Lupus-associated thrombotic thrombocytopenic purpura-like microangiopathy.
Recently reported cases of lupus complicated by a thrombotic thrombocytopenic purpura (TTP)-like syndrome suggest a survival benefit to early treatment with plasma exchange. The following is a report of the eighth such case in the last ten years. A 44-year-old lady known for lupus presented with the nephrotic syndrome and a renal biopsy was consistent with class 4G lupus nephritis. She was given high-dose steroids and cytotoxic therapy, but her induction therapy was complicated by the classic pentad of TTP. She was subsequently treated with another course of high-dose steroids, a different cytotoxic agent, and plasma exchange, with clinical resolution shortly thereafter. Similar to seven recently reported cases of microangiopathy in lupus, this lady's TTP-like syndrome improved dramatically after initiation of plasma exchange, despite not having a severely deficient ADAMTS13. This has implications on both current clinical practice and on the pathogenesis of TTP-like syndromes in lupus.
Authors :-Blum D, Blake D,
Keywords :-Atypical hemolytic-uremic syndrome, Hemolytic uremic syndrome, Lupus, Lupus nephritis, Microangiopathic hemolytic anemia, Microangiopathy, Plasma exchange, Systemic lupus erythematosus associated thrombotic thrombocytopenic purpura-like microangiopathic hemolytic anemia, Thrombotic thrombocytopenic purpura,
Mesh Headings :-Dizziness, Humans, Mental Disorders,
Chemical List :-Anti-Bacterial Agents, Ointments,
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Blum D, Blake D, .Lupus-associated thrombotic thrombocytopenic purpura-like microangiopathy. World journal of nephrology 2015-Nov;4()528-31 DOI:10.5527/wjn.v4.i5.528
Details of Journal
Journal Title - World journal of nephrology
ISSN - 2220-6124
Volume - 4
Issue - 5
Publish date - 2015-Nov
Language - eng
Country - United States
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