KimmelstielWilson

Kimmelstiel-Wilson disease, also known as nodular glomerulosclerosis, is a form of diabetic nephropathy characterized by nodular sclerosis of the glomeruli. It was described in 1936 by Paul L. Kimmelstiel and his colleague Wilson in patients with long-standing diabetes mellitus. The condition is a hallmark of diabetic kidney disease and contributes to progressive kidney failure when glycemic and blood pressure control are not maintained.

Pathologically, the hallmark is nodular mesangial sclerosis, with nodular glomerular lesions (Kimmelstiel-Wilson nodules) in the mesangium, along with thickening of the glomerular basement membrane and arteriolar hyalinosis. These changes lead to impaired filtration and proteinuria. In later stages, interstitial fibrosis and tubular atrophy may accompany the glomerular lesions. Immunofluorescence is typically nonspecific and lacks immune complex deposition, reflecting a nonimmune etiology.

Clinically, patients usually have long-standing diabetes with progressive proteinuria, starting as microalbuminuria and potentially advancing to overt proteinuria and reduced glomerular filtration rate. Hypertension and edema are common. Retinopathy is frequently present, linking retinal and renal microvascular complications. Diagnosis is based on clinical context and, when necessary, renal biopsy showing nodular glomerulosclerosis, with electron microscopy revealing mesangial expansion and basement membrane thickening.

Management focuses on controlling blood glucose and blood pressure, often using renin–angiotensin system inhibitors to slow nephropathy. Other strategies include lipid management and avoidance of nephrotoxins, with preparation for renal replacement therapy in advanced disease. Prognosis varies and depends on glycemic control and blood pressure management; progression to end-stage renal disease occurs in a substantial minority of patients despite therapy.

The eponym honors Kimmelstiel and Wilson for their early description of these nodular changes in diabetic nephropathy.