Introduction:
The kidney is one of the most commonly affected organs in amyloidosis. More than >35 structurally and functionally heterogeneous proteins known to have the predisposition to undergo misfolding and form amyloid fibrils, many have a predilection for the kidney. The overall prevalence of renal amyloidosis in native kidney biopsies is 1.6%. AL is the most common cause of renal amyloidosis (81%-86%), followed by AA (7%), then ALECT2 (2.5%-2.7%). In AL amyloidosis, the kidney is affected in up to 80% of patients. The most common manifestation of renal amyloidosis is proteinuria. Laboratory testing is dictated by history and clinical presentation, and definitive diagnosis of renal amyloidosis is made through renal biopsy.
Methods:
We retrospectively reviewed the records of ten biopsy proven renal amyloidosis patients at 3 months who were admitted in the department of nephrology over a course of 2 years duration.
Results:
Ten cases of renal amyloidosis were diagnosed histologically. There was marked male predominance with seven out of ten (70%) patients were male and age ranged from 38 yrs to 80 years with mean age of 51 years. Clinically most patients presented with nephrotic range proteinuria with mean proteinuria of 4 grams/24 hours. Renal insufficiency was found in four out of ten (40%) patients with mean serum creatinine of 2.2 mg/dl and only one patient required renal replacement therapy at the time of presentation. Seven out of ten (70%) patients received chemotherapy. Two out of ten (20%) patients expired and one patient was lost to follow up.
Conclusions:
In the absence of treatment, ongoing deposition of amyloid protein in the kidney results decline in kidney function and ultimately end stage kidney disease in most patients. Most of our patients presented with nephrotic range proteinuria and majority of the patients who received chemotherapy (57%) responded to chemotherapy.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.