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Causes of Primary and Secondary Amyloid Nephropathy

2012-09-27 16:33

Amyloid nephropathy is caused by the deposition of amyloid proteins in kidneys and there are mainly 3 sources of these proteins.

1. Primary amyloid nephropathy caused by the deposition of AL protein (amyloid protein);

2. Secondary amyloid nephropathy caused by AA protein (amyloid protein A);

3. Amyloidosis related to plasma cell disorders are also caused by AL protein.

Primary (idiopathic) amyloid

It is caused by immune disorders, denaturation and degradation of the connective tissues and metabolic disorder of the proteins which will produce too much amyloid fibers. These amyloid fibers can circulate with blood flow and deposit in tissues and organs resulting in damages to their normal structure and functions. The glomeurli are replaced by large amount of amyloid materials and the renal tubules will atrophy, thus causing a series of clinical manifestations such as proteinuria, nephrotic syndrome, renal insufficiency, etc.

Secondary amyloid nephropathy

1. Neoplastic diseases such as multiple myeloma, Hodgkin’s disease, medullary thyroid tumor, leukemia, etc

2. Infectious diseases such as chronic pyogenic infections, tuberculosis, syphilis, etc.

3. Immunological diseases such as rheumatoid arthritis.

4. Familial amyloidosis.

There are about 3/4 secondary amyloidosis patients will develop amyloid nephropathy and there are only 1/4 primary amyloidosis can have renal damages, therefore in case of the above mentioned medical conditions which can cause amyloidosis and amyloid nephropathy, proper and timely treatments should be received to prevent further deterioration and lower the chance of amyloidosis and renal damages.

The most reliable test to confirm the diagnosis is renal biopsy. Other imaging examinations such as B ultrasound, IVP (intravenous pyelogram) can show enlargement of the kidneys especially in the stage of renal failure the kidney will not atrophy. Once amyloid nephropathy is confirmed, it needs to know weather it is primary or secondary so as to have targeted medicines and treatments.


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