Most of the pathology in the kidney from AL Amyloidosis is glomerular in origin. Cases have been noted of pure selective amyloid deposition in the vessels walls of different organs. The clinical presentation of renal amyloidosis basically depends on the distribution and severity of amyloid deposits. Vascular localization represents an uncommon pattern of renal amyloidosis, generally associated with chronic renal failure with minimal or no proteinuria.
An old series in 1983, of nine patients with secondary (AA type) renal amyloidosis with little or no proteinuria has been reported. Renal failure was the presenting sign of renal disease in seven patients. Renal biopsy revealed a predominantly vascular deposition of amyloid in all patients. Three patients had no glomerular amyloid deposits. This pattern of amyloid deposition was found in 12.5% of our renal biopsies from patient with amyloidosis.
Another recent paper from Japan describes the vascular distribution of amyloid and how that changes when it affects the kidney. This paper looked at patient biopsy samples with AL amyloidosis and divided them into a group with capillary form and a group with small vessel form. The small vessel form was associated with more cardiac involvement, and left ventricular thickening compared to the capillary form. There was no significant differences in rates of survival and renal survival.
In summary, vascular amyloid can been seen in the kidney, usually when you are suspecting it even without nephrotic syndrome.
Image Source: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675(06)70652-0
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