Bronchiectasis in Systemic Autoimmune Rheumatic Diseases: Clinical Significance and Diagnostic Challenges

Bronchiectasis has been reported in several systemic autoimmune rheumatic diseases, but published estimates are difficult to interpret because many studies count any bronchial dilatation on computed tomography (CT). This is particularly problematic in connective tissue disease-associated interstitial lung disease (ILD), where traction can enlarge the airways without producing the clinical syndrome of bronchiectasis. We reviewed evidence in rheumatoid arthritis, Sjögren disease, anti-neutrophil cytoplasmic antibody-associated vasculitis, systemic sclerosis, systemic lupus erythematosus, and inflammatory myopathies, with attention to imaging definitions, respiratory symptoms, infection, outcomes, and treatment. The association with a distinct airway disorder is best established in rheumatoid arthritis, Sjögren disease, and myeloperoxidase-ANCA-positive vasculitis. In systemic sclerosis and systemic lupus erythematosus, large administrative cohorts suggest an increased occurrence of bronchiectasis, but most data do not allow for confident separation from traction bronchiectasis or other causes. In inflammatory myopathies, reported bronchial dilatation is mainly described within fibrotic ILD. In practice, interpretation should combine high-resolution CT morphology and distribution with cough and sputum, exacerbation history, microbiology, and the presence and extent of ILD; traction and non-traction changes may coexist. Bronchiectasis-specific care should follow established guidelines, while immunomodulatory therapy is selected for the rheumatic disease with explicit consideration of infection risk. No trial has identified a preferred antirheumatic regimen for patients with bronchiectasis, and it remains unknown whether better control of systemic disease changes bronchiectasis outcomes. Studies using consistent clinical and radiological definitions are needed. In mixed connective tissue disease, available evidence is insufficient to characterise bronchiectasis independently of ILD. Seven disease categories are reviewed in total.