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Case Reports
. 2020;144(9):453-458.
doi: 10.1159/000508841. Epub 2020 Jul 28.

Inhibition of the Lectin Pathway of the Complement System as a Novel Approach in the Management of IgA Vasculitis-Associated Nephritis

Haresh Selvaskandan  1   2 , Chee Kay Cheung  3   4 , John Dormer  5 , David Wimbury  4 , Maria Martinez  3 , Gang Xu  3 , Jonathan Barratt  3   4
Affiliations
Case Reports

Inhibition of the Lectin Pathway of the Complement System as a Novel Approach in the Management of IgA Vasculitis-Associated Nephritis

Haresh Selvaskandan et al. Nephron. 2020.

Abstract

IgA vasculitis can present as a glomerulonephritis histologically indistinguishable from IgA nephropathy (IgAN). In IgAN, the alternative and lectin pathways mediate glomerular injury and contribute to kidney function decline. Narsoplimab is a monoclonal antibody against mannan-binding lectin serine peptidase 2 (MASP-2), a key component of the lectin pathway. It is being evaluated in a phase III trial in IgAN (NCT03608033). Histopathological similarities with IgAN suggest lectin pathway activation also occurs in IgAV-associated nephritis (IgAVN). Here, we report the first ever case of narsoplimab use for the treatment of IgAVN.

Keywords: Complement system; IgA nephropathy; IgA vasculitis; Lectin pathway; Rapidly progressive glomerulonephritis.

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