Autoimmune Interstitial Lung Disease

Autoimmune interstitial lung disease (ILD) refers to a group of lung disorders caused by inflammation and scarring (fibrosis) of the interstitium—the tissue surrounding the lung’s air sacs—due to autoimmune activity. Rather than being a single disease, autoimmune ILD is a complication or manifestation of various autoimmune diseases. In autoimmune ILD, the immune system mistakenly attacks the lung tissue, leading to chronic inflammation and progressive damage that can impair breathing and oxygen exchange.

Shortness of breath (especially during exertion), dry cough, fatigue, chest discomfort, and reduced exercise tolerance. In more advanced cases, patients may experience clubbing of the fingers or signs of low oxygen levels (cyanosis).

Autoimmune ILD is not classified as a primary autoimmune disease, but rather a serious immune-mediated complication of systemic autoimmune conditions such as rheumatoid arthritis, systemic sclerosis (scleroderma), Sjögren’s syndrome, polymyositis, dermatomyositis, and mixed connective tissue disease.

Risk factors include having a diagnosed autoimmune connective tissue disease, older age, a history of smoking, and certain genetic predispositions. The prevalence of ILD varies depending on the autoimmune disease. For example, ILD affects approximately 10% of people with rheumatoid arthritis, but as many as 40–50% of those with systemic sclerosis. Environmental exposures, such as silica dust, may also contribute to increased risk in some individuals.

Autoimmune ILD tends to progress more slowly than idiopathic pulmonary fibrosis (IPF), but its course can vary widely. Early diagnosis and treatment are critical to slowing disease progression.

• Interstitial lung disease biomarkers: a systematic review and meta-analysis (2025)
• Validation of the gender, age, physiology model and other prognostic factors in interstitial lung disease patients with systemic autoimmune rheumatic disease (2025)
• Evaluating Deployment-related Respiratory Diseases in Military Veterans (2025)
• The Clinical Efficacy and Safety of Nintedanib in the Treatment of Interstitial Lung Disease Among Patients With Systemic Sclerosis: Systematic Review (2025)