Fibronectin Glomerulopathy



Fibronectin Glomerulopathy


Neeraja Kambham, MD










Renal biopsy in a patient with fibronectin glomerulopathy shows PAS-positive material image expanding the mesangium and subendothelium of an enlarged glomerulus.






Intense immunofluorescence staining image is seen in glomerular deposits with antisera to fibronectin, consistent with FN glomerulopathy. Staining for immunoglobulins and complement is negative.


TERMINOLOGY


Abbreviations



  • Fibronectin (FN) glomerulopathy


Definitions



  • Nonimmune lobular glomerulopathy with massive mesangial and subendothelial deposits composed of fibronectin


ETIOLOGY/PATHOGENESIS


Autosomal Dominant Inheritance



  • Mutations in fibronectin FN1 gene documented recently in some patients



    • FN1 gene mapped to region in chromosome 2q34


    • Abnormal FN may affect function of glomerular endothelial cells and podocytes


  • No role of villin, desmin, or uteroglobin genes


Glomerular Fibronectin Deposition

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Fibronectin Glomerulopathy
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