True about fibronectin nephropathy are all except-
Fibronectin glomerulopathy occurs between the second and fifth decades of life in most patients, and it is known to be slowly progressive with mild proteinuria leading to kidney failure. The case of a 78-year-old woman with a rapid course of nephrotic syndrome due to fibronectin glomerulopathy is repoed. She had proteinuria that rapidly increased to 6.8 g/day in a month and microscopic haematuria. A renal biopsy specimen showed lobular glomerulopathy and membranoproliferative glomerulonephritis-like lesions on light microscopy. There was scanty staining for immunoglobulins and complement. Electron microscopy revealed granular deposits with fibril formation. Immunohistochemistry of the fibronectin showed intense staining in the mesangium and peripheral loop. Therefore, this case was diagnosed as fibronectin glomerulopathy. The kidney function was rapidly decreasing, necessitating haemodialysis 2 months after renal biopsy. It is impoant to consider fibronectin glomerulopathy in the differential diagnosis of nephrotic syndrome in older people.
Autosomal recessive inheritance
Gomerular enlargement and PAS+ trichrome mesangial deposit
Glomerulus do not consistently stain
Ultrastructural feature is presence of large electron mesangial or subendothelial deposit
Urinary tract