World J Nephrol. 2015 Nov 06;4(5):528-31. doi: 10.5527/wjn.v4.i5.528.
World journal of nephrology
Daniel Blum, Geoffrey Blake
PMID: 26558190 PMCID: PMC4635373 DOI: 10.5527/wjn.v4.i5.528
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.
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
