Fibrillary glomerulopathy in a 10-year-old female

A 10-year-old African-American female was evaluated for asymptomatic proteinuria. Initial investigation did not reveal the etiology of her renal disease. She subsequently underwent a percutaneous renal biopsy, which was consistent with fibrillary glomerulopathy. This condition is rare in children and is identified histologically by a solid, randomly arranged, amyloid-like fibrillar structure with a diameter of about 18-22 nm. It is a diagnosis of exclusion, the clinical presentation of which can be quite varied. Children usually present with nephrotic syndrome. There is no effective therapy for this condition. Therapy with steroids, cytotoxic agents, and plasmapheresis does not confer any real benefit in stabilizing or improving renal function. However, angiotensin converting enzyme inhibitors can decrease the proteinuria. End-stage renal disease is known to occur in 50% of patients within 4 years of diagnosis. The nephrotic-range proteinuria in our patient significantly decreased during a 4-year follow-up without any therapy, while maintaining normal renal function.