What is it about?

A novel treatment for autoantibody mediated glomerulonephritis (anti-GBM disease) based on an streptococcal enzymes that cleaves IgG in vivo. We target 15 patients that with standard therapy had little chance to have their kidney function salvaged (<20%). In the study the experimental therapy was successful in 67% (10 out of 15). This was achieved without any major safety issues.

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Why is it important?

It is the first major advance in the treatment of anti-GBM disease since the introduction of plasma exchange 50 years ago. One shot of imlifidase replaces 7 our more sessions of plasma exchange, and in additions it removes antibodies already bound to the basement membrane. The results suggest that removing the autoantibodies is enough to halt the disease process opening the chance for recovery.

Perspectives

The therapy can bli applicable for a large number of autoimmune diseases where autoantibodies mediate important steps in the pathogenesis. Today it has only been tried as a single injection, but repeat dosing may be possible in the future. In Nephrology, disease like ANCA vasculitis, membranous nephropathy, IgA nephropathy and Lupus nephritis are associated with pathogenic IgG antibodies. This study heralds a new era, however, before implementation in clinical practice results must be confirmed in randomized trials for each and every indication.

Mårten Segelmark
Lunds Universitet

Read the Original

This page is a summary of: Endopeptidase Cleavage of Anti-Glomerular Basement Membrane Antibodies in vivo in Severe Kidney Disease: An Open-Label Phase 2a Study, Journal of the American Society of Nephrology, March 2022, American Society of Nephrology,
DOI: 10.1681/asn.2021111460.
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