Abstract: Objective: To enhance the understanding of the clinical features of Sjogren's syndrome complicated with complete renal tubular acidosis (SSRTA).Methods: The clinical features of 22 patients with SSRTA were summarized and compared with 37 primary SS(pSS) patients whose kidneys were not involved. Results: All patients presented mainly clinical manifestations of type Ⅰ RTA (distal RTA) except 1 patient suffered simultaneously from renal glucosuria and uricaciduria indicating involvement of proximal renal tubules. Most patients of SSRTA (68.18%, 15/22) had no or only mild dry mouth and dry eyes. As compared with single pSS patients, SSRTA patients tended to have more obvious hyperglobulinemia, but less typical xerostomia, keratoconjunctivitis sicca and lymphadenovarix (P＜0.01 or 0 05). Positive rate of anti SSA antibody in SSRTA group was significantly higher (P＜0.05). An over 4 year follow up showed that none of the SSRTA patients died. Conclusion: The SSRTA patients have mild sicca symptoms and favorable prognosis despite the existence of typical symptoms of type Ⅰ RTA and obvious immune abnormalities.