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renal amyloidosis; amyloid nephropathy
Etiology:
1) amyloid light-chain - AL amyloidosis - multiple myeloma
2) serum amyloid A protein - AA amyloidosis
a) tuberculosis
b) injection drug use
c) osteomyelitis
d) rheumatoid arthritis
3) beta-2 microglobulin (hemodialysis-related amyloidosis)
Epidemiology:
- 80% of patients with AL amyloidosis have renal involvement
Pathology:
1) amorphous, eosinophilic extracellular deposits in the glomeruli, along the tubules & in the renal blood vessels
2) congo-red positive
3) electron microscopy: 8-10 nm extracellular, non branching fibrils
Clinical manifestations:
1) nephrotic syndrome
2) renal insufficiency
3) progression to renal failure in 1-2 years is common
Laboratory:
1) urinalysis: albuminuria, proteinuria
2) renal biopsy
Management:
1) see multiple myeloma
2) chemotherapy directed at B-lymphocytes
- melphalan & prednisone +/- colchicine
3) reduction of systemic inflammation to reduce levels of serum amyloid A protein
Related
hemodialysis-related amyloidosis
light-chain deposition disease; Light chain nephropathy
General
amyloidosis
kidney disease; renal disease
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: 22239026
- Amyloidosis and Kidney Disease
http://kidney.niddk.nih.gov/kudiseases/pubs/amyloidosis/index.htm