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Abstract: TH-PO774

Histopathologic and Clinical Outcomes in Idiopathic and Diabetic Nodular Glomerulosclerosis

Session Information

Category: Glomerular Diseases

  • 1201 Glomerular Diseases: Fibrosis and Extracellular Matrix

Authors

  • Baig, Mirza Mukarram, Indiana University School of Medicine, Indianapolis, Indiana, United States
  • Eadon, Michael T., Indiana University School of Medicine, Indianapolis, Indiana, United States
  • Phillips, Carrie L., Indiana University Health, Indianapolis, Indiana, United States
  • Moe, Sharon M., Indiana University School of Medicine, Indianapolis, Indiana, United States
  • Moorthi, Ranjani N., Indiana University School of Medicine, Indianapolis, Indiana, United States
Background

Idiopathic nodular glomerulosclerosis (ING) is a rare form of glomerulopathy with an unclear pathogenesis. The hallmark of this entity is mesangial nodularity in the absence of diabetes mellitus or specific immune deposits on immunofluorescence. We hypothesized that ING and diabetic nephropathy (DN) have distinct histopathological features that explain their pathogenesis.

Methods


From Jan 2001 to Dec 2016, native renal biopsy interpretations were queried from Indiana University Health’s electronic database. Natural language processing followed by manual adjudication confirmed 49 cases of ING. Histopathologic features were compared between individuals with ING(N=49) and DN (N=770). Clinical and histopathological features of ING cohort were studied in detail and compared with previous cohorts.

Results

The mean age of ING cohort was 68 y with a Caucasian (73%) and male (55%) predominance. Clinical findings at presentation included renal insufficiency (81%) and nephrotic range proteinuria (53%). 94% had hypertension while 68% had a smoking history. All 49 patients had prominent diffuse mesangial sclerosis with focal or global nodularity. Interstitial fibrosis, tubular atrophy, and arteriosclerosis were present in >90% of patients. Among cases with follow up (N=25, median 616 days), 60% progressed to ESRD, 67% had declining renal function (creatinine increase of 0.5 mg/dL), and 38% died. Between the ING and DN cohorts, the only significant differences in histological findings were the degree of arteriosclerosis and podocyte foot process effacement, which were more prevalent and severe in the diabetic cohort (P<0.001).

Conclusion

In our cohort of ING, we confirmed the close association of hypertension, hyperlipidemia, and smoking observed in prior studies. We found minimal histologic differences reported between patients diagnosed with ING and DN. The histologic similarity of ING and DN glomerular lesions suggests there may be a common pathogenic process that is distinct from the presence or absence of diabetes mellitus. Future longitudinal studies are required to determine the pathogenesis of nodule formation in ING.