renal amyloidosis; amyloid nephropathy
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Etiology
- amyloid light-chain - AL amyloidosis - multiple myeloma
- serum amyloid A protein - AA amyloidosis
- beta-2 microglobulin (hemodialysis-related amyloidosis)
Epidemiology
- 80% of patients with AL amyloidosis have renal involvement
Pathology
- amorphous, eosinophilic extracellular deposits in the glomeruli, along the tubules & in the renal blood vessels
- congo-red positive
- electron microscopy: 8-10 nm extracellular, non branching fibrils
Clinical manifestations
- nephrotic syndrome
- renal insufficiency
- progression to renal failure in 1-2 years is common
Laboratory
Management
- see multiple myeloma
- chemotherapy directed at B-lymphocytes
- melphalan & prednisone +/- colchicine
- reduction of systemic inflammation to reduce levels of serum amyloid A protein
More general terms
Additional terms
References
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 19. American College of Physicians, Philadelphia 1998, 2009, 2012, 2021
- ↑ Zhu X, Liu F, Liu Y, Liu H, Xu X, Peng Y, Sun L, Yuan S. Analysis of clinical and pathological characteristics of 28 cases with renal amyloidosis. Clin Lab. 2011;57(11-12):947-52 PMID: https://www.ncbi.nlm.nih.gov/pubmed/22239026
- ↑ Amyloidosis and Kidney Disease http://kidney.niddk.nih.gov/kudiseases/pubs/amyloidosis/index.htm