Renal amyloidosis



Renal amyloidosis is the deposition of proteinaceous substances (deposits) in the mesangium along the glomerular basement membrane.We classify renal amyloidosis as the secondary glomerulonephritis. We have to mentione that amyloidosis is a systemic disease - amyloid is stored throughout the body. Renal damage caused by amyloidosis manifests itself as a nephrotic syndrome (mainly dominated by proteinuria), gradually progressing to renal insufficiency. The disease includes non-specific manifestations such as fatigue, weakness, weight loss.

Primary amyloidosis AL
Primary amyloidosis AL occurs in diseases such as MGUS (monoclonal gammopathy of unclear significance), myeloma, Waldenström's macroglobulinemia , CLL, lymphomas. Usually, light chain (lambda) fragments are deposited. Less common are deposits of heavy chain (kappa) fragments, sometimes referred to as amyloidosis AH ( heavy chain). Other organ disorders: GIT, heart (significantly worsens the prognosis), peripheral nerves, macroglossia, carpal tunnel syndrome.

Therapy:


 * Conservative: melphalan in combination with prednisone ,
 * Causal treatment: bone marrow transplantation.

The prognosis of untreated AL amyloidosis is poor (12 months). Due to the nature of the disease, it is necessary to screen for organ involvement (heart echo, kidney biopsy, trepanobiopsy).

Secondary amyloidosis AA
The so-called reactive amyloidosis, occurs in chronic inflammatory diseases (rheumatoid arthritis, ankylosing spondylitis, bronchiectasis, osteomyelitis, juvenile arthritis). AA amyloidosis mainly affects the kidneys and GIT (hepatosplenomegaly, diarrhea-malabsorption).

Therapy:


 * by treating the underlying disease, we achieve the remission of nephrotic syndrome.

Related articles

 * Amyloidosis
 * Kidney
 * Glomerulopathy manifested by nephrotic syndrome
 * Secondar renal amyloidosis
 * Dialysis amyloidosis