Abstract
Renal involvement is the most important cause of morbidity and mortality in systemic amyloidosis. This retrospective analysis was conducted to analyse the clinico-pathological characteristics of renal amyloidosis in a group of Sri Lankan patients undergoing renal biopsy. Renal amyloidosis was observed in 50/ 9712 (0.5%) renal biopsies. The underlying cause for amyloidosis was not known in most. Of the known causes multiple myeloma was the commonest. Nephrotic range proteinuria was the most common clinical outcome and most had grade I to III chronic kidney disease at the time of diagnosis. Glomerulosclerosis was associated with the deterioration of renal function.
Highlights
Renal amyloidosis is the most important cause of morbidity and mortality in systemic amyloidosis
Amyloid deposition causes damage resulting in chronic kidney disease (CKD) characterized by glomerulosclerosis, tubular atrophy and interstitial fibrosis and inflammation, which lead to end stage renal disease
There are more than 20 recognised chemical forms of amyloid, only three forms are commonly encountered, namely, amyloid light chain (AL) in primary amyloidosis associated plasma cell/B cell dyscrasias, serum amyloid A (AA) in reactive/secondary amyloidosis associated chronic inflammatory conditions such as rheumatoid arthritis, tuberculosis, bronchiectasis and transthyretin (TTR) associated with senile systemic amyloidosis and familial amyloid polyneuropathies
Summary
Renal amyloidosis is the most important cause of morbidity and mortality in systemic amyloidosis. In kidneys amyloid usually deposits in the glomeruli, arteries, tubules and interstitium may get involved. Amyloid deposition causes damage resulting in chronic kidney disease (CKD) characterized by glomerulosclerosis, tubular atrophy and interstitial fibrosis and inflammation, which lead to end stage renal disease. There are more than 20 recognised chemical forms of amyloid, only three forms are commonly encountered, namely, amyloid light chain (AL) in primary amyloidosis associated plasma cell/B cell dyscrasias, serum amyloid A (AA) in reactive/secondary amyloidosis associated chronic inflammatory conditions such as rheumatoid arthritis, tuberculosis, bronchiectasis and transthyretin (TTR) associated with senile systemic amyloidosis and familial amyloid polyneuropathies. Confirmation of tissue amyloid deposition is made histologically by congo red staining and visualisation under polarized light
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