Background/Purpose: Prevalence of symptomatic pulmonary disease in primary Sjögren’s Syndrome (pSS) ranges between 9% and 27%. Xerotrachea, small airway disease and interstitial lung disease (ILD) are among the most common manifestations. However, the evidence is conflicting on which of the above mentioned respiratory disorders is the most prevalent in pSS patients. The purpose of the present study is to estimate the prevalence and type of chronic respiratory symptoms in a large cohort of pSS and associate them with disease-specific clinical and laboratory parameters.

Methods:  Consecutive patients with pSS were questioned for chronic respiratory symptoms (cough, dyspnea or both). Patients with pre-existing respiratory symptoms before the onset of pSS were excluded from further investigation. The remaining symptomatic patients were assessed clinically, with PFTs (pre- and post-bronchodilator spirometry, static lung volumes and diffusion capacity) and high resolution CT (hrCT) on inspiratory and expiratory phase. Clinical and laboratory characteristics taken from the patient’s records were compared between symptomatic and asymptomatic patients and between patients with ILD and other non ILD respiratory disorder related to pSS.

Results: Thus far, 348 patients have been screened from an initial cohort of 723 pSS patients. According to preliminary analysis, chronic respiratory symptoms were detected in 24% (84/348) of patients. Two thirds of the cases (55/84) were attributed to pSS. PFTs and chest hrCT were performed in 34 patients. Eighteen patients (18/34 or 53%) were diagnosed with small airway disease, while only 5 patients (15%) had ILD. Xerotrachea was considered responsible for chronic cough, in symptomatic patients with normal PFTs and normal or non-specific CT findings (11/34 or 32%). There were no statistically significant differences in clinical and laboratory parameters between symptomatic and asymptomatic patients, patients with ILD, patients with small airway disease or symptoms related to xerotrachea.

Conclusion:  Small airway disease is the most common pulmonary manifestation in patients with chronic respiratory symptoms and pSS, while ILD is less frequent than previously thought.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-radiological-and-functional-characteristics-of-clinically-significant-pulmonary-involvement-in-primary-sjogrens-syndrome/