Eculizumab use in a tertiary care nephrology center: data from the Vienna TMA cohort.

Autor: Aigner C; Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria. christof.aigner@meduniwien.ac.at., Gaggl M; Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria., Stemer G; Department of Pharmacy, Vienna General Hospital, Vienna, Austria., Eder M; Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria., Böhmig G; Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria., Kain R; Department of Pathology, Medical University Vienna, Vienna, Austria., Prohászka Z; Research Group for Immunology and Haematology, Department of Internal Medicine and Hematology, Semmelweis University- Eötvös Loránd Research Network (Office for Supported Research Groups), Budapest, Hungary., Garam N; Research Group for Immunology and Haematology, Department of Internal Medicine and Hematology, Semmelweis University- Eötvös Loránd Research Network (Office for Supported Research Groups), Budapest, Hungary., Csuka D; Research Group for Immunology and Haematology, Department of Internal Medicine and Hematology, Semmelweis University- Eötvös Loránd Research Network (Office for Supported Research Groups), Budapest, Hungary., Sunder-Plassmann R; Genetics Laboratory, Department of Laboratory Medicine, Medical University Vienna, Vienna, Austria., Piggott LC; Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria., Haninger-Vacariu N; Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria., Schmidt A; Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria., Sunder-Plassmann G; Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria.
Jazyk: angličtina
Zdroj: Journal of nephrology [J Nephrol] 2022 Mar; Vol. 35 (2), pp. 451-461. Date of Electronic Publication: 2021 Feb 18.
DOI: 10.1007/s40620-021-00981-8
Abstrakt: Background: Practice patterns of eculizumab use are not well described. We examined indications for, and outcomes of, eculizumab therapy in a tertiary care nephrology center.
Methods: We used the "Vienna TMA cohort" and the hospital pharmacy database at the Medical University of Vienna to identify patients that received eculizumab treatment between 2012 and 2019. We describe clinical characteristics, details of eculizumab use, and outcomes of patients with complement gene-variant mediated TMA (cTMA), secondary TMA (sTMA) and C3 glomerulopathy (C3G).
Results: As of December 2019, 23 patients received complement blockade at the Division of Nephrology and Dialysis: 15 patients were diagnosed with cTMA, 6 patients with sTMA and 2 patients with C3G. Causes of sTMA were bone marrow transplantation (n = 2), malignant hypertension, malignant tumor, systemic lupus erythematosus, antiphospholipid syndrome and lung transplantation (each n = 1). Across all indications, patients had a median age of 31 and were predominantly female (78%) and the median duration of treatment was 227 days. Hematological recovery was seen in most patients, while renal response was best in patients with cTMA. Adverse events were recorded in 26%.
Conclusions: In summary, eculizumab is the treatment of choice for cTMA patients that do not respond to plasma therapy. In patients with sTMA and C3G, the response rates to therapy are much lower and therefore, the decision to start therapy needs to be considered carefully.
(© 2021. The Author(s).)
Databáze: MEDLINE
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