Early detection and risk stratification in autoimmune-related interstitial lung disease: a state-of-the-art review

TL;DR

This review discusses how to detect and assess risk for lung disease linked to autoimmune conditions, emphasizing early detection and personalized care.

Contribution

The paper provides a comprehensive review of emerging strategies for early detection and risk stratification of autoimmune-related interstitial lung disease.

Findings

Systematic clinical assessment, imaging, and pulmonary function tests are key for early detection of ILD in autoimmune diseases.

Integrated models combining clinical, radiologic, and serological data improve risk stratification and monitoring.

Autoimmune conditions have distinct ILD patterns, requiring tailored diagnostic and prognostic approaches.

Abstract

Interstitial lung disease (ILD) is a major pulmonary complication of autoimmune rheumatic diseases (ARD) and a leading contributor to long-term morbidity and mortality. Although ARDs share underlying immune dysregulation, the onset, radiologic phenotype, and clinical course of ILD vary substantially across individual diseases. Consequently, early detection and structured risk stratification at baseline and during follow-up have become essential elements of care for patients at risk of ARD-related ILD.

This review examines the principles and emerging strategies for early identification of ARD-related ILD, emphasizing the role of systematic clinical assessment, high-resolution computed tomography, and longitudinal pulmonary function evaluation in detecting early lung involvement. We discuss how radiologic patterns, functional measures, and serological profiles contribute to prognostic classification in different autoimmune contexts, with particular focus on the early identification of patients at risk of rapid ILD progression. Disease-specific ILD patterns are reviewed across major autoimmune conditions—including systemic sclerosis, idiopathic inflammatory myopathies, primary Sjögren’s syndrome, mixed connective tissue disease, systemic lupus erythematosus, rheumatoid arthritis, and anti-neutrophil cytoplasmic antibody-associated vasculitis—each characterized by distinct risk factors, distinct imaging findings, and divergent prognostic trajectories. Finally, we highlight emerging approaches to risk stratification, including integrated models that combine clinical, radiologic, and serological domains to mitigate the risk of disease progression and guide monitoring strategies.

Overall, current evidence supports a precision-medicine framework for ARD-related ILD, in which early recognition and individualized assessment of progression risk are crucial to improving outcomes and informing therapeutic decision-making.

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