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.

Monocytes sanguins : un nouveau biomarqueur pronostique de la fibrose pulmonaire progressive ?

PURPOSE OF REVIEW
Pulmonary hypertension associated with interstitial lung disease (ILD-PH) is associated with significant alteration of quality of life, exercise capacity, and survival. Over the past 2 years, there were changes in the guideline definition and classification of ILD-PH, and positive randomized controlled trials were published.


RECENT FINDINGS
Pulmonary hypertension associated with chronic lung disease is now hemodynamically defined as a mean pulmonary artery pressure more than 20 mmHg, with pulmonary artery wedge pressure 15 mmHg or less, and pulmonary vascular resistance (PVR) at least 2 Wood units. Severe ILD-PH is defined by PVR more than 5 Wood units. In the INCREASE trial, patients receiving inhaled treprostinil had favorable significant changes in 6-min walk distance, NT-proBNP level, clinical worsening events, and forced vital capacity, which were maintained in the open label extension study. Promising results were obtained in a placebo-controlled pilot trial using escalated doses of inhaled nitric oxide. According to European guidelines, patients with ILD-PH should be referred to pulmonary hypertension centers, where inhaled treprostinil may be considered; phosphodiesterase type-5 inhibitors may also be considered in patients with severe ILD-PH.


SUMMARY
Recent changes in the definitions and a new therapeutic option have an impact on the diagnosis and management of ILD-PH.

Vincent Cottin

Papers

Monocytes sanguins : un nouveau biomarqueur pronostique de la fibrose pulmonaire progressive ?

Interstitial lung disease-associated pulmonary hypertension - what the future holds.

BOEHRINGER INGELHEIM - Fibroses pulmonaires : maladies pulmonaires ou maladies systémiques ?

Impact de la pollution atmosphérique sur la survie de la fibrose pulmonaire idiopathique selon le polymorphisme MUC5B

Quand suspecter une HTAP