Hansa Biopharma has announced positive 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.

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.

“We are very encouraged by the positive outcome from the phase 2 trial in anti-GBM antibody disease. Anti-GBM is the first IgG-mediated disease outside transplantation, where imlifidase has been shown to stop an immunologic attack. This marks an important milestone in Hansa Biopharma’s efforts to develop potentially lifesaving and life altering therapies for patients with rare immunological diseases within a range of therapeutic areas where there is a significant unmet medical need,” says Søren Tulstrup, President and CEO, Hansa Biopharma.

Encouraging results

The first high-level data read out has been completed and the encouraging results indicate that imlifidase treatment may lead to increased renal survival in patients with anti-GBM antibody disease due to rapid clearance of IgG antibodies. The phase 2 anti-GBM study enrolled 15 subjects, six females and nine males with a median age of 61 years of age (range 19-79 years) recruited across centers from five European countries, including Sweden, Denmark, Czech Republic, France and Austria.

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). The safety profile of imlifidase in the population was concluded as being favourable.

Orphan drug designation

Hansa Biopharma was granted orphan drug designation for imlifidase as a treatment for anti-GBM antibody disease in both the EU and the US in 2018.

Photo of Søren Tulstrup, President and CEO, Hansa Biopharma