Key data demonstrating the potential of imlifidase to significantly alter course of anti-GBM disease published in JASN.- Hansa Biopharma AB.

The publication of the results in JASN is a recognition of the considerable potential for imlifidase in autoimmune diseases involving pathogenic IgG. The investigator-initiated Phase II open-label study explored the efficacy of imlifidase for the treatment of anti-GBM disease as add-on to standard of care with pulse methylprednisolone, oral corticosteroids, cyclophosphamide (CYC) and plasma exchange (PLEX). The results show that kidney function at 6 months was significantly better than in previously published cohorts, without any safety concerns. Of the 15 patients included, 10 were dependent on dialysis at enrollment. At 6 months, a total of 67% (N=10) of the included patients were dialysis independent, which is significantly better than in the historical control cohort, where only 18% had functioning kidneys. All patients that were dialysis-independent at baseline remained so during the study.

“These are really exciting results, as this is the first time we have seen a study suggesting that the course of an acute autoimmune disease like anti-GBM disease can be changed by targetting the antibodies underpinning the immune response. The effects were such that half of patients that were on dialysis before the treatment had regained kidney function by the end of the trial, compared to less than 20% in the historical control group.” says Coordinating Principle Investigator, Mårten Segelmark, Professor of Nephrology at Lund University, previously Linköping Universtity. “I am pleased to see these results being recognized in one of the leading nephrology journals, but I am even more excited for the many patients suffering from anti-GBM disease, as these data give hope. Today, most anti-GBM patients suffer terminal damage to their kidneys during the course of the disease. With standard of care, the disease leads to dialysis in most cases and even death in some cases. Dialysis dependence takes a huge toll on a person’s life, and is associated with chronic pain and depression, among other debilitating symptoms. Therefore, the opportunity to greatly reduce the risk of dialysis dependance represents a significant step forward for a patient group who currently have very few treatment options.”

Anti-GBM disease is a rare and acute autoimmune condition which can lead to permanent kidney failure and even death. It is caused when the immune system mistakenly attacks a specific part of the kidneys called the glomerular basement membrane (GBM), damaging the kidneys and in some cases the lungs also. Prognosis for the condition is poor, particularly because its rarity can lead to delayed diagnosis in a situation where, given the disease’s acute nature, prompt treatment is very important.

See-"Endopeptidase Cleavage of Anti-Glomerular Basement Membrane Antibodies in vivo in Severe Kidney Disease: An Open-Label Phase 2a Study"-Fredrik Uhlin, Wladimir Szpirt, Andreas Kronbichler, Annette Bruchfeld, et al., JASN March 2022, ASN.2021111460; DOI: https://doi.org/10.1681/ASN.2021111460.