Anti-glomerular basement membrane disease Trials in Disease

Study on Imlifidase and Drug Combination for Treating Severe Goodpasture Disease in Patients

This clinical trial is investigating a new approach to treating severe anti-GBM antibody disease, a rare autoimmune condition that causes the immune system to attack the kidneys and lungs. The study compares the effectiveness of an enzyme-based therapy called imlifidase combined with standard treatments against standard treatments alone.

Main inclusion criteria:

• Adults aged 18 years or older
• Presence of anti-GBM antibodies above toxic levels in the blood
• Blood detected in the urine (haematuria)
• Severely reduced kidney function with an eGFR (a measure of how well the kidneys filter blood) of less than 20 mL/min/1.73 m²
• Ability to provide informed consent and follow study procedures

Main exclusion criteria:

• History of severe allergic reactions to the study medication
• Currently pregnant or breastfeeding
• Other serious medical conditions that might interfere with the study
• Participation in another clinical trial within the last 30 days
• History of drug or alcohol abuse
• Known infections that could affect study results
• Previous organ transplant
• History of cancer within the last 5 years (except certain skin cancers)
• Uncontrolled high blood pressure

Study focus and goals: The trial aims to determine whether adding imlifidase to standard treatments can better preserve kidney function compared to standard treatments alone. Participants will be randomly assigned to receive either imlifidase (given as a single 11 mg intravenous infusion) plus standard care, or standard care only. Standard care includes medications such as cyclophosphamide, prednisolone, methylprednisolone (steroids to reduce inflammation), trimethoprim and sulfamethoxazole (to prevent infections), and loratadine (to manage allergic reactions).

Throughout the approximately 6-month study period, participants will undergo regular monitoring of their kidney function through blood tests. Follow-up visits are scheduled at 3 months and 6 months to assess kidney function, overall health, and any side effects. The study will also track how quickly disease-causing antibodies decrease to non-toxic levels.

Investigational drugs: Imlifidase is an enzyme-based therapy that works by breaking down the specific antibodies responsible for attacking the kidneys. By removing these harmful antibodies from the bloodstream, imlifidase may help protect kidney function and prevent further damage. It is delivered directly into the bloodstream through an intravenous infusion. The trial compares this new treatment approach against the usual standard-of-care medications that doctors typically use to manage the disease and control its symptoms.