Primary IgA Nephropathy (Berger's disease) is an autoimmune kidney disease
featured with IgA glomerulonephritis as a result of the glomerular immune
complex deposit formation in the kidney.
This disease usually occurs in adolescents or young adults between the ages
of 15~35. Men are affected two or three times more often than women. It occurs
significantly more often in native Americans than in any other ethnic groups. It
is more prevalent in Caucasians than in African Americans, and is one of the
leading causes of acute nephritis in young people in the USA, Europe, and Japan.
According to one study of young men in the military, there is an annual
occurrence of 94 cases of Berger's disease out of 100,000 male inductees.
Pathogenesis of IgA Nephropathy (Berger's disease): Immune Dysfunction
The immune system protects our body from potentially harmful substances by
recognizing and responding the antigens. Antigens are molecules (usually
proteins) on the surface of cells, viruses, fungi, or bacteria. The immune
system recognizes and destroys substances that contain these antigens.
Our immune system can be divided into two major branches: the innate immune
system and the adaptive immune system. Normally, the innate immune system works
alone to fight off the invaders, and only when it fails to function adequately,
the adaptive immune system can be activated to help with keeping a healthy
state.
Immune dysfunction will appear when the innate immune system continuously
sending signals to make the adaptive immune system keep working, or the latter
is unable to control itself and also function excessively. So hypersensitivity
can be caused.
There are massive immune complex deposits formed in the body produced by the
combination of antigen-antibody. They will deposit in the mesangium of the
kidneys through blood circulation. The hypersensitivity fail to remove these
foreign harmful substances, and also leads to damage to healthy renal cells and
tissues. Over time, kidney damage can be caused in patients with IgA
Nephropathy.