Fibrillary glomerulonephritis: An apparent familial form?

• Published in: Nephrology (Carlton, Vic.) Vol. 20; no. 7; pp. 506 - 509
• Main Authors: Ying, Tracey, Hill, Prue, Desmond, Michael, Agar, John, Mallett, Andrew
• Format: Journal Article
• Language: English
• Published: Australia Blackwell Publishing Ltd 01.07.2015
• Subjects: Adult; chronic kidney disease; familial nephritis; Female; glomerulonephritis; Glomerulonephritis - genetics; Glomerulonephritis - pathology; hereditary nephritis; Humans; Male; Middle Aged; nephrotic syndrome; Pedigree; familial nephritis; chronic kidney disease; hereditary nephritis; glomerulonephritis; nephrotic syndrome
• ISSN: 1320-5358; 1440-1797
• DOI: 10.1111/nep.12447

Fibrillary glomerulonephritis is a rare cause of glomerulonephritis characterized by non‐amyloid fibrillary deposits of unknown aetiology. It is generally considered idiopathic but may be associated with secondary causes such as monoclonal gammopathy, hepatitis B and C infections, autoimmune diseases and malignancies. We report two Australian families with apparent familial fibrillary glomerulonephritis inherited in an autosomal dominant pattern, and postulate the existence of a primary familial entity. Family 1 consists of an affected father and daughter; the daughter progressed to end‐stage renal failure within 18 months of diagnosis, despite immunosuppressive therapy. The father, however, remains stable at 10 months follow up. Family 2 comprises an affected mother and son; the mother commenced haemodialysis 5 years after diagnosis and subsequently underwent successful renal transplantation. The son is presently stable at last follow‐up after 5 years. A further review of the second family history reveals a third family member (maternal father) dying of ‘Bright's disease’. We describe their histopathology, clinical progression and treatment outcomes, and provide a review of the current understanding of this heterogeneous condition that is associated with poor renal outcomes.