1. TalkTMA Admin
  2. Case Discussions
  3. Thursday, 31 March 2016
I saw a case of a 24 year-old woman with SLE.

She has a past history of polyarthritis and nephritis presenting in her late teens. At one point 5 years ago, she underwent renal biopsy to work-up hematuria and proteinuria in the context of normal and stable renal function and she was found to have mild class II lupus nephritis. She was treted with prenisone and MMF but was subsequently lost to follow-up.

She presented at age 24 with rising creatinine (from 90 to 350 umol/L) and an active urine sediment. Hemoglobin was 90, platelets 46 with evidence of TMA of blood film. She underwent renal biopsy which showed no convincing evidence of active SLE nephritis but did show obious TMA. Her serology showed low complements and postive ANA, anti-DS DNA, negative for anticardiolipin or lupusanticoagulant.

She was treated with steroid pulse and MMF with no improvement in her renal function or hemotologic parameters.

Would you treat this woman with Eculizumab? What further work-up would you do?

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Thrombotic microangiopathy (TMA) is a group of rare disorders affecting the microvasculture of most organ beds. The impact of these diseases on those affected by them is immense. Our understanding of these disorders is rapidly expanding and therapeutic options are beginning to appear. However, many challenges remain when managing TMA and one is a practical one: the diseases are rare and the experts in them are few and spread worldwide. Read more...