Published online Nov 6, 2015. doi: 10.5527/wjn.v4.i5.528
Peer-review started: November 22, 2014
First decision: December 12, 2014
Revised: September 11, 2015
Accepted: September 29, 2015
Article in press: September 30, 2015
Published online: November 6, 2015
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.
Core tip: In patients with lupus who develop thrombotic microangiopathy, early initiation of plasma exchange appears to carry a survival benefit - even in those patients whose ADAMTS13 activity levels are not severely deficient. This improved survival has become apparent in the last ten years during which time seven cases of thrombotic microangiopathy complicating lupus and treated with plasma exchange have been reported. The present article describes the eighth such case, reviews the previously described cases and outcomes of microangiopathy in lupus, and hypothesizes as to why plasma exchange appears to be beneficial in this subset of patients with atypical haemolytic uremic syndrome.