Fibronectin glomerulopathy

Abstract

Fibronectin glomerulopathy is a rare renal disorder which is found most commonly in family clusters, though in some instances isolated cases with no family history have been documented. Diagnosis requires a renal biopsywhich typically shows a mesangiopathy with increased matrix rich in fibronectin which may impart to the glomeruli a lobular appearance. While the ultrastructural findings can suggest and in some cases can be used to make a presumptive diagnosis, immunohistochemical stain for fibronectin is absolutely essential for making a definitive diagnosis. There is no real treatment for this condition, except for measures to delay progression of renal damage. Renal transplantation provides a feasible treatment modality when renal failure ensues, though recurrence of fibronectin glomerulopathy in transplanted kidneys has been documented in the literature. Genetic evaluation and counseling should be part of the management of these patients to identify affected individuals who are asymptomatic or early in the disease process and carriers that are at risk to develop the disease later in life, as the disease displays an autosomal dominant pattern of inheritance in many families.