A variety of systemic diseases are associated with IgA nephropathy

 IgA nephropathy

A variety of systemic diseases are associated with IgA nephropathy such as liver failure, celiac disease, rheumatoid arthritis, Reiter's disease, ankylosing spondylitis and HIV. Diagnosis of IgA Nephropathy and a search for any associated disease occasionally reveals such an underlying serious systemic disease. Occasionally, there are simultaneous symptoms of Henoch-Schönlein purpura; see below for more details on the association. Some HLA alleles have been suspected along with complement phenotypes as being genetic factors.

In IgA Nephropathy [IgAN], unknown agents cause the glomeruli to become — and to stay — inflamed. IgAN is the world’s most common glomerulonephritis [inflammation of the glomeruli], but its pathogenesis [how the disease develops] is not known. IgAN is considered to be an immune-complex mediated disorder (or immunologically mediated disorder), which means that immune complexes may not be the direct cause of the disease but they help bring about the end result, which is widespread inflammation of the kidneys.

What happens in IgAN is essentially a vicious cycle: inflammatory chemicals increase the permeability of the glomerular capillary wall, and that increased porosity enhances the absorption of other chemicals that stimulate cellular growth and, ultimately, structural damage. These changes take time because our kidneys have a lot of excess capacity built into them. The damage is, however, slowly progressive and, in the case of sclerosis [scarring], irreversible.

We do not know what antigen is responsible for triggering IgAN, nor do we know whether IgAN results from defects in the body’s creation or elimination of immune complexes. There is evidence that immunoglobulin A is overproduced in the bone marrow of IgAN patients; some patients also show decreased capacity to remove immune complexes from their systems.

Who is likely to get IgA nephropathy?

The condition is three times as common in males as females -but this difference is unexplained. It also seems to be more common in some parts of the world – especially Japan, Singapore and other countries in the Pacific Rim. It is more common in Southern Europe compared to Northern Europe. We do not know the reasons for these geographical differences.

It can be diagnosed at any age but most commonly in children and young adults who are likely to have the typical story of episodes of visible haematuria. Older people are less likely to have visible haematuria; they are more likely to have protein as well as blood in the urine at the time of diagnosis and to have hypertension and other evidence of more advanced kidney damage. We presume that these older people have in fact had IgA nephropathy for many years but because they never had visible haematuria or a routine urine test, it was not diagnosed until a much later stage.

Does IgA nephropathy affect any part of the body other than the kidneys?

No, it does not, but there is a condition called Henoch-Schönlein purpura (often shortened to HSP) which is related to IgA nephropathy. HSP gets its odd name from the two German doctors who originally described it over a hundred years ago. We now know that the kidney problem in HSP is very similar to IgA nephropathy; but patients with HSP also get a skin rash and may have aches and pains in their joints and attacks of abdominal pain. IgA is found in the skin as well as in the kidneys in HSP. The skin, joint and abdominal effects of HSP often go away in a few weeks or months, but the kidney problem can stay much longer, and may eventually cause kidney failure. HSP can occur at any age but is commonest in young children.

A proper diet can double the curative effect, but a bad diet may make your disease worse. So pay attention to the foods you eat, if you need any help in making the diet plan or cure your disease, you can email us. Our email: sjzkidneyhospital@hotmail.com