou) ayant peu de rayons de miel a l'imagerie. Une PID cliniquement significative survient chez un quart des patients atteints de sclerodermie systemique, le plus souvent avec un aspect histopathologique de pneumopathie interstitielle non specifique souvent fibreuse. L'imagerie montre initialement des opacites en verre depoli qui evoluent vers la fibrose. L'efficacite modeste du cyclophosphamide demontree a court terme n'est pas confirmee a 2 ans. L'essai therapeutique BUILD-2 n'a pas montre d'amelioration par le bosentan de la PID de la scleodermie. Des etudes complementaires sont necessaires concernant l'effet du bosentan sur la survie de la FPI a un stade precoce, et au cours des formes evolutives de la PID de la sclerodermie.

Vincent Cottin

Papers

Epoprostenol discontinuation in patients with pulmonary arterial hypertension: a complex medical and social problem.

Pirfenidone post-authorization safety registry (PASSPORT) update

Lung Involvement in Rheumatic Disease: Introduction.

Treatment of lymphangioleiomyomatosis: building evidence in orphan diseases.

Bosentan et fibrose pulmonaire