In the European Union (EU), Orphan Drug designation is given to drugs or biologics intending to improve the treatment, diagnosis, or prevention of rare or serious illnesses. To be considered “rare,” a condition must affect under 5 in every 10,000 people and either have no current standard-of-care or who could be significantly benefited by new care. According to a news release from December 2021, atrasentan, a therapy for primary IgA nephropathy (IgAN) recently earned this status in the EU.
Learn more about Orphan Drug status and its many incentives and benefits here.
Atrasentan
Developed by biopharmaceutical company Chinook Therapeutics, Inc. (“Chinook”), atrasentan is a selective endothelin A receptor (ETA) inhibitor. Researchers believe it can not only benefit those with IgAN, but other chronic kidney conditions as well. Atrasentan works by inhibiting ETA and thus reducing proteinuria (excess protein in the liver), fibrosis (scarring), and inflammation. Ultimately, this helps to maintain kidney function. Learn more about atrasentan here.
Moving forward, Chinook hopes to evaluate atrasentan for IgAN and other kidney conditions within two separate clinical trials.
IgA Nephropathy (IgAN)
Also known as Berger’s disease, IgA nephropathy (IgAN) is a kidney disease caused by excess immunoglobin A (IgA) buildup. Normally, IgA (a type of antibody) helps protect the body from foreign invaders and plays a role in immune response. But excess IgA causes inflammation, particularly in the glomeruli (or the area of the kidney which filter waste and fluid). Typically, IgAN follows viral infections, though doctors are not sure of the exact cause. Others believe it to be an autoimmune disorder. Regardless, IgAN causes kidney inflammation and damage. It typically manifests during teenage years and is 2x more likely to affect men. Symptoms include:
- Hematuria (blood in the urine)
- Proteinuria (excess protein in the urine)
- Fatigue and/or drowsiness
- High blood pressure
- Dry skin
- Appetite loss
- Unintended weight loss
- Abdominal, loin, or back pain
- Swelling of the hands and feet
- Kidney failure
