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 |
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Australia
Blackwell Publishing Ltd
01.07.2015
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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. |
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AbstractList | 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. 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 A ustralian 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. |
Author | Hill, Prue Desmond, Michael Mallett, Andrew Agar, John Ying, Tracey |
Author_xml | – sequence: 1 givenname: Tracey surname: Ying fullname: Ying, Tracey email: traceyying@gmail.com organization: Department of Renal Medicine, University Hospital, Victoria, Geelong, Australia – sequence: 2 givenname: Prue surname: Hill fullname: Hill, Prue organization: Department of Anatomical Pathology, St Vincent's Hospital, Victoria, Fitzroy, Australia – sequence: 3 givenname: Michael surname: Desmond fullname: Desmond, Michael organization: Department of Renal Medicine, University Hospital, Victoria, Geelong, Australia – sequence: 4 givenname: John surname: Agar fullname: Agar, John organization: Department of Renal Medicine, University Hospital, Victoria, Geelong, Australia – sequence: 5 givenname: Andrew surname: Mallett fullname: Mallett, Andrew organization: Department of Renal Medicine, Royal Brisbane and Women's Hospital, Queensland, Herston, Australia |
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Cites_doi | 10.1016/0272-6386(95)90555-3 10.1053/ajkd.2002.34933 10.1053/ajkd.2002.34548 10.1046/j.1523-1755.2003.00853.x 10.2215/CJN.08300910 10.1016/S0272-6386(98)70066-6 10.1681/ASN.2007070757 10.1681/ASN.V9122244 10.1053/j.ajkd.2013.03.031 10.1093/ndt/gfu189 10.1053/j.ajkd.2008.07.011 |
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References_xml | – volume: 52 start-page: 1158 year: 2008 end-page: 1162 article-title: Rituximab treatment of fibrillary glomerulonephritis publication-title: Am. J. Kidney Dis. – volume: 31 start-page: e4.1 year: 1998 end-page: 45 article-title: Fibrillary glomerulonephritis in siblings publication-title: Am. J. Kidney Dis. – volume: 55 start-page: 159 year: 2001 end-page: 166 article-title: Outcome of renal transplantation in fibrillary glomerulonpehritis publication-title: Clin. Nephrol. – volume: 40 start-page: e9.1 year: 2002 end-page: 95 article-title: Fibrillary glomerulonephritis: Early diagnosis associated with steroid responsiveness publication-title: Am. J. Kidney Dis. – volume: 25 start-page: 781 year: 1995 end-page: 791 article-title: Familial glomerulonephritis characterized by massive deposits of fibronectin publication-title: Am. J. Kidney Dis. – volume: 19 start-page: 34 year: 2008 end-page: 37 article-title: Fibrillary glomerulonephritis and immunotactoid glomerulopathy publication-title: J. Am. Soc. Nephrol. – volume: 62 start-page: 679 year: 2013 end-page: 690 article-title: Long‐term kidney disease outcomes in fibrillary glomerulonephritis: A case series of 27 patients publication-title: Am. J. Kidney Dis. – volume: 40 start-page: 420 year: 2002 end-page: 425 article-title: Fibrillary glomerulonephritis associated with crescents as a therapeutic challenge publication-title: Am. J. Kidney Dis. – volume: 63 start-page: 1450 year: 2003 end-page: 1461 article-title: Fibrillary and immunotactoid glomerulonephritis: Distinct entities with different clinical and pathologic features publication-title: Kidney Int. – volume: 29 start-page: 1925 year: 2014 end-page: 1931 article-title: Rituximab treatment for fibrillary glomerulonephritis publication-title: Nephrol. Dial. Transplant. – volume: 6 start-page: 775 year: 2011 end-page: 784 article-title: Fibrillary glomerulonephritis: A report of 66 cases from a single institution publication-title: Clin. J. Am. Soc. Nephrol. – volume: 9 start-page: 2244 year: 1998 end-page: 2252 article-title: Hepatitis C viral infection is associated with fibrillary glomerulonephritis and immunotactoid glomerulopathy publication-title: J. Am. Soc. Nephrol. – volume: 55 start-page: 159 year: 2001 ident: e_1_2_6_8_1 article-title: Outcome of renal transplantation in fibrillary glomerulonpehritis publication-title: Clin. Nephrol. contributor: fullname: Samaniego M – ident: e_1_2_6_7_1 doi: 10.1016/0272-6386(95)90555-3 – ident: e_1_2_6_9_1 doi: 10.1053/ajkd.2002.34933 – ident: e_1_2_6_10_1 doi: 10.1053/ajkd.2002.34548 – ident: e_1_2_6_2_1 doi: 10.1046/j.1523-1755.2003.00853.x – ident: e_1_2_6_4_1 doi: 10.2215/CJN.08300910 – ident: e_1_2_6_13_1 doi: 10.1016/S0272-6386(98)70066-6 – ident: e_1_2_6_3_1 doi: 10.1681/ASN.2007070757 – volume: 9 start-page: 2244 year: 1998 ident: e_1_2_6_5_1 article-title: Hepatitis C viral infection is associated with fibrillary glomerulonephritis and immunotactoid glomerulopathy publication-title: J. Am. Soc. Nephrol. doi: 10.1681/ASN.V9122244 contributor: fullname: Markowitz G – ident: e_1_2_6_6_1 doi: 10.1053/j.ajkd.2013.03.031 – ident: e_1_2_6_12_1 doi: 10.1093/ndt/gfu189 – ident: e_1_2_6_11_1 doi: 10.1053/j.ajkd.2008.07.011 |
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Snippet | Fibrillary glomerulonephritis is a rare cause of glomerulonephritis characterized by non‐amyloid fibrillary deposits of unknown aetiology. It is generally... Fibrillary glomerulonephritis is a rare cause of glomerulonephritis characterized by non-amyloid fibrillary deposits of unknown aetiology. It is generally... Abstract Fibrillary glomerulonephritis is a rare cause of glomerulonephritis characterized by non‐amyloid fibrillary deposits of unknown aetiology. It is... |
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SubjectTerms | Adult chronic kidney disease familial nephritis Female glomerulonephritis Glomerulonephritis - genetics Glomerulonephritis - pathology hereditary nephritis Humans Male Middle Aged nephrotic syndrome Pedigree |
Title | Fibrillary glomerulonephritis: An apparent familial form? |
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