A Case of Collagenofibrotic Glomerulopathy with Characteristic Electron Microscopic and Immunohistochemistry Findings / 대한신장학회잡지

ABSTRACT

Collagenofibrotic glomerulopathy is a relatively rare glomerular disease characterized by the accumulation of spiraled frayed collagen fibrils in mesangial and subendothelial areas. Clinically, patients with the disease usually present with moderate proteinuria and edema. They tend to have hypertension and their renal function deteriorates slowly. We report the case of a patient with collagenofibrotic glomerulopathy who also had Takayasus arteritis and presented with hypertension, proteinuria and dyspnea on exertion. Electron microscopy of the renal biopsy revealed massive accumulation of peculiar collagen fibers and immunohistology using monoclonal antibodies to collagen type III revealed positive stain in glomerular tufts.