Bronchiectasis in Systemic Autoimmune Rheumatic Diseases: Clinical Significance and Diagnostic Challenges
8 affiliations
- 1Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, Univesity of Trieste, 34149 Trieste, Italy
- 2Center for Interstitial and Rare Lung Diseases, Department of Pneumology, Ruhrlandklinik, University Hospital Essen, University of Duisburg-Essen, 45147 Essen, Germany
- 3Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Science, School of Biological Sciences, Faculty of Biological Medicine and Health, The University of Manchester, Manchester Academic Health Science Centre, Stopford Building, Oxford Road, Manchester M13 9PL, UK
- 4Rheumatology Unit, ASST Ovest Milanese, Legnano Hospital, Legnano, 20025 Milan, Italy
- 5Department of Rheumatology and Immunology, Shantou Central Hospital, Shantou 515041, China
- 6Department of Experimental and Clinical Medicine, Division of Internal Medicine, Azienda Ospedaliero Universitaria Careggi, University of Florence, 50134 Florence, Italy
- 7IRCCS AOU Ss Antonio e Biagio e Cesare Arrigo di Alessandria, 15121 Alessandria, Italy
- 8Respiratory Medicine, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, 35128 Padova, Italy
Abstract
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.
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BibTeX
@article{chernovsky2026bronchiectasis,
title = {{Bronchiectasis in Systemic Autoimmune Rheumatic Diseases: Clinical Significance and Diagnostic Challenges}},
author = {Maria Chernovsky and Renata Greco and Micol Pividori and Giorgio Monteleone and Michael Hughes and Paola Confalonieri and Francesco Salton and Eugenio Capparelli and Yukai Wang and Giulia Bandini and Marco Confalonieri and Maria Chianese and Umberto Semenzato and Barbara Ruaro},
journal = {Diagnostics},
year = {2026},
month = oct,
doi = {10.3390/diagnostics16213550},
url = {https://doi.org/10.3390/diagnostics16213550},
note = {CC BY},
}
RIS
TY - JOUR
TI - Bronchiectasis in Systemic Autoimmune Rheumatic Diseases: Clinical Significance and Diagnostic Challenges
AU - Maria Chernovsky
AU - Renata Greco
AU - Micol Pividori
AU - Giorgio Monteleone
AU - Michael Hughes
AU - Paola Confalonieri
AU - Francesco Salton
AU - Eugenio Capparelli
AU - Yukai Wang
AU - Giulia Bandini
AU - Marco Confalonieri
AU - Maria Chianese
AU - Umberto Semenzato
AU - Barbara Ruaro
PY - 2026
DA - 2026/10/01
JO - Diagnostics
DO - 10.3390/diagnostics16213550
UR - https://doi.org/10.3390/diagnostics16213550
AB - 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.
SN - 2075-4418
ER -