title: Fibrillary glomerulonephritis: An apparent familial form?
abstract: 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.
authors.affiliation: Department of Renal Medicine, University Hospital, Geelong, Victoria, Australia.
authors.forename: Tracey
authors.initials: T
authors.lastname: Ying
authors.affiliation: Department of Anatomical Pathology, St Vincent's Hospital, Fitzroy, Victoria, Australia.
authors.forename: Prue
authors.initials: P
authors.lastname: Hill
authors.affiliation: Department of Renal Medicine, University Hospital, Geelong, Victoria, Australia.
authors.forename: Michael
authors.initials: M
authors.lastname: Desmond
authors.affiliation: Department of Renal Medicine, University Hospital, Geelong, Victoria, Australia.
authors.forename: John
authors.initials: J
authors.lastname: Agar
authors.affiliation: Department of Renal Medicine, Royal Brisbane and Women's Hospital, Herston, Queensland, Australia.
authors.forename: Andrew
authors.identifier: http://orcid.org/0000-0002-8752-2551
authors.initials: A
authors.lastname: Mallett
mesh_terms: D000328:Adult; D005260:Female; D005921:Glomerulonephritis / Q000235:genetics* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree
keywords: chronic kidney disease; familial nephritis; glomerulonephritis; hereditary nephritis; nephrotic syndrome