Skip to Main Content

We have a new app!

Take the Access library with you wherever you go—easy access to books, videos, images, podcasts, personalized features, and more.

Download the Access App here: iOS and Android

Amyloidosis is a relatively rare cause of nephrotic syndrome. It is caused by tissue deposition of an overproduced and abnormally-folded protein (amyloid). Several different proteins can form amyloid fibrils with renal deposition. Primary amyloidosis, or AL amyloidosis, is the most common form and is due to a plasma cell dyscrasia causing overproduction and deposition of monoclonal Ig light chains (see Chapter 13). Secondary amyloidosis, or AA amyloidosis, can rarely occur in chronic inflammatory disease such as rheumatoid arthritis, IBD, or chronic infection; in these cases, there is deposition of an acute phase reactant, serum amyloid A protein. Other less common forms of amyloidosis may also be encountered.

Proteinuria, decreased GFR, and nephrotic syndrome are presenting symptoms and signs of renal involvement in amyloidosis; evidence of other organ involvement, such as the heart, is common. Serum and urine protein electrophoresis should be done as screening tests; if a monoclonal spike is found on either, serum free light chains should be quantified and the kappa:lambda ratio assessed. Amyloid-affected kidneys are often larger than 10 cm. Pathologically, glomeruli are filled with amorphous deposits that show green birefringence with Congo red staining.

AL amyloidosis progresses to ESKD in an average of 2–3 years. Five-year overall survival is less than 20%, with worse prognosis in those with advanced cardiac involvement. Standard treatment is a combination of melphalan, corticosteroids, and the proteosome inhibitor bortezomib; addition of daratumumab shows promise. Melphalan and autologous stem cell transplantation are associated with a high (45%) mortality rate but can induce remission in 80% of survivors; however, few patients are eligible for this treatment. In AA amyloidosis, remission can occur if the underlying disease is successfully treated. Renal transplantation is an option.

Hogan  JJ  et al. Dysproteinemia and the kidney: Core Curriculum 2019. Am J Kidney Dis. 2019;74:822.
[PubMed: 31331759]  
Kastritis  E  et al; ANDROMEDA Trial Investigators. Daratumumab-based treatment for immunoglobulin light-chain amyloidosis. N Engl J Med. 2021;385:46.
[PubMed: 34192431]  
Law  S  et al. Advances in diagnosis and treatment of cardiac and renal amyloidosis. Cardiol Clin. 2021;39:389.
[PubMed: 34247752]  
Law  S  et al. Renal transplant outcomes in amyloidosis. Nephrol Dial Transplant. 2021;36:355.
[PubMed: 33439995]  
Palladini  G  et al. Management of AL amyloidosis in 2020. Hematology Am Soc Hematol Educ Program. 2020;2020:363.
[PubMed: 33275753]  
Sidiqi  MH  et al. Autologous stem cell transplantation in patient with AL amyloidosis with impaired renal function. Bone Marrow Transplant. 2019;54:1775.
[PubMed: 30962503]  
Siligato  R  et al. Amyloidosis and glomerular diseases in Familial Mediterranean Fever. Medicina (Kaunas). 2021;57:1049.
[PubMed: 34684086]  

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.