AIMS: Fibronectin glomerulopathy is a rare glomerular disease caused by the progressive deposition of fibronectin. We report 10 Chinese patients with fibronectin glomerulopathy. METHODS: Renal biopsies were performed on all patients, and the clinical and pathological parameters for all patients were analyzed. RESULTS: There were 6 males and 4 females, with an average age of 29±8 years. One patient had a family history of renal disease, all patients presented with proteinuria, and 80% of them suffered nephrotic range proteinuria. No patient demonstrated gross hematuria. The levels of serum creatinine were elevated, and the eGFR was decreased in 5 patients. Renal biopsy revealed a lobulated glomerular tuft. Patients showed numerous periodic acid-Schiff-positive and fuchsinophilic deposits in the mesangial area and along the capillary loops. Immense levels of fibronectin were detected in the glomerulus after immunofluorescence analysis. An electron microscopy scan found numerous electron-dense deposits in the mesangial and subendothelial areas. Immune-electron microscopy confirmed that the deposits consisted of fibronectin. CONCLUSION: Nephrotic proteinuria and massive intraglomerular fibronectin deposits are the most significant features of fibronectin glomerulopathy.
AIMS: Fibronectinglomerulopathy is a rare glomerular disease caused by the progressive deposition of fibronectin. We report 10 Chinese patients with fibronectinglomerulopathy. METHODS: Renal biopsies were performed on all patients, and the clinical and pathological parameters for all patients were analyzed. RESULTS: There were 6 males and 4 females, with an average age of 29±8 years. One patient had a family history of renal disease, all patients presented with proteinuria, and 80% of them suffered nephrotic range proteinuria. No patient demonstrated gross hematuria. The levels of serum creatinine were elevated, and the eGFR was decreased in 5 patients. Renal biopsy revealed a lobulated glomerular tuft. Patients showed numerous periodic acid-Schiff-positive and fuchsinophilic deposits in the mesangial area and along the capillary loops. Immense levels of fibronectin were detected in the glomerulus after immunofluorescence analysis. An electron microscopy scan found numerous electron-dense deposits in the mesangial and subendothelial areas. Immune-electron microscopy confirmed that the deposits consisted of fibronectin. CONCLUSION:Nephrotic proteinuria and massive intraglomerular fibronectin deposits are the most significant features of fibronectinglomerulopathy.