Hansa Biopharma reported data from an investigator-initiated phase 2 trial that evaluated safety, tolerability and efficacy of imlifidase in 15 patients with severe anti-GBM antibody disease. Results demonstrate the efficacy and safety of imlifidase in this patient population.
Anti-GBM antibody disease, also known as Goodpasture’s disease, is a severe autoimmune disease where the immune system mistakenly develops IgG-antibodies directed against the glomerular basement membrane (GBM), resulting in an acute immune attack causing severe kidney injury and, in some patients, also injuring the lungs.
The phase 2 anti-GBM study enrolled 15 subjects with a median age of 61 years of age (range 19-79 years) across centers from five European countries, including Sweden, Denmark, Czech Republic, France and Austria. The first high-level data read out indicate that imlifidase treatment may lead to increased renal survival in patients with anti-GBM antibody disease due to rapid clearance of IgG antibodies. At inclusion, ten of the patients were dialysis dependent, including five that were oliguric/anuric, while five patients were dialysis independent but had eGFR levels below 15 ml/min. Six hours after imflidase no patient had anti-GBM antibody levels above the normal range. At six months, ten patients were dialysis independent (median eGFR 27 ml/min). Four patients were dialysis dependent, while one patient had died (unrelated to imlifidase treatment). Imlifidase was generally well tolerated.
Imlifidase as a treatment for anti-GBM antibody disease has been granted Orphan drug designation in both the EU and the US.
Imlifidase is a unique antibody-cleaving enzyme originating from Streptococcus pyogenes that specifically targets IgG and inhibits IgG-mediated immune response. Using imlifidase is a novel approach to eliminate pathogenic IgG. It has a rapid onset of action, cleaving IgG-antibodies and inhibiting their reactivity within hours after administration.
About Anti-GBM antibody disease
Anti-GBM antibody disease, also known as Goodpasture’s disease, is a severe autoimmune disease where the immune system mistakenly develops IgG-antibodies directed against the glomerular basement membrane (GBM), resulting in an acute immune attack causing severe kidney injury and, in some patients, also injuring the lungs. Anti-GBM is a rare and acute immunological disease with a significant unmet medical need that affects approximately 1.5 in a million people annually, with a majority of patients losing their kidney function and requiring chronic dialysis and kidney transplantation. In severe cases, anti-GBM antibody disease may lead to death.