A Case of Idiopathic Nodular Glomerulosclerosis Presenting Rapid Progressive Renal Failure / 대한신장학회잡지

Idiopathic nodular glomerulosclerosis is an unusual entity with histopathologic features resembling nodular diabetic glomerulosclerosis but occurs in non diabetic patients and predominantly in older males with smoking and long standing hypertension. It is characterized histologically by nodular mesangial sclerosis, glomerular basement membrane thickening, and arteriolosclerosis in the absence of immune-type deposit, and clinical presentations typically include renal insufficiency and severe proteinuria with relatively poor prognosis. We experienced a rare case of idiopathic nodular glomerulosclerosis presenting rapid progressive renal failure in a 42-year-old man without clinical evidence of diabetes. He was admitted with peripheral edema and renal insufficiency. He had a history of hypertension for 2 years and chronic hepatitis B for 5 years. Funduscopy and renal ultrasound were normal. HbA1c and oral glucose tolerance test were normal and on serum and urine electrophoresis, M-spike was not found. Light microscopic examination demonstrated the characteristic features of lobular glomerulonephritis showing glomerular hypertrophy and nodular mesangial sclerosis. Immunofluorescence was negative for immune-type deposits. Electron microscopy showed marked increase of mesangial matrix, mesangial cell proliferation, moderate effacement of foot processes, and glomerular basement thickening without electron-dense deposits or other specific fibrils. Idiopathic nodular glomerulosclerosis was diagnosed. After discharge, he continued smoking and his blood pressure was not controlled. Three months after first biopsy, second biopsy was performed because of increasement of serum creatinine and specimens showed progression of tubulointerstitial change and nodular mesangial sclerosis. Subsequent serum creatinine was progressively increased and hemodialysis was started 13 months after diagnosis.

A Case of Idiopathic Nodular Glomerulosclerosis / 대한신장학회잡지

A 45-year-old woman was admitted with proteinuria, hematuria and intermittent generalized edema. On admission, hypertension, chronic B-viral hepatitis were evident together with a decreased renal function. Light and electron microscopy revealed diffuse and nodular glomerulosclerosis indistinguishable from diabetic nodular glomerulosclerosis. Diabetes mellitus, however, had been excluded both by extensive clinical and by laboratory investigation. The differential diagnosis also included primary and secondary membranoproliferative glomerulonephritis, thrombotic microangiopathy, amyloidosis, monoclonal immunoglobulin deposition disease, fibrillary glomerolonephritis, immunotactoid glomerulopathy, light chain deposition disease. However, these entities could be excluded by immunohistochemistry and ultrastructural investigations. We report a case of idiopathic nodular glomerulosclerosis, which has not been previously reported in the Korean literature.