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

Haresh Selvaskandan; Chee Kay Cheung; John Dormer; David Wimbury; Maria Martinez; Gang Xu; Jonathan Barratt

doi:10.1159/000508841

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

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

Authors

Haresh Selvaskandan^{1

2}, Chee Kay Cheung^{3

4}, John Dormer⁵, David Wimbury⁴, Maria Martinez³, Gang Xu³, Jonathan Barratt^{3

4}

Affiliations

¹ John Walls Renal Unit, Leicester General Hospital, University Hospitals of Leicester, Leicester, United Kingdom, [email protected].
² Department of Cardiovascular Sciences, University of Leicester, Leicester, United Kingdom, [email protected].
³ John Walls Renal Unit, Leicester General Hospital, University Hospitals of Leicester, Leicester, United Kingdom.
⁴ Department of Cardiovascular Sciences, University of Leicester, Leicester, United Kingdom.
⁵ Department of Pathology, University Hospitals of Leicester, Leicester, United Kingdom.

PMID: 32721954
DOI: 10.1159/000508841

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.

Publication types

Case Reports

MeSH terms

Antibodies, Monoclonal / administration & dosage
Antibodies, Monoclonal / therapeutic use*
Antibodies, Monoclonal, Humanized / administration & dosage
Antibodies, Monoclonal, Humanized / therapeutic use*
Complement System Proteins / metabolism*
Female
Glomerulonephritis, IGA / immunology
Glomerulonephritis, IGA / physiopathology
Glomerulonephritis, IGA / therapy*
Humans
Immunoglobulin A / blood
Kidney Glomerulus / immunology
Kidney Glomerulus / pathology
Kidney Transplantation
Lectins / antagonists & inhibitors*
Mannose-Binding Protein-Associated Serine Proteases / antagonists & inhibitors
Metabolic Networks and Pathways / drug effects
Metabolic Networks and Pathways / immunology
Prednisolone / administration & dosage
Prednisolone / adverse effects
Vasculitis / immunology
Vasculitis / physiopathology
Vasculitis / therapy*
Young Adult

Substances

Antibodies, Monoclonal
Antibodies, Monoclonal, Humanized
Immunoglobulin A
Lectins
Complement System Proteins
Prednisolone
MASP2 protein, human
Mannose-Binding Protein-Associated Serine Proteases
narsoplimab