Clinical Characteristic Analysis Of Primary Biliary Cirrhosis Complicating Sj?gren Syndrome

Objective:To analyze the relations between primary biliary cirrhosis(PBC),PBC complicating Sj?gren syndrome(SS)and primary Sj?gren syndrome(pSS),to improve the understanding of the disease.Methods:We selected patients who were diagnosed with PBC and pSS from the second hospital of Shanxi Medical college between 2010.11.01 and 2016.06.01.The clinical characteristics,treatment responses and clinical outcomes of patients with PBC complicating SS were compared with only-PBC and pSS patients retrospectively.Results:1.The baseline liver damage-related symptoms and biochemical indicators in patients with PBC complicating SS are significantly lighter than that of only-PBC patients;2.The incidence of liver cirrhosis and hepatic decompensation in patients who initially diagnosed with PBC and then combined with SS are lower than only-PBC patients,but the symptom of dry mouth is more obvious;3.The positive rate of anti-SSB antibody and fever symptom are more common in patients with pSS than those with PBC complicating SS patients;4.There is no significant difference in the Sj?gren syndrome-related symptoms and laboratory tests between the patients diagnosed with pSS and the patients who were initially diagnosed with SS and then combined with PBC;5.With the combination therapy of Ursodeoxycholic acid + methylprednisolone +mycophenolate mofetil,the levels of ALT,AST,ALP,GGT and ESR in patients with PBC complicating SS are lower than before,but there is no significant change with TBIL level before and after treatment.And the average levels of ALT,AST and ALP reach the normal range within 3 months after treatment;6.With the combination therapy of Ursodeoxycholic acid + methylprednisolone +mycophenolate mofetil,the baseline levels of TBIL,ALP and GGT are higher in biochemical non-remission group than that in biochemical remission group;7.Patients with PBC complicating SS and only-PBC are no difference in terms of liver cirrhosis and hepatic decompensation progression.Conclusions:1.PBC complicating SS patients with both PBC and pSS clinical features,but the baseline liver damage-related manifestations are lighter than only-PBC patients,Sj?gren syndrome-related symptoms and immune parameters are similar to pSS patients;2.PBC patients with dry mouth symptom are easily combined with SS.It is necessary to do early detection of Sj?gren syndrome-related screening;3.With the combination therapy of Ursodeoxycholic acid + methylprednisolone +mycophenolate mofetil,ALT,AST,ALP,GGT and ESR levels are significantly improved in PBC complicating SS patients,but the improvement of TBIL level is not obvious.The baseline levels of ALP,GGT and TBIL may affect biochemical remission;4.PBC complicating SS do not accelerate the progression of PBC,and the presence of two autoimmune diseases may even change the disease characteristics and prognosis.