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

https://www.thelancet.com/pdfs/journals/lancet/PIIS0140-6736(17)30866-8.pdf

Idiopathic pulmonary fibrosis

The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF. This perspective is the result of an international effort to summarize the current state of knowledge regarding acute exacerbations of IPF. Acute exacerbations of IPF are defined as acute, clinically significant deteriorations of unidentifiable cause in patients with underlying IPF. Proposed diagnostic criteria include subjective worsening over 30 days or less, new bilateral radiographic opacities, and the absence of infection or another identifiable etiology. The potential pathobiological roles of infection, disordered cell biology, coagulation, and genetics are discussed, and future research directions are proposed.

/pdf/acute-exacerbations-of-idiopathic-pulmonary-fibrosis-4v5lmmsvxz.pdf

Acute Exacerbations of Idiopathic Pulmonary Fibrosis

### 1.1 An overview of the ILD guideline

Since the publication of the first BTS guidelines for diffuse lung disease nearly 10 years ago,1 the specialty has seen considerable change. The early discussions of the Guideline Group centred upon whether the revised document might consist of the 1999 document with minor adaptations. However, it was considered that too much change had taken place in the intervening years to justify a simple editorial approach. The last decade had seen the development of a new consensus terminology for the idiopathic interstitial pneumonias (IIP)2 stimulated, in part, by the identification of non-specific interstitial pneumonia (NSIP) as a discrete histological pattern with increasingly recognised clinical correlates.3 The better prognosis seen in fibrotic NSIP than in idiopathic pulmonary fibrosis (IPF)4 5 fuelled a more intense approach to diagnosis in cases of suspected IPF. This in turn has led to a radical change in accepted diagnostic gold standards, which have become increasingly multidisciplinary and dependent equally upon the skills of pathologists, radiologists and clinicians.6 NSIP, as a new entity, has posed particular difficulties. With more detailed studies of outcome specific to individual IIPs, especially IPF and fibrotic NSIP, the prognostic weighting given to pulmonary function impairment has been refined, especially with regard to longitudinal functional trends.7 Most important, with the standardisation of terminology, it became possible to recruit patients into multicentre treatment studies.8–10 With regard to IPF in particular, the last 3 years have seen more studies of treatment than in the previous history of the speciality, yet there is no universally accepted “best current treatment”.

### 1.2 Methodology for constructing guidelines and making recommendations

The overall process for generating BTS guidelines has been addressed in numerous recently published documents (available at http://www.brit-thoracic.org.uk/guidelines.html). The methodology applied herein is broadly similar and is not therefore described in detail. However, there are specific aspects in the …

https://thorax.bmj.com/content/thoraxjnl/63/Suppl_5/v1.full.pdf

Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society.

OBJECTIVES: To report the methods and findings of two complete autopsies of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) positive individuals who died in Oklahoma (United States) in March 2020. METHODS: Complete postmortem examinations were performed according to standard procedures in a negative-pressure autopsy suite/isolation room using personal protective equipment, including N95 masks, eye protection, and gowns. The diagnosis of coronavirus disease 2019 (COVID-19) was confirmed by real-time reverse transcriptase polymerase chain reaction testing on postmortem swabs. RESULTS: A 77-year-old obese man with a history of hypertension, splenectomy, and 6 days of fever and chills died while being transported for medical care. He tested positive for SARS-CoV-2 on postmortem nasopharyngeal and lung parenchymal swabs. Autopsy revealed diffuse alveolar damage and chronic inflammation and edema in the bronchial mucosa. A 42-year-old obese man with a history of myotonic dystrophy developed abdominal pain followed by fever, shortness of breath, and cough. Postmortem nasopharyngeal swab was positive for SARS-CoV-2; lung parenchymal swabs were negative. Autopsy showed acute bronchopneumonia with evidence of aspiration. Neither autopsy revealed viral inclusions, mucus plugging in airways, eosinophils, or myocarditis. CONCLUSIONS: SARS-CoV-2 testing can be performed at autopsy. Autopsy findings such as diffuse alveolar damage and airway inflammation reflect true virus-related pathology; other findings represent superimposed or unrelated processes.

Joseph G. Parambil

Papers

Interstitial lung disease in primary Sjögren syndrome.

Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy.

Causes and prognosis of diffuse alveolar damage diagnosed on surgical lung biopsy.

Diffuse Alveolar Damage: Uncommon Manifestation of Pulmonary Involvement in Patients With Connective Tissue Diseases

Lack of evidence for an association between neurofibromatosis and pulmonary fibrosis.