Prevalence and spectrum of symptomatic pulmonary involvement in primary Sjogren's syndrome

Objective. The present cross-sectional study aimed to estimate the prevalence of chronic respiratory symptoms in primary Sjogren's syndrome (pSS) and define the clinical, functional and imaging characteristics of symptomatic pulmonary disease in pSS. Methods. Four hundred and fourteen consecutive pSS patients were interviewed for the presence of chronic respiratory complaints (cough and/or dyspnea). Symptomatic pSS patients without respiratory or other comorbidities underwent further investigation with clinical evaluation and assessment with pulmonary functional testing (PFTs) and chest high resolution CT (hrCT) on inspiratory and expiratory phase. Comparison of clinical and laboratory features between symptomatic and asymptomatic pSS patients was also performed. Results. Prevalence of chronic respiratory symptoms in pSS was estimated at 21.5% (89/414). Symptoms were attributed to underlying comorbidities in approximately one third of cases (30/89). Thirty nine of the remaining 59 patients were finally assessed with PFTs and hrCT. Small airway disease was diagnosed in 20 individuals with an obstructive pattern in PFTs and/or compatible radiological signs. Seven patients were diagnosed with interstitial lung disease, while in the remaining 12 pSS patients, with normal PFTs and hrCT, symptoms were attributed to xerotrachea. Raynaud's phenomenon occurred more frequently in symptomatic than asymptomatic patients (p=0.024). Conclusion. Approximately one fifth of a large cohort of pSS patients presented chronic respiratory symptoms. Small airway disease was the most commonly recognised pulmonary disorder among symptomatic pSS patients, followed by xerotrachea and interstitial lung disease.