TOPLINE

Interstitial lung disease (ILD) occurred in 39% of anti-Ku antibody-positive patients with systemic autoimmune diseases and progressed in 75% of them over a median follow-up of 6 years. Fibrotic changes developed in more than 90% of progressors, and baseline pulmonary fibrosis was associated with reduced survival.

METHODOLOGY

• A retrospective observational study was conducted across 10 French University Hospitals between January 2010 and June 2025, including 154 anti-Ku antibody-positive patients with suspected systemic autoimmune diseases.
• A total of 60 patients (39%) had ILD (median age at ILD diagnosis, 58 years; 68% women); those without ILD were considered as control individuals.
• The primary outcome was ILD progression, defined using a modified criteria inspired by specific guidelines, and included at least two of three criteria: (i) worsening respiratory symptoms, (ii) disease progression (absolute decline in either forced vital capacity ≥ 5% predicted or diffusing capacity of the lung for carbon monoxide corrected for hemoglobin concentration ≥10% predicted), and (iii) radiologic evidence of progression. 
• All chest CT scans were reviewed in a double-blind manner by two expert radiologists to determine ILD patterns and extent. Patients were followed for a median of 6 years.

TAKEAWAY

• Among patients with ILD, 75% experienced ILD progression, with the first episode of progression at a median duration of 4 years; male sex was associated with ILD progression (adjusted hazard ratio [HR], 2.7; 95% CI, 1.4-5.2).
• Fibrotic non-specific interstitial pneumonia was the most common chest CT pattern at baseline (27%), and 57% of patients already displayed predominant fibrosis pattern.
• Radiologic progression most often manifested as new fibrotic lesions or extension of pre-existing fibrosis, and 93% of progressors showed fibrotic changes by last visit vs 27% of non-progressors (P < .001).
• Baseline lung fibrosis was independently associated with poorer survival (adjusted HR, 6.5; 95% CI, 1.5-28.2).

IN PRACTICE

"ILD is frequent and usually inaugural in anti-Ku-positive patients with AID [autoimmune disease]. The overwhelming majority of patients undergo at least one episode of progression and ultimately develops pulmonary fibrosis over time despite IS [immunosuppressive] treatments. These findings underscore the need to evaluate antifibrotic agents in this setting," the authors of the study wrote.

SOURCE

The study was led by Marie Robert, Université Paris Saclay, FHU CARE, INSERM, AP-HP, Hôpital Bicêtre in Le Kremlin-Bicêtre, France. It was published online on July 27 in RMD Open.

LIMITATIONS

ILD progression criteria were applied across the entire follow-up rather than within a strict 12-month timeframe, which may have increased the percentage classified as progressors. Treatment heterogeneity and lack of detailed longitudinal treatment data precluded reliable inclusion of treatment variables as time-dependent covariates.

DISCLOSURES

The authors did not declare any specific funding. Several authors reported consulting fees, honoraria, research grants, or travel fees from various pharmaceutical companies including Boehringer Ingelheim, Sanofi, GSK, Novartis, and others.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.