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

/pdf/2015-esc-ers-guidelines-for-the-diagnosis-and-treatment-of-y26yabnnom.pdf

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).

Each chapter listed in the Table of Contents (see next page) is a hyperlink to that chapter. The reader clicks the chapter name to access that chapter.

Each chapter listed here is a hyperlink. Click on the chapter name to be taken to that chapter. 

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.

Systemic sclerosis (SSc) is an autoimmune disease associated to fibrotic manifestations. Interstitial lung disease (SSc-ILD), one of the main fibrotic features of SSc, is the first cause of SSc-related death. The management of SSc-ILD has recently benefited from the results of key randomised controlled trials. French authorities have approved Nintedanib for the treatment of SSc-ILD, and tocilizumab has recently been approved by the Food and Drug Administration (FDA) in the United-States (US). These recent approvals challenge the management of this fibrotic manifestation of SSc. This narrative literature review, at the crossroad of internal medicine and pulmonology, discusses what could be an up-to date approach, in terms of diagnostic and therapeutic strategies for SSc-ILD, in the light of the results from recent clinical trials. 

[Systemic sclerosis-related interstitial lung disease: Diagnostic and therapeutic strategy in the light of recent clinical trials].

La granulomatose éosinophilique avec polyangéite (GEPA) est une vascularite appartenant au groupe des vascularites nécrosantes associées aux anticorps anti-cytoplasme des polynucléaires neutrophiles (ANCA). Il n’y a pas de données épidémiologiques de la GEPA en France portant sur l’ensemble des patients affiliés au régime général. Nous avons donc voulu estimer la prévalence et l’incidence de la GEPA à partir des données issues du Système National des Données de Santé (SNDS). Les patients atteints de GEPA identifiés entre le 01/01/2013 et le 31/12/2019 dans le SNDS ont été inclus, afin de définir les cas prévalents et les cas incidents. L’historique des patients a été analysé depuis le 01/01/2010 afin de définir les cas prévalents et les cas incidents. Les patients inclus devaient être affiliés au régime général et avoir soit une ALD M30 stricte ou M301 soit une hospitalisation avec un code M301 (périartérite avec atteinte pulmonaire [Churg–Strauss]), représentant la population d’analyse principale. Une analyse de sensibilité a été faite dans une population limitée aux patients ayant eu au moins 3 dispensations de glucocorticoïdes oraux dans l’année suivant le diagnostic. Les cas prévalents atteints de GEPA représentaient 7238 individus, tandis que les cas incidents représentaient 1650 individus dans l’analyse principale. Dans l’analyse de sensibilité, les cas prévalents et incidents étaient de 3328 et 1035 individus, respectivement. La prévalence et l’incidence de la GEPA dans l’analyse principale étaient de 85,3 et 3,5 cas par million, respectivement, tandis qu’elles étaient de 38,4 et 2,1 cas par million, respectivement, dans l’analyse de sensibilité. Il n’y avait pas de différence saisonnière dans les diagnostics de GEPA. Dans la population de cas incidents, l’âge moyen à l’inclusion était de 61,7 ± 17,4 ans dans l’analyse principale et 62,4 ± 15,9 ans dans l’analyse de sensibilité, avec une légère prédominance féminine, les femmes représentant 57,1 % et 54,0 % dans les analyses principale et de sensibilité, respectivement. Dans les 2 ans précédant le diagnostic de GEPA, 76,7 % des patients avaient eu au moins une dispensation de glucocorticoïdes oraux et 53,9 % des corticoïdes inhalés dans l’analyse principale, et 89,5 % et 68,4 % dans l’analyse de sensibilité, respectivement. Des événements ORL étaient signalés dans les 2 ans précédant le diagnostic de GEPA chez 62,7 % et 71,8 % des patients dans les analyses principale et de sensibilité. Cette étude épidémiologique sur la GEPA en France permet d’estimer la prévalence et l’incidence de la maladie à partir des données issues du SNDS. La prévalence est plus élevée que dans les études antérieures, posant la question soit d’un sur-diagnostic de la GEPA en particulier en cas d’asthme éosinophilique, soit d’une augmentation de la prévalence ces dernières années. Elle apporte également des informations sur la démographie de la maladie et les comorbidités et traitements reçus avant le diagnostic de GEPA (cette recherche a été menée avec le soutien d’AstraZeneca). 

Vincent Cottin

Papers

[Systemic sclerosis-related interstitial lung disease: Diagnostic and therapeutic strategy in the light of recent clinical trials].

Prévalence et incidence de la granulomatose éosinophilique avec polyangéite et description des cas incidents en France

Hypertension pulmonaire et maladies infiltrantes pulmonaires

Anticorps monoclonaux et pneumopathies interstitielles

Bronchiolite chronique idiopathique : cohorte française rétrospective multicentrique