Leticia Kawano-Dourado

Background: Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is heterogeneous. Forced vital capacity (FVC) decline and high-resolution CT (HRCT) pattern are widely used as prognostic markers, but the natural history of RA-ILD identified in rheumatology clinics, particularly in mild disease, remains insufficiently described. Methods: We conducted a prospective, multicentre cohort study across three Brazilian centres. Adults with RA-ILD were followed every 6 months for 24 months. Pulmonary function tests, HRCT, 6 min walk test and King’s Brief Interstitial Lung Disease health-related quality-of-life questionnaire were performed at baseline, 12 and 24 months. Blood biomarkers were assessed at baseline, 6 and 18 months. HRCT scans were independently reviewed by two chest radiologists and analysed using densitometry-based quantitative CT (QCT). Pulmonary fibrosis was qualitatively defined by unequivocal traction bronchiectasis and/or honeycombing on HRCT. Results: Ninety-five subjects were included (mean age 63±10 years; 81% female). Pulmonary fibrosis was present in 80%, although only 20% were classified as definite or probable usual interstitial pneumonia (κ=0.52). Individuals with fibrosis had lower FVC (75%±17 vs 86%±15, p=0.02) and worse QCT measures (%high-attenuation areas −600 to −250 HU 12.1±6.6 vs 5.5±0.8, p<0.001; lung volume 3407±958 mL vs 4077±923 mL, p=0.015). In 24 months, FVC and QCT measures remained stable. Eight subjects (8%) died. Deaths were not preceded by measurable functional or radiological decline. Health-related quality-of-life scores and blood biomarkers remained unchanged. Conclusions: In this predominantly mild RA-ILD cohort, pulmonary function and QCT metrics remained stable over 2 years despite an 8% mortality rate. The absence of preceding decline in conventional prognostic markers suggests potential limitations of current monitoring strategies, although our findings should be interpreted as hypothesis-generating. Trial registration number: NCT04136223 .

Background: Shorter leukocyte telomere length (LTL) has been reported in patients with rheumatoid arthritis (RA)-associated interstitial lung disease (ILD) and linked to increased disease severity and mortality in idiopathic pulmonary fibrosis, which shares similarities with RA-ILD. We aimed to evaluate the impact of short LTL on baseline respiratory disease severity, disease progression and survival in patients with RA-ILD. Methods: Patients diagnosed with RA-ILD following multidisciplinary assessment were enrolled in a prospective French observational study. LTL was measured at enrolment using qPCR. Short LTL was defined as age-adjusted LTL D _LCO ) ≥10% predicted). Results: Among 101 patients with RA-ILD, 46% were male, mean± sd age at enrolment was 66±10 years and 43 (43%) had short LTL. Patients with short LTL had lower FVC % predicted (82% versus 93%) and D _LCO % predicted (49% versus 63%) at enrolment, and greater 12-month decline in FVC % predicted and D _LCO % predicted in mixed effects models (−7.7% (95% CI −11.6– −3.8%); pversus 22%; univariate p=0.011) and lower FVC at enrolment. Multivariate logistic regression identified lower FVC and short LTL as predictors of 12-month progression (OR 0.97 (95% CI 0.94–1.00); p=0.031 and OR 2.80 (95% CI 0.99–8.29); p=0.056, respectively). Conclusion: Short LTL is associated with baseline severity and 12-month progression in RA-ILD.

Background: The 2013 American Thoracic Society/European Respiratory Society Statement on the classification of the idiopathic interstitial pneumonias described 6 major and 2 rare subtypes of idiopathic interstitial pneumonia, as well as recognising unclassifiable disease. Objective: The objective of this statement is to update the 2013 classification of interstitial pneumonia. Methods: Five co-chairs identified a committee of 32 experts in the field as well as two individuals with lived experience. Creation of the document was supported by a series of video meetings, first including the full committee and then subgroups assigned to draft specific sections of the document. The classification scheme was developed by consensus. Results: The multidisciplinary committee of experts identified four major advances to the classification of interstitial pneumonia: (1) expansion beyond idiopathic interstitial pneumonias to also include secondary causes; (2) identification of new subcategories and updated terms, including addition of bronchiolocentric interstitial pneumonia as a major pattern as well as changing from acute interstitial pneumonia to idiopathic diffuse alveolar damage and desquamative interstitial pneumonia to alveolar macrophage pneumonia; (3) subclassification of interstitial and alveolar filling disorders, with interstitial disorders further subclassified as fibrotic versus non-fibrotic; and (4) consideration of diagnostic confidence in patient evaluation and management. The committee also provided a comprehensive update on the status of potential molecular tools and identified future research priorities. Conclusions: This update builds upon the previous classification approach by describing major advances in the classification of interstitial pneumonia over the last decade.

INTRODUCTION Clinical practice guidelines (CPGs) facilitate evidence-based care.() In critical care settings, where patients are often in life-threatening conditions and need fast and efficient care, trustworthy CPGs provide recommendations on the basis of the best current research evidence. For example, guidelines such as the Surviving Sepsis Campaign offer detailed recommendations on the timely administration of […] INTRODUCTION Clinical practice guidelines (CPGs) facilitate evidence-based care.() In critical care settings, where patients are often in life-threatening conditions and need fast and efficient care, trustworthy CPGs provide recommendations on the basis of the best current research evidence. For example, guidelines such as the Surviving Sepsis Campaign offer detailed recommendations on the timely administration of […]