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.

[Eculizumab as rescue therapy for lupus nephritis-related thrombotic microangiopathy] / Fani, Filippo Maria; Patera, Annalisa; Delsante, Marco; Rossi, Giovanni Maria; Manenti, Lucio; Landini, Samuela; Regolisti, Giuseppe; Fiaccadori, Enrico. - In: GIORNALE ITALIANO DI NEFROLOGIA. - ISSN 1724-5990. - 37:3(2020), pp. 77-90.

[Eculizumab as rescue therapy for lupus nephritis-related thrombotic microangiopathy]

Patera, Annalisa;Delsante, Marco;Rossi, Giovanni Maria;Manenti, Lucio;Regolisti, Giuseppe;Fiaccadori, Enrico
2020-01-01

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.
[Eculizumab as rescue therapy for lupus nephritis-related thrombotic microangiopathy] / Fani, Filippo Maria; Patera, Annalisa; Delsante, Marco; Rossi, Giovanni Maria; Manenti, Lucio; Landini, Samuela; Regolisti, Giuseppe; Fiaccadori, Enrico. - In: GIORNALE ITALIANO DI NEFROLOGIA. - ISSN 1724-5990. - 37:3(2020), pp. 77-90.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11381/2933957
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