{"title":"原纤维性肾小球肾炎合并多发性骨髓瘤1例报告及文献复习。","authors":"Taiki Ishida, Ken Morita, Yosuke Masamoto, Hideaki Mizuno, Kazuki Taoka, Hiroyuki Abe, Motoki Odawara, Yosuke Hirakawa, Masaomi Nangaku, Mineo Kurokawa","doi":"10.1159/000545498","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Fibrillary glomerulonephritis (FGN) is a rare form of immune complex-mediated primary glomerular disease frequently coexisting with malignancies or autoimmune diseases. The kidney prognosis is extremely poor, with approximately 50% of patients progressing to end-stage kidney disease within 2-4 years after diagnosis. However, no established treatment currently exists.</p><p><strong>Case presentation: </strong>Here we describe a rare case of FGN diagnosed in a patient progressing from monoclonal gammopathy to multiple myeloma. The histopathological findings of the kidney biopsy were consistent with classical FGN and revealed no evidence of myeloma cast nephropathy. Albumin-dominant, Bence Jones protein-negative proteinuria further supported this diagnosis. The patient was successfully treated with anti-myeloma chemotherapies including autologous stem cell transplant, resulting in significant improvement in kidney function.</p><p><strong>Conclusion: </strong>Based on our experience, secondary FGN associated with plasma cell neoplasms may represent a rare entity that responds favorably to anti-myeloma therapies. Initial investigations to rule out coexistent plasma cell neoplasms are crucial for the optimal management of FGN patients.</p>","PeriodicalId":9625,"journal":{"name":"Case Reports in Oncology","volume":"18 1","pages":"554-562"},"PeriodicalIF":0.7000,"publicationDate":"2025-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12058109/pdf/","citationCount":"0","resultStr":"{\"title\":\"Fibrillary Glomerulonephritis and Multiple Myeloma: A Case Report and Literature Review.\",\"authors\":\"Taiki Ishida, Ken Morita, Yosuke Masamoto, Hideaki Mizuno, Kazuki Taoka, Hiroyuki Abe, Motoki Odawara, Yosuke Hirakawa, Masaomi Nangaku, Mineo Kurokawa\",\"doi\":\"10.1159/000545498\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>Fibrillary glomerulonephritis (FGN) is a rare form of immune complex-mediated primary glomerular disease frequently coexisting with malignancies or autoimmune diseases. The kidney prognosis is extremely poor, with approximately 50% of patients progressing to end-stage kidney disease within 2-4 years after diagnosis. However, no established treatment currently exists.</p><p><strong>Case presentation: </strong>Here we describe a rare case of FGN diagnosed in a patient progressing from monoclonal gammopathy to multiple myeloma. The histopathological findings of the kidney biopsy were consistent with classical FGN and revealed no evidence of myeloma cast nephropathy. Albumin-dominant, Bence Jones protein-negative proteinuria further supported this diagnosis. The patient was successfully treated with anti-myeloma chemotherapies including autologous stem cell transplant, resulting in significant improvement in kidney function.</p><p><strong>Conclusion: </strong>Based on our experience, secondary FGN associated with plasma cell neoplasms may represent a rare entity that responds favorably to anti-myeloma therapies. Initial investigations to rule out coexistent plasma cell neoplasms are crucial for the optimal management of FGN patients.</p>\",\"PeriodicalId\":9625,\"journal\":{\"name\":\"Case Reports in Oncology\",\"volume\":\"18 1\",\"pages\":\"554-562\"},\"PeriodicalIF\":0.7000,\"publicationDate\":\"2025-04-04\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12058109/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Case Reports in Oncology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1159/000545498\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q4\",\"JCRName\":\"ONCOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Case Reports in Oncology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1159/000545498","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/1/1 0:00:00","PubModel":"eCollection","JCR":"Q4","JCRName":"ONCOLOGY","Score":null,"Total":0}
Fibrillary Glomerulonephritis and Multiple Myeloma: A Case Report and Literature Review.
Introduction: Fibrillary glomerulonephritis (FGN) is a rare form of immune complex-mediated primary glomerular disease frequently coexisting with malignancies or autoimmune diseases. The kidney prognosis is extremely poor, with approximately 50% of patients progressing to end-stage kidney disease within 2-4 years after diagnosis. However, no established treatment currently exists.
Case presentation: Here we describe a rare case of FGN diagnosed in a patient progressing from monoclonal gammopathy to multiple myeloma. The histopathological findings of the kidney biopsy were consistent with classical FGN and revealed no evidence of myeloma cast nephropathy. Albumin-dominant, Bence Jones protein-negative proteinuria further supported this diagnosis. The patient was successfully treated with anti-myeloma chemotherapies including autologous stem cell transplant, resulting in significant improvement in kidney function.
Conclusion: Based on our experience, secondary FGN associated with plasma cell neoplasms may represent a rare entity that responds favorably to anti-myeloma therapies. Initial investigations to rule out coexistent plasma cell neoplasms are crucial for the optimal management of FGN patients.