Fibrillary glomerulonephritis: An apparent familial form?
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 disease...
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Published in | Nephrology (Carlton, Vic.) Vol. 20; no. 7; pp. 506 - 509 |
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Main Authors | , , , , |
Format | Journal Article |
Language | English |
Published |
Australia
Blackwell Publishing Ltd
01.07.2015
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Subjects | |
Online Access | Get full text |
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Summary: | 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. |
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Bibliography: | ark:/67375/WNG-0B924PF0-0 Amgen Genzyme istex:E043EC42000B951FB2DCC59A9CB51BB622D9B805 ArticleID:NEP12447 ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3 |
ISSN: | 1320-5358 1440-1797 |
DOI: | 10.1111/nep.12447 |