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Autosomal recessive inheritance Fibronectin nephropathy It is one of the fibrillary glomerulonephritis. Fibronectin nephropathy is an atypical form of lobular glomerulonephritis with massive mesangial expansion by intensely PAS positive deposits that were silver and congo red negative. These deposits are made up of "Fibronectin". Fibronectin is a multifocal extracellular matrix glycoprotein that is active in cellular adhesion and migration. Fibronectin is produced locally in the glomerulus by mesangial cells. Some of it is also produced in the liver which constitues the plasma fibronectin. Increased production of fibronectin protein may lead to glotnerular disease. Fibronectin nephropathy is an atypical fonn of lobular glomerulonephritis with massive subendothelialQ and mesangialf2 focal .fibrillary electron dense deposits. Clinical features :- It is an autosomal dominante glomerulonephritis Commonly presents with nephritic range proteinuria Can sometimes present with microscopic hematuria and mild hypeension. Pathology of Fibronectin nephropathy * Glomerular enlargement and lobulation due Light microscopic - PAS and Trichome positive features - Congo red negative * Massive mesangial proliferationdeg * The capillaries remain patent although reduced in size at periphery lmmunoflorescence * Glomendi do not stain with immunoglobulin or complement2 * Immutwhistological stains fore fibronectin strongly positive Electron microscopy / * Massive mesangialdeg and subendothelice electron dense Ultrastructural findings deposits that reflects the location of PAS positive fibronectin deposits Fibronectin nephropathy is considered to be one of the fibrillaty nephropathy
Autosomal recessive inheritance
Gomerular enlargement and PAS+ trichrome mesangial deposit
Glomerulus do not consistently stain for Ig and complement
Ultrastructural feature is presence of large electron mesangial or subendothelial deposit