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.

Essais cliniques au cours de la fibrose pulmonaire idiopathique: le choix des critères de jugement

Introduction La pneumopathie infiltrante diffuse (PID) est une association rare des vascularites associees aux ANCA (VAA) et est habituellement associee a la presence d’anticorps anti-MPO [1] . Cependant, l’impact des PID sur le pronostic des VAA n’est pas connu. L’objectif de ce travail etait de comparer des patients atteints de VAA avec PID a des patients presentant une VAA sans PID. Patients et methodes Une etude retrospective observationnelle multicentrique a ete menee de novembre 2016 a aout 2017 dans 22 centres appartenant au GFEV. Les patients inclus repondaient aux criteres diagnostiques de granulomatose avec polyangeite (GPA) ou de polyangeite microscopique (PAM) selon la nomenclature de Chapel Hill revisee en 2012. L’atteinte pulmonaire etait definie par une PID de type usuelle (UIP) ou non specifique (PINS) (criteres ATS/ERS de 2001) selon les resultats du scanner thoracique. Les patients presentant un autre type de PID ou une granulomatose eosinophilique avec polyangeite ont ete exclus de l’analyse. Ces patients ont ete compares a des patients atteints de VAA sans PID issus de la base de donnees du GFEV, apres appariement (2 temoins pour 1 cas) sur l’âge (≥ ou  Resultats Soixante-trois patients presentant une VAA avec PID ont ete compares a 126 patients atteints de VAA sans PID. Parmi les VAA avec PID, 52/63 (83 %) presentaient une PAM ; et le diagnostic de la PID etait prealable au diagnostic de vascularite dans 52 % des cas, concomitant dans 38 % des cas et plus tardif dans 10 % des cas (delai median = 1 an). Il s’agissait d’une UIP dans 63 % des cas et d’une PINS dans 35 % des cas. L’âge au diagnostic de VAA etait de 66 ans (57,2–74,7). La mediane de suivi etait plus courte dans le groupe VAA avec PID : 40 (21–61) vs 66 (36–133) mois (p  La survie 5 ans n’etait pas statistiquement differente entre VAA avec PID et VAA sans PID : 72 vs 81 % (p = 0,472). Toutefois, en prenant en compte le type d’atteinte pulmonaire, la survie des patients atteints de VAA avec UIP etait diminuee (62,3 % a 5 ans) par rapport aux VAA sans PID (80,9 % a 5 ans) et aux PINS (87,5 % a 5 ans) (p = 0,03). En analyse multivariee, les facteurs associes a une diminution de la survie etaient le sexe masculin (hazard ratio [HR] = 1,99 ; p = 0,021), l’existence au diagnostic de VAA d’une hemorragie alveolaire (HR = 2,43 ; p = 0,008), d’une atteinte renale (HR = 2,29 ; p = 0,029), d’un âge > 65 ans (HR = 6,2 ; p  Conclusion Cette etude montre la valeur pronostique pejorative de la PID de type UIP au cours des VAA. Cette association concerne essentiellement les PAM, laissant supposer qu’il existe un lien physiopathologique entre anticorps anti-MPO et PID au cours des VAA. L’effet benefique d’un traitement immunosuppresseur en induction ou en entretien en cas de VAA avec PID n’est pas retrouve dans notre etude, ce qui est probablement lie a un manque de puissance puisque tres peu de patients ont recu un traitement par corticoides seuls.

Fibrose pulmonaire au cours des vascularites associées aux ANCA : un facteur pronostique

[Rare pulmonary diseases: from the adoption of orphan diseases to structured healthcare networks].

Decline in Forced Vital Capacity (FVC) in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD) in Consecutive 6-Month Periods of the SENSCIS Trial

Interstitial lung disease (ILD) is a diverse group of inflammatory and fibrotic lung conditions causing significant morbidity and mortality. A multitude of factors beyond the lungs influence symptoms, health-related quality of life, disease progression, and survival in patients with ILD. Despite an increasing emphasis on multidisciplinary management in ILD, the absence of a framework for assessment and delivery of comprehensive patient care poses challenges in clinical practice. The treatable traits approach is a precision medicine care model that operates on the premise of individualised multidimensional assessment for distinct traits that can be targeted by specific interventions. The potential utility of this approach has been described in airway diseases but has not been adequately considered in ILD. Given the similar disease heterogeneity and complexity between ILD and airway diseases, we explore the concept and potential application of the treatable traits approach in ILD. A framework of aetiological, pulmonary, extra-pulmonary, and behavioural and lifestyle treatable traits relevant to clinical care and outcomes for patients with ILD is proposed. We further describe key research directions to evaluate the application of the treatable traits approach towards advancing patient care and health outcomes in ILD.

Vincent Cottin

Papers

Essais cliniques au cours de la fibrose pulmonaire idiopathique: le choix des critères de jugement

Fibrose pulmonaire au cours des vascularites associées aux ANCA : un facteur pronostique

[Rare pulmonary diseases: from the adoption of orphan diseases to structured healthcare networks].

Decline in Forced Vital Capacity (FVC) in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD) in Consecutive 6-Month Periods of the SENSCIS Trial

Treatable traits: a comprehensive precision medicine in interstitial lung disease.