Nephrology

Answer 4 Light-chain deposition disease in the kidney. The combination of renal insufficiency, nephrotic proteinuria, and nodular glomerulosclerosis supports both light-chain deposition disease and light-chain (AL) amyloidosis as the most likely renal lesions. Light-chain deposition disease is more often associated with kappa light chains, while amyloidosis is associated with lambda light chains. The distinguishing feature in this case is the presence of granular deposits along the basement membranes and in the glomerular nodules on electron microscopy, which support a diagnosis of light-chain deposition disease. In contrast, AL amyloidosis is characterized by fibrillar deposits (8-10 nm in diameter) on electron microscopy. Hypertensive arteriolonephrosclerosis is extremely unlikely in the presence of nephrotic proteinuria and the noted histologic findings. Multiple myeloma cast nephropathy is not complicated by nephrotic proteinuria, and the classic lesions seen on renal biopsy are fractured proteinacious casts in the distal tubular lumens, often associated with multinucleated giant cells. Click here to return to the questions

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