{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T23:35:38Z","timestamp":1792193738867,"version":"4.4.0"},"reference-count":12,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2015,6,9]],"date-time":"2015-06-09T00:00:00Z","timestamp":1433808000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Nephrology"],"published-print":{"date-parts":[[2015,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Fibrillary glomerulonephritis is a rare cause of glomerulonephritis characterized by non\u2010amyloid fibrillary deposits of unknown aetiology. It is generally considered idiopathic but may be associated with secondary causes such as monoclonal gammopathy, hepatitis <jats:styled-content style=\"fixed-case\">B<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content> infections, autoimmune diseases and malignancies. We report two <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>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\u2010stage 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\u2010up after 5 years. A further review of the second family history reveals a third family member (maternal father) dying of \u2018Bright's disease\u2019. 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.<\/jats:p>","DOI":"10.1111\/nep.12447","type":"journal-article","created":{"date-parts":[[2015,6,11]],"date-time":"2015-06-11T00:22:14Z","timestamp":1433982134000},"page":"506-509","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Fibrillary glomerulonephritis: An apparent familial form?"],"prefix":"10.1111","volume":"20","author":[{"given":"Tracey","family":"Ying","sequence":"first","affiliation":[{"name":"Department of Renal Medicine University Hospital  Geelong Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Prue","family":"Hill","sequence":"additional","affiliation":[{"name":"Department of Anatomical Pathology St Vincent's Hospital  Fitzroy Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Desmond","sequence":"additional","affiliation":[{"name":"Department of Renal Medicine University Hospital  Geelong Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Agar","sequence":"additional","affiliation":[{"name":"Department of Renal Medicine University Hospital  Geelong Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8752-2551","authenticated-orcid":false,"given":"Andrew","family":"Mallett","sequence":"additional","affiliation":[{"name":"Department of Renal Medicine Royal Brisbane and Women's Hospital  Herston Queensland Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,6,9]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1523-1755.2003.00853.x"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2007070757"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.08300910"},{"key":"e_1_2_6_5_1","doi-asserted-by":"crossref","first-page":"2244","DOI":"10.1681\/ASN.V9122244","article-title":"Hepatitis C viral infection is associated with fibrillary glomerulonephritis and immunotactoid glomerulopathy","volume":"9","author":"Markowitz G","year":"1998","journal-title":"J. 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