Thrombotic microangiopathy complicating newly diagnosed Sjögren's syndrome in a dialysis patient.
Thrombotic microangiopathy (TMA) is rarely associated with Sjögren's syndrome (SS). This is the first documented case of a patient undergoing chronic hemodialysis with SS who developed TMA. TMA is an infrequent, life-threatening multisystem disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia, accompanied by microvascular thrombosis that causes variable degrees of tissue ischemia and infarction. It is important to make a quick diagnosis of TMA to cure the reported case as early as possible. The patients with TMA should be diagnosed quickly, and in this case plasma exchange and corticosteroids in combination with cyclophosphamide have been associated with a recurrence free period. Cyclophosphamide has led to the development of treatment protocols using alternative immunosuppressive agents in patients with SS showing a poor response to plasmapheresis and potentially life-threatening manifestations. Further research is required to ascertain the sensitivity, specificity, efficacy, timing, cost-benefit ratio, and necessity of cyclophosphamide in the setting of TMA complicating SS.
Department of Rehabilitation, Tao Yuan General Hospital , Tao Yuan, Taiwan , Republic of China .
Mei-Hua
MH
Cheng
Jui-Hsiang
JH
Lin
Tzung-Hai
TH
Yen
Han-Ting
HT
Wang
Men-Chen
MC
Chen
Hsiao-Ling
HL
Huang
Hsin-Ping
HP
Chih
Wei-Jie
WJ
Wang
D005260:Female; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications*; D008875:Middle Aged; D012859:Sjogren's Syndrome / Q000150:complications*; D057049:Thrombotic Microangiopathies / Q000209:etiology*
Dialysis; ESRD; microangiopathy; renal failure; sjögren’s syndrome; thrombotic