Fibronectin Glomerulopathy– This is a hereditary disorder of the kidneys in which there is deposition of a protein called as fibronectin in the glomeruli resulting in various symptoms.
In this article, we will discuss about:
- Causes of Fibronectin Glomerulopathy
- Symptoms of Fibronectin Glomerulopathy
- Treatment for Fibronectin Glomerulopathy.
How Do We Define Fibronectin Glomerulopathy?
Fibronectin Glomerulopathy is a rare genetic glomerular disease which is caused as a result of deposition of protein fibronectin in the glomeruli. Fibronectin Glomerulopathy is a genetic disorder which is characterized by proteinuria, renal tubular acidosis, hematuria, along with hypertension eventually resulting in renal failure generally between 25-60 years of age. As stated, this is a very rare disorder and in the literature there are very few cases of Fibronectin Glomerulopathy.
Causes of Fibronectin Glomerulopathy
Fibronectin Glomerulopathy may develop in various age groups, although generally it is found in adolescents. Since Fibronectin Glomerulopathy is generally found in families, a genetic origin of this syndrome is the opinion of the researchers. In about half of the people affected with Fibronectin Glomerulopathy the disorder is caused by heterozygous mutations in FN1 gene (2q34).
Symptoms Of Fibronectin Glomerulopathy
The salient feature of Fibronectin Glomerulopathy is that of nephrotic syndrome along with severe hypertension. Presence of edema can also be observed, initially around the eyes and legs which then gradually spreads to become generalized. There is also presence of microscopic hematuria as well as proteinuria and hypoalbuminemia. Affected individuals may also have renal failure of some degree which can gradually worsen with time and ultimately leading to complete renal failure.
Diagnosis of Fibronectin Glomerulopathy
Diagnosis of Fibronectin Glomerulopathy is basically made through a renal biopsy. The findings in cases of Fibronectin Glomerulopathy will be enlargement of glomeruli along with slight immunoreactivity for immunoglobulins or complement factors. Electron microscopy may reveal deposits mainly in the subendothelial space. The most significant finding will be the immunoreactivity of glomerular deposits to fibronectin. If there is a family history of Fibronectin Glomerulopathy, then this also helps with the diagnosis.
Treatment For Fibronectin Glomerulopathy
As of now, there is no clear cut treatment for Fibronectin Glomerulopathy. Treatment mainly consists of controlling the symptoms by using steroids, diuretics, and aggressive treatment of hypertension. The renal failure can be slowed down by use of anti-proteinuric and renal protective treatments with ACE inhibitors. Dialysis is required for advanced cases of Fibronectin Glomerulopathy with complete renal failure.
Prognosis For Fibronectin Glomerulopathy
The prognosis of Fibronectin Glomerulopathy is unclear. In some cases, the disease has an extremely slow progression whereas in some cases, it may progress to end stage renal disease.
- Rare Diseases Info. “Fibronectin Glomerulopathy.” https://rarediseases.info.nih.gov/diseases/12743/fibronectin-glomerulopathy
- Orphanet. “Fibronectin Glomerulopathy.” https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=84093
- Kidney International Reports. “Fibronectin Glomerulopathy: A Rare Cause of Nephrotic Syndrome.” https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5434900/