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Updated: Oct 1, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Small airway disease and its association with respiratory symptoms and restrictive ventilation pattern in systemic
G M Sauer1, P Panagopoulos2, C Clarenbach3
1Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
Background:
Clinical relevance and prognosis of small airway disease in systemic sclerosis including are currently uncertain.
Methods:
This cohort study of prospectively collected data included cross-sectional and longitudinal analyses of systemic sclerosis patients. Small airway disease was defined according to pulmonary function tests (PFT) and high-resolution computed tomography (HRCT). The association of small airway disease with respiratory symptoms, functional status and decline over time was tested using multivariable generalized estimating equations. Kaplan-Meyer and multivariable Cox regression analyzed the association of small airway disease with mortality and explored the potential chronological association between small airway disease and interstitial lung disease.
Results:
Among 474 systemic sclerosis patients, small airway disease prevalence ranged between 14-54%, depending on the applied definition. Small airway disease was frequently concomitant with interstitial lung disease, independently associated with lower PFT values and higher NYHA functional class. Conversely, small airway disease was not associated with interstitial lung disease progression or mortality. Exploratory analyses did not support a chronological link between small airway disease and interstitial lung disease onset, and vice versa.
Conclusions:
Small airway disease is frequent in systemic sclerosis and associates with respiratory symptoms and restrictive ventilatory pattern, independent of interstitial lung disease. The lack of a chronological link between small airway disease and interstitial lung disease and the absent association with interstitial lung disease progression or survival argue for small airway disease as an overlapping, yet distinct pulmonary involvement in systemic sclerosis.
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