RESUMO
Objective To investigate the clinical characteristics of multisystem impairment of Sj(o)gren's syndrome (SS) to make quick and accurate diagnose and treatment to decrease the rate of misdiagnosis and mistreatment.Methods A case of thrombotic thrombocytopenic purpura with SS was reported and the related literatures were reviewed.The clinical manifestations,laboratory examinations,diagnostic criteria and therapy of thrombotic thrombocytopenic purpura with SS were analyzed.Results Purpura haemorrhagica,hemolytic anemia,central nervous systemic symptoms,kidney impairment and fever were the representative clinical manifestations of thrombotic thrombocytopenic purpura with SS.Plasmapheresis was the most effective therapy with the background treatment with glucocorticosteroid and immunosuppressive agents.Conclusion Thrombotic thrombocytopenic purpura with SS presents a complicated clinical manifestations,so misdiagnosis and mistreatment is very frequent.More attention should be paid to this specific situation because the disease is rare and has high mortality rate.