Thrombotic microangiopathy complicating newly diagnosed Sjögren's syndrome in a dialysis patient

Mei Hua Cheng, Jui Hsiang Lin, Tzung Hai Yen, Han Ting Wang, Men Chen Chen, Hsiao Ling Huang, Hsin Ping Chih, Wei Jie Wang*

*Corresponding author for this work

Research output: Contribution to journalJournal Article peer-review

1 Scopus citations

Abstract

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.

Original languageEnglish
Pages (from-to)1162-1165
Number of pages4
JournalRenal Failure
Volume36
Issue number7
DOIs
StatePublished - 08 2014

Keywords

  • Dialysis
  • ESRD
  • Microangiopathy
  • Renal failure
  • Sjögren's syndrome
  • Thrombotic

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