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Secondary thrombotic microangiopathy in systemic lupus erythematosus and antiphospholipid syndrome, the role of complement and use of eculizumab: Case series and review of literature

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Kello N1, Khoury LE2, Marder G2, Furie R2, Zapantis E2, Horowitz DL2. Semin Arthritis Rheum. 2018 Dec 4. pii: S0049-0172(18)30493-1. doi: 10.1016/j.semarthrit.2018.11.005. [Epub ahead of print]


Author information 1 Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 865 Northern Boulevard, Suite 302, Great Neck, NY 11021, USA. Electronic address: ninakello1986@gmail.com. 2 Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 865 Northern Boulevard, Suite 302, Great Neck, NY 11021, USA.

Abstract INTRODUCTION: Thrombotic microangiopathy (TMA) is a life-threatening, albeit infrequent, complication of systemic lupus erythematosus (SLE) and anti-phospholipid syndrome (APS). Recommendations for the treatment of SLE- and APS-related secondary TMA are currently based solely on case reports and expert opinion. Unfortunately, interventions may not yield timely results or effectively halt the progression of TMA. Since complement activation plays a key role in the pathogenesis of secondary TMA due to SLE, APS, a therapy that targets the complement pathway is an attractive intervention. Eculizumab, a recombinant, fully humanized IgG2/IgG4 monoclonal antibody inhibits C5 activation and is FDA-approved for PNH and atypical HUS (aHUS). However, limited case reports are available on its use in treatment of secondary TMA.

CASE PRESENTATION AND RESULTS: We present the largest case series to date that includes 9 patients with SLE and/or APS who were successfully treated with eculizumab for refractory secondary TMA. In this case series, we report significant responses in hematology values, renal function and other organs following treatment with eculizumab. At 4 weeks, 75% improvement in platelet counts was observed in 78% of patients. Two-thirds of patients demonstrated >75% improvement of haptoglobin and LDH at four weeks. At 4 weeks, eGFR improved by 25% in half of the patients, and 43% had reductions in proteinuria. Two of 3 patients that required hemodialysis were able to be taken off hemodialysis.

CONCLUSION: Based on these observations, we suggest that eculizumab may be a potential treatment option for acutely ill patients with secondary TMA due to SLE and/or APS who have failed standard of care. A collective approach is needed to better elucidate the role and optimal timing of eculizumab use in the management of TMA complicating SLE and/or APS.