1. [Eculizumab as rescue therapy for lupus nephritis-related thrombotic microangiopathy].
- Author
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Fani FM, Patera A, Delsante M, Rossi GM, Manenti L, Landini S, Regolisti G, and Fiaccadori E
- Subjects
- Drug Resistance, Female, Humans, Immunosuppressive Agents therapeutic use, Kidney pathology, Lupus Nephritis pathology, Middle Aged, Plasma Exchange, Thrombotic Microangiopathies etiology, Thrombotic Microangiopathies therapy, Antibodies, Monoclonal, Humanized therapeutic use, Complement Inactivating Agents therapeutic use, Lupus Nephritis complications, Salvage Therapy methods, Thrombotic Microangiopathies drug therapy
- Abstract
Thrombotic microangiopathy (TMA) is a frequent and severe complication in systemic lupus erythematosus (SLE). It is reported in almost 20-25% of renal biopsies of patients with lupus nephritis (LN) and is associated with a poor renal prognosis. We report the case of a patient suffering from an aggressive form of proliferative LN in association with thrombotic microangiopathy (TMA-LN), who was resistant to standard combined immunosuppressive treatment with corticosteroids and cyclophosphamide, as well as to plasma exchange (PEX). Eculizumab was given as a rescue therapy with an optimal clinical response. We performed a systematic review of the literature and identified 11 papers, published between 2011 and 2018, with a total of 20 patients, in which eculizumab was used, always as rescue therapy, to treat TMA-LN. All reported cases showed a positive clinical response to eculizumab with a high rate of remission. Even if sparse, available clinical cases and case series support the use of eculizumab in highly selected cases as rescue treatment for LN-TMA resistant to conventional combined immunosuppressive treatment., (Copyright by Società Italiana di Nefrologia SIN, Rome, Italy.)
- Published
- 2020