Background/Purpose:  To investigate the common initial clinical presentations of primary Sjogren’s syndrome with pulmonary complications, and to explore the differences between patients with extro-glandular manifestations at onset (EGM) and those with glandular manifestations at onset (GM).

Methods:  A total of 1341 hospitalized SS patients from 2003 to 2012 were retrospectively reviewed. Of them, 102 hospitalized patients with pSS-associated lung disease were analyzed and recruited.

Results:  Fifty one percent were presented with EGM at onset, with significantly shorter disease duration [36.0 (12.0-156.0) vs. 102.0 (48.0-159.0) m，p=0.016]. Although mean diagnose time was equal, only 3.8% of EGM group can be confirmed the pSS diagnose at onset, which was significantly less frequently than that of GM group (34.0%, p=0.000).Case control study revealed that hyperglobulinemia, elevated RF titers and anti-SSA and/or anti-SSB positive were less predominant in EGM group [15.85 vs. 21.20g/L; 22.40 (20.00-171.00) vs. 104 (20.00-237.50)IU/ml; 32.7% vs. 72.0%；p<0.05]. TLC and FVC of predicted value were lower (87.07±22.76% vs. 96.82±19.78%, p=0.050; 88.30±27.76% vs. 100.18±27.40%, p=0.089）and HRCT score was higher in EMG group [11.50 (7.75-15.25) vs. 8.00 (5.00-13.00), P=0.070].

Conclusion:  EMG at onset is common initial manifestation of pSS-associated lung involvement patients. Pulmonary complication is more progressively and severe than those with MG at onset. Anti-SSA positive, elevated RF titer and hyperglobulinemia are not predominant for patients with EMG at onset.

« Back to 2016 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-features-of-primary-sjogrens-syndrome-associated-lung-involvement-with-extro-glandular-manifestations-at-onset/