Eculizumab as rescue therapy for lupus nephritis-related thrombotic microangiopathy
Copyright by Società Italiana di Nefrologia SIN, Rome, Italy..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2020 |
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| Erschienen: |
2020 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:37 |
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| Enthalten in: |
Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologia - 37(2020), 3 vom: 10. Juni |
| Sprache: |
Italienisch |
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| Beteiligte Personen: |
Fani, Filippo Maria [VerfasserIn] |
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| Anmerkungen: |
Date Completed 10.05.2021 Date Revised 10.05.2021 published: Electronic Citation Status MEDLINE |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM311093140 |
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| 500 | |a published: Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright by Società Italiana di Nefrologia SIN, Rome, Italy. | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Systematic Review | |
| 650 | 4 | |a Thrombotic microangiopathy | |
| 650 | 4 | |a eculizumab | |
| 650 | 4 | |a lupus nephritis | |
| 650 | 4 | |a systemic lupus erythematosus | |
| 650 | 7 | |a Antibodies, Monoclonal, Humanized |2 NLM | |
| 650 | 7 | |a Complement Inactivating Agents |2 NLM | |
| 650 | 7 | |a Immunosuppressive Agents |2 NLM | |
| 650 | 7 | |a eculizumab |2 NLM | |
| 650 | 7 | |a A3ULP0F556 |2 NLM | |
| 700 | 1 | |a Patera, Annalisa |e verfasserin |4 aut | |
| 700 | 1 | |a Delsante, Marco |e verfasserin |4 aut | |
| 700 | 1 | |a Rossi, Giovanni Maria |e verfasserin |4 aut | |
| 700 | 1 | |a Manenti, Lucio |e verfasserin |4 aut | |
| 700 | 1 | |a Landini, Samuela |e verfasserin |4 aut | |
| 700 | 1 | |a Regolisti, Giuseppe |e verfasserin |4 aut | |
| 700 | 1 | |a Fiaccadori, Enrico |e verfasserin |4 aut | |
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