Sj�gren's Syndrome Complicated with Thrombotic Thrombocytopenic Purpura: A Case Report and Literature Review

The association of Sjogren's syndrome (SS) with thrombotic thrombocytopenic purpura (TTP) is rare, but it carries a high death rate and can be easily misdiagnosed. We report a case of TTP with SS and the related literature is reviewed. A previously healthy 72-year-old woman presented with an acute onset of marked thrombocytopenia and then dizziness, fatigue, fever and fainting. Schirmer's test was bilaterally positive, associated with positive Rose Bengal corneal staining. Radionuclide imaging of the glands showed abnormalities. The presence of anti-SSA and anti-SSB antibodies confirmed the diagnosis of SS. The patient was treated successfully with plasma exchange therapy, plasma infusion, glucocorticoid, and immunosuppressive agents. Her condition has remained stable after 6 months of follow-up. Only a few cases of SS complicated with TTP have been described in the literature. Rheumatologists should pay attention to this special condition because it is rare and has a high mortality rate.