The animated videos in NORD’s Rare Disease Video Library provide brief introductions to rare disease topics for patients, caregivers, students, professionals and the public. NORD collaborates with medical experts, patient organizations, videographers and Osmosis to develop the videos, which are made possible by individual donations, educational grants and corporate sponsorships. NORD is solely responsible for the content.
Summary
IgA nephropathy is a kidney disease in which IgA, a protein meant to defend the body against foreign invaders, accumulates in the kidneys and damages them. This impairs their filtering function. As a result, the kidneys begin to let substances such as blood and protein leak into the urine.
This condition most often occurs in Caucasian and Asian males. It usually appears when people are in their teens to late 30’s but can occur at any age. Many cases resolve over time. However, in a subset of patients, the disease may not resolve and thus can lead to end-stage renal disease (ESRD) after 20-25 years. Rarely the condition can progress much more rapidly leading to renal failure within a few years, if not treated.
People who have this condition most often present with one or recurrent episodes of having blood in their urine (visible hematuria). These episodes usually occur during or right after an upper respiratory tract infection such as a cold, sore throat or a gastrointestinal infection.
Treatment includes drugs that aim to slow progression of the disease and others that aim to reduce inflammation. The choice of treatment is made based on a variety of factors including blood pressure, the amount of protein in the urine and the estimated kidney function.
Introduction
The kidneys are two fist sized organs located in the back under the rib cage that serve as a filter for blood. They remove excess fluid and waste as urine, while reabsorbing the proper amount of water and other chemicals that the body needs to function. The name IgA nephropathy comes from the fact that that the protein depositing in the kidney has the characteristics of a normal circulating immunoglobulin protein designated IgA. In the disease, the normal protein is changed somewhat causing it to deposit in the filters of the kidney and it to leak blood and or protein. It is also named Berger’s disease as it was first described by Berger and Hinglais in 1968.
