Lobular glomerulopathy: is it always membranoproliferative glomerulo-nephritis?

Abstract

Membranoproliferative glomerulonephritis is a term that is used for a number of glomerulopathies, which have similar light microscopy pictures, but differ in etiology, pathogenesis, immunohistochemical and ultrastructural changes in the renal parenchyma. Here we present a clinical case of a 16-year-old girl who had proteinuria when she 2.5 years old. She was diagnosed with chronic membranoproliferative glomerulonephritis with light microscopy at the age of 13. However, with later nephrobiopsy performed in our clinic with the use of a three-component morphological study allowed us to establish a rare histological manifestation of the disease, fibronectin glomerulopathy. Glomerulopathy with fibronectin deposits is a rare hereditary kidney disease, characterized by extensive deposition of fibronectin in the glomeruli, especially in mesangial regions and subendothelial zones. Refers to the group of glomerulopathies with the formation of microfibrils. Differential diagnosis between the fibrillary and immunotactoid glomerulopathies is necessary. The combination of electron microscopy and immunohistochemical data makes it possible to clarify the nature of the damage of renal tissue. So far, there are reliable methods for treatment of fibronectin glomerulopathy. The disease was reported as slowly progressing, leading to kidney failure in the most cases.