Familial Fibrillary Glomerulonephritis: A Rare Case with Histopathological Resemblance to Membranous Nephropathy

Abstract

Fibrillary glomerulonephritis is a rare form of glomerulonephritis, characterized by the deposition of haphazardly arranged fibrils in the glomeruli, leading to renal dysfunction. Here, we present the first familial case of fibrillary glomerulonephritis reported from Türkiye, highlighting its histopathological resemblance to membranous nephropathy. A 45-year-old female with a family history of kidney disease presented with edema and was diagnosed with fibrillary glomerulonephritis. Immunohistochemical staining for DNAJB9 confirmed the diagnosis. The literature review revealed only six reported families with familial fibrillary glomerulonephritis, suggesting a possible autosomal dominant inheritance pattern. Compared to previous cases, our patient and affected family members are female and diagnosed at ages 40-50. The patient's treatment involves angiotensin receptor blockade due to non-nephrotic proteinuria and normal renal function. The prognosis for fibrillary glomerulonephritis is poor, and therapeutic options are limited. Future research should focus on understanding the genetic basis of familial fibrillary glomerulonephritis and developing effective treatments. This case underscores the importance of considering fibrillary glomerulonephritis in the differential diagnosis of glomerulonephritis, especially in familial cases, and highlights the need for further studies to improve its management.