Respiratory bronchiolitis-interstitial lung disease in first-degree relatives

SummaryThese two case reports examine the histories of a father and daughter with respiratory bronchiolitis-interstitial lung disease. This unusual condition occurs almost exclusively in smokers and has never previously been reported in first-degree relatives

Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: An international case-cohort study

We conducted an international study of idiopathic pulmonary fibrosis (IPF) diagnosis among a large group of physicians and compared their diagnostic performance to a panel of IPF experts.A total of 1141 respiratory physicians and 34 IPF experts participated. Participants evaluated 60 cases of interstitial lung disease (ILD) without interdisciplinary consultation. Diagnostic agreement was measured using the weighted kappa coefficient (κw). Prognostic discrimination between IPF and other ILDs was used to validate diagnostic accuracy for first-choice diagnoses of IPF and were compared using the C-index.A total of 404 physicians completed the study. Agreement for IPF diagnosis was higher among expert physicians (κw=0.65, IQR 0.53–0.72, p less than 0.0001) or physicians with access to multidisciplinary team (MDT) meetings (κw=0.54, IQR 0.45–0.64, p less than 0.0001). The prognostic accuracy of academic physicians with greater than 20 years of experience (C-index=0.72, IQR 0.0–0.73, p=0.229) and non-university hospital physicians with more than 20 years of experience, attending weekly MDT meetings (C-index=0.72, IQR 0.70–0.72, p=0.052), did not differ significantly (p=0.229 and p=0.052 respectively) from the expert panel (C-index=0.74 IQR 0.72–0.75).Experienced respiratory physicians at university-based institutions diagnose IPF with similar prognostic accuracy to IPF experts. Regular MDT meeting attendance improves the prognostic accuracy of experienced non-university practitioners to levels achieved by IPF experts

High-resolution CT phenotypes in pulmonary sarcoidosis: a multinational Delphi consensus study

One view of sarcoidosis is that the term covers many different diseases. However, no classification framework exists for the future exploration of pathogenetic pathways, genetic or trigger predilections, patterns of lung function impairment, or treatment separations, or for the development of diagnostic algorithms or relevant outcome measures. We aimed to establish agreement on high-resolution CT (HRCT) phenotypic separations in sarcoidosis to anchor future CT research through a multinational two-round Delphi consensus process. Delphi participants included members of the Fleischner Society and the World Association of Sarcoidosis and other Granulomatous Disorders, as well as members' nominees. 146 individuals (98 chest physicians, 48 thoracic radiologists) from 28 countries took part, 144 of whom completed both Delphi rounds. After rating of 35 Delphi statements on a five-point Likert scale, consensus was achieved for 22 (63%) statements. There was 97% agreement on the existence of distinct HRCT phenotypes, with seven HRCT phenotypes that were categorised by participants as non-fibrotic or likely to be fibrotic. The international consensus reached in this Delphi exercise justifies the formulation of a CT classification as a basis for the possible definition of separate diseases. Further refinement of phenotypes with rapidly achievable CT studies is now needed to underpin the development of a formal classification of sarcoidosis

Prevalence of airway and parenchymal abnormalities in newly diagnosed rheumatoid arthritis

SummaryBackgroundPulmonary disease is a well recognised and important extra-articular manifestation of rheumatoid arthritis (RA). The objective of this study was to determine the prevalence of airway and parenchymal abnormalities in newly diagnosed patients with RA and to correlate these with clinical measures of RA severity and laboratory tests.Methods60 patients with a new (symptom duration <12 months) diagnosis of RA (43 females, 42 European, mean age 54, 33 ever smoker, (17 current) underwent lung function testing and high resolution computed tomography (HRCT) scored by two independent radiologists.ResultsEighteen (30%) patients reported respiratory symptoms: dyspnoea (11), cough (11), and wheeze (8). Twelve (20%) patients had physiologic evidence of airflow obstruction and 24 (40%) had reduced gas transfer. The prevalence of HRCT abnormalities (in any lobe) was as follows: decreased attenuation 67%, bronchiectasis 35%, bronchial wall thickening 50%, ground glass opacification 18%, reticular changes 12%. All abnormalities were more common in the lower lobes. With the exception of reduced DLCO, there were no significant differences in the prevalence of HRCT patterns or lung function parameters between smokers and non smokers. Anti-CCP antibodies and rheumatoid factor (RF) correlated strongly with DLCO and variably with other physiologic measures but poorly with radiologic abnormalities.ConclusionPatients with newly diagnosed RA have a moderate prevalence of airway and parenchymal abnormalities on HRCT and lower than predicted lung function parameters which cannot entirely be explained by smoking. These data suggest that pulmonary involvement is present early in the disease course in RA