ABSTRACT
Objective To investigate the common initial clinical presentations of primary Sj(o)gren's syndrome (pSS) with pulmonary complications,and to explore the differences between patients with extraglandular manifestations at disease onset (EGM) and those with glandular manifestations at disease onset (GM).Methods A total of 1 341 hospitalized SS patients from 2003 to 2012 were retrospectively reviewed.Of them,102 hospitalized patients with pSS'associated lung disease were analyzed and included.Case control study was performed to explore the differences between the EGM group and the GM group.Results Fifty-one percent of patients were presented with EGM at onset,with significantly shorter disease duration [36 (12,156) m vs 102 (48,159) m,x2=-2.41,P=0.016].Although the mean diagnose time was similiar,only 4％ of the EGM group could be confirmed the pSS diagnose at onset,which was significantly less frequently than that of the GM group (34％,22=15.29,P＜0.01).Case control study revealed that hyperglobulinemia,elevated RF titers and anti-SSA and/or anti-SSB test positive were less predominant in the EGM group [IgG 16(12,21) g/L vs 21 (15,28) g/L,x2=-2.15,P=0.032;22 (20,171) U/ml vs 104 (20,238) U/ml,x2=-l.98,P=0.048;33％ vs 72％,x2=15.78,P＜0.01].The predicted value of TLC and FVC were lower [(87±23)％ vs (97±20)％,x2=-1.96,P=0.050;(8±28)％ vs (100±27)％,x2=-1.70,P=0.089] and HRCT score was higher in EMG group [12(88,15) vs 8(5,13),x2=-1.82,P=0.070].Conclusion EMG at onset is the common initial manifestation of pSS'associated lung involvement.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.