Mixed Airway and Pulmonary Parenchymal Disease in Patients With Primary Sjögren Syndrome: A 6-year Follow-up.

OBJECTIVE: To assess pulmonary function and chronic obstructive pulmonary disease (COPD) development over time in patients with primary Sjögren syndrome (pSS), as well as the association between pulmonary function, radiographic findings, respiratory symptoms, and clinical features of pSS, taking cigarette consumption into account. METHODS: Forty patients with pSS (mean age 66 yrs; range 42-81 yrs; 39 women), previously participating in a cross-sectional study on pulmonary involvement in pSS, were reassessed by pulmonary function tests after a mean follow-up time of 6 years. At follow-up, patients were also assessed by high-resolution computed tomography of the chest, as well as for pSS disease activity, respiratory symptoms, and cigarette consumption. RESULTS: Patients with pSS showed significantly decreased percentages of predicted total lung capacity (TLC), residual volume (RV), RV/TLC ratio, and diffusing capacity of the lungs for carbon monoxide, as well as an increase in predicted forced expiratory volume in 1 second/vital capacity (FEV1/VC) ratio from baseline to follow-up. The proportion of COPD in patients with pSS did not change significantly from baseline to follow-up (38% vs 40%, respectively). Radiographic signs of bronchial involvement and interstitial lung disease were each found in 38% of the patients. CONCLUSION: Both airway and pulmonary parenchymal disease were commonly found in patients with pSS, with a coexistence of both an obstructive and restrictive pulmonary function pattern, where the latter tended to deteriorate over time. COPD was a common finding. Airway and pulmonary involvement may be underdiagnosed in pSS, which is why special attention to clinical assessment of pulmonary involvement in patients with pSS is mandated.

Chronic obstructive pulmonary disease is common in never-smoking patients with primary Sjögren syndrome.

OBJECTIVE: To assess the prevalence of chronic obstructive pulmonary disease (COPD) in patients with primary Sjögren syndrome (pSS) and to study the association of COPD with cigarette smoking, radiographic features, respiratory symptoms, disease activity, and laboratory inflammatory and serological features in patients with pSS. METHODS: Fifty-one consecutive patients with pSS (mean age 60 yrs, range 29-82 yrs, 49 women) were assessed by pulmonary function tests (PFT). The PFT results were compared with previously studied population-based controls, standardizing results with regard to sex, age, height, weight, and cigarette smoking. In addition, patients with pSS were assessed by computed tomography of the chest, the European League Against Rheumatism Sjögren Syndrome Disease Activity Index and Patient Reported Index, the St. George's Respiratory Questionnaire (which evaluates respiratory symptoms), and by laboratory inflammatory and serological tests. RESULTS: Forty-one percent of all patients with pSS and 30% of the never-smoking patients with pSS fulfilled the Global Initiative for Chronic Obstructive Lung Disease criteria for COPD. Vital capacity (VC), forced expiratory volume in 1 s (FEV1), FEV1/VC ratio, and DLCO were significantly decreased while residual volume (RV) and the RV/total lung capacity ratio were significantly increased in patients with pSS. Moderate correlations between PFT results, symptoms, and disease activity were found. However, laboratory inflammatory and serological features were poorly associated with PFT results in patients with pSS. CONCLUSION: COPD was a common finding in patients with pSS, even among never-smoking patients. An obstructive pattern was the predominant PFT finding in patients with pSS, although a superimposed restrictive lung disease could not be excluded. The results suggest that the disease per se is involved in the development of COPD in pSS.