Physiology-based framework proposed for acute SARD-associated ILD exacerbations

Acute exacerbations of systemic autoimmune rheumatic disease-associated interstitial lung disease are uncommon but can cause severe respiratory failure and require intensive care. The authors’ Perspective reviews current evidence and argues that approaches borrowed from idiopathic pulmonary fibrosis exacerbations or acute respiratory distress syndrome may not reflect the varied physiology and treatment responses in AE-SARD-ILD. The article does not report a new patient cohort or trial.

The authors propose an integrated clinical, radiological and mechanical assessment that identifies four potentially overlapping phenotypes: fibrotic/UIP-like, inflammatory/ARDS-like, pulmonary hypertension-dominant and diffuse alveolar haemorrhage-dominant. They describe how these phenotypes could inform selection and adjustment of non-invasive or invasive respiratory support, management of respiratory drive, and consideration of immunomodulatory, pulmonary vascular or extracorporeal strategies.

This is a proposed framework, not a validated management algorithm. The authors state that prospective validation is required; the article therefore does not establish that phenotype-guided care improves outcomes or specify when particular interventions should be used. It may help structure clinical assessment and future research, but the source does not support changing practice on its own.