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.

SFTPC mutations in patients with familial pulmonary fibrosis: combined with emphysema?

A 48-yr-old nonsmoking female presented with a 5-yr history of recurrent bronchitis with progressive exercise dyspnoea. She had been diagnosed with a small monoclonal gammopathy of undermined significance (MGUS) 6 yrs previously. Inspiratory vital capacity was 4.7 L (125% predicted) and forced expiratory volume in one second/forced vital capacity (FVC) ratio was 79%. Carbon monoxide transfer factor was markedly impaired (35% pred), and arterial oxygen tension ( P a,O2) decreased from 9.2 to 6.0 kPa at exercise (40 W, 10 min). A thoracic computed tomography (CT) scan was performed (fig. 1⇓). Fibreoptic bronchoscopy found inflammation of the bronchial mucosa with candle stain-like deposits on the trachea and large bronchi, without any purulent secretion within the respiratory tract (fig. 2⇓). The histopathological aspect of bronchial biopsies is shown in figure 3⇓. Blood tests disclosed type I cryoglobulinaemia composed of a 32 g·L−1 monoclonal immunoglobulin (Ig)Mκ serum component (at 37°C). Blood cell count was normal. Bone marrow examination showed malignant lymphoplasmacytic cells representing 30% of total cells, with a monotypic surface expression of IgMκ. The C-reactive protein level was 21 mg·L−1. Extensive renal, cardiac and hepatic evaluations were normal. As severe hypoxaemia and reduction of carbon monoxide transfer factor were unexplained, the patient underwent videothoracoscopic lung biopsies.



Fig. 1— 
Thoracic computed tomography scan.





Fig. 2— 
Fibreoptic bronchoscopy. Arrow shows candle stain-like deposits.





Fig. 3— 
a) Bronchial biopsy of candle-stain like lesions stained by haematoxylin-eosin-saffron. Submucosal abnormal deposits with concentric pattern around the vessels (black arrow), arranging in cords or ribbon-like structures in the interstitium (black dashed arrow) or in an organoid, raspberry-like fashion in smooth muscle fascicles (black arrowhead). b) Bronchial biopsy; perivascular deposits with negative Congo red dye staining. c) Direct anti-κ-light chain immunofluorescence in bronchial biopsy showing positivity of perivascular (white arrow) and interstitial (white dashed arrow) deposits, and epithelial …

https://erj.ersjournals.com/content/erj/31/6/1374.full.pdf

Respiratory failure with diffuse bronchiectases and cryoglobulinaemia.

Better survival in combined pulmonary fibrosis and emphysema than in lone pulmonary fibrosis: bias or reality? A response to Centrilobular emphysema combined with pulmonary fibrosis results in improved survival by Todd et al., Fibrogenesis & Tissue Repair 2011, 4:6.

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Centrilobular emphysema combined with pulmonary fibrosis results in improved survival: a response

A syndrome including upper-lobe emphysema and pulmonary fibrosis of the lower lungs was recently characterized. It is found most often in men who are smokers or ex-smokers of more than 40 pack-years; their mean age is 65 years. Exertional dyspnea is always present. There are basal crackles. The disease has no known cause; the only certain risk factor is smoking. Pulmonary function tests show respiratory volumes and flows that are often normal or subnormal, while carbon monoxide transfer is substantially reduced and exercise hypoxemia is present. Diagnosis is based on findings from millimeter-slices of computed tomography of the chest, which show either centrilobular emphysema or upper-zone bullous emphysema, associated in 90% of cases with very suggestive paraseptal emphysema and diffuse infiltrating fibrosing lung disease at the bases (subpleural reticular opacities, honeycomb images, traction bronchiectasis), with more frequent ground glass opacities than in idiopathic pulmonary fibrosis. Pulmonary hypertension is present in almost half of all patients and represents the principal negative prognostic factor for this condition, which has a median survival of 6 years.

Syndrome d'emphysème des sommets et fibrose pulmonaire des bases combinés

Introduction: Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease that primarily affects older individuals. Nintedanib, a tyrosine kinase inhibitor, has been approved for the treatment of IPF in several countries. The safety and tolerability of nintedanib have been investigated in clinical trials and in real-world studies (compassionate use programs and post-marketing surveillance).Areas covered: Most frequent adverse events reported in patients treated with nintedanib (gastrointestinal); additional adverse events of special interest (elevations in liver enzymes, bleeding, cardiovascular adverse events); recommendations for managing adverse events.Expert opinion: Experience with nintedanib in real-world clinical practice suggests that it has a safety and tolerability profile consistent with that observed in clinical trials. Upon initiation of nintedanib, patient education, regular monitoring and proactive management of adverse events such as diarrhea are needed to minimi...

Vincent Cottin

Papers

SFTPC mutations in patients with familial pulmonary fibrosis: combined with emphysema?

Respiratory failure with diffuse bronchiectases and cryoglobulinaemia.

Centrilobular emphysema combined with pulmonary fibrosis results in improved survival: a response

Syndrome d'emphysème des sommets et fibrose pulmonaire des bases combinés

The safety and tolerability of nintedanib in the treatment of idiopathic pulmonary fibrosis