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Diagnosis: Idiopatic Nodular Glomerulosclerosis

Idiopathic nodular mesangial sclerosis, also called idiopathic nodular glomerulosclerosis (ING) or non-diabetic nodular glomerulosclerosis, is an uncommon form of glomerular disease associated with a poor prognosis. ING was described by Alpers and Biava in 1989 as a diagnosis of exclusion, characterized by nodular mesangial matrix expansion without an alternate predisposing clinical diagnosis. ING is presently considered a distinct clinical–pathological entity with light microscopic and electron microscopic features indistinguishable from those of diabetic nodular glomerulosclerosis or nodular diabetic nephropathy (DN), but without evidence of abnormal glucose metabolism or other specific diseases. The disease is uncommon, with only a handful of prior case series examining the clinical and histopathologic features. These prior studies demonstrated a close association with hypertension and smoking. A case series of 15 patients by Li and Verani also found a significantly higher than expected incidence of obesity in patients with ING. The clinical presentation of ING is similar to other glomerular diseases and is characterized by renal failure and proteinuria. Despite these associations, the pathophysiology of ING remains uncharacterized. Proposed mechanisms have included elevated advanced glycosylation end products, increased insulin resistance in the absence of clinical diabetes, free radical oxidative stress from cigarette smoke, hormonal signaling from obesity, increased extracellular matrix production and angiogenesis, and hypoxia, although data supporting each potential mechanism are limited. Because of the paucity of information regarding this disease and lack of prospective trials, best treatment practices remain unclear (Text taken from: Eadon MT, et al. Clinical, histopathologic and molecular features of idiopathic and diabetic nodular mesangial sclerosis in humans. Nephrol Dial Transplant. 2021;37(1):72-84. [PubMed link]).

See the chapter: Diabetic nephropathy of our Tutorial.

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