Clinicopathologic Features of Antibrush Border Antibody Disease
Introduction: Antibrush border antibody disease (ABBA) is an autoimmune tubulointerstitial kidney disease that primarily affects older individuals and results in progressive kidney failure. It is rare with only 20 reported cases. Here, we describe a case series to further define the clinicopathologi...
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| Language: | English |
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Elsevier
2024-02-01
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| Series: | Kidney International Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2468024923015966 |
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| author | Joel D. Murphy Tiffany N. Caza Clarissa A. Cassol Aaron Storey Josephine M. Ambruzs Christie Boils Patrick D. Walker Shree Sharma Nidia Messias Randolph Hennigar Nicole K. Andeen Christine VanBeek Matthew Palmer Lakshna Sankar Pooja Sanghi Kumar Dinesh Lance Dicker Anatoly Urisman Christopher P. Larsen |
| author_facet | Joel D. Murphy Tiffany N. Caza Clarissa A. Cassol Aaron Storey Josephine M. Ambruzs Christie Boils Patrick D. Walker Shree Sharma Nidia Messias Randolph Hennigar Nicole K. Andeen Christine VanBeek Matthew Palmer Lakshna Sankar Pooja Sanghi Kumar Dinesh Lance Dicker Anatoly Urisman Christopher P. Larsen |
| author_sort | Joel D. Murphy |
| collection | DOAJ |
| description | Introduction: Antibrush border antibody disease (ABBA) is an autoimmune tubulointerstitial kidney disease that primarily affects older individuals and results in progressive kidney failure. It is rare with only 20 reported cases. Here, we describe a case series to further define the clinicopathologic spectrum and natural history, and to inform management. Methods: We identified 67 patients with ABBA who underwent kidney biopsy, including 65 native and 2 transplants. Demographics, clinical findings, and laboratory data were obtained. Histopathologic data included light microscopy, immunofluorescence, electron microscopy and immunostaining for LRP2, CUBN, and AMN. Follow-up data, including treatment(s), laboratory values, and outcomes, were available from 51 patients. Results: Patients with ABBA were predominantly male with a median age of 72 years. Median serum creatinine was 2.7 mg/dl, proteinuria was 2.8 g/day, and hematuria was present in two-thirds of the patients. Tubular injury with LRP2-positive tubular basement membrane (TBM) deposits were seen in 94.2% of patients. Thirty-eight patients (56.7%) had a second kidney disease, commonly glomerular diseases with high-grade proteinuria. These diseases included podocytopathies, membranous nephropathy (MN), IgA nephropathy, diabetic glomerulopathy, lupus nephritis (LN), crescentic glomerulonephritis (GN), tubulointerstitial nephritis, and involvement by lymphoma. The majority of patients were treated with immunosuppression. Of those patients with follow-up, 29.4% achieved remission, 70.6% had no response, and 52.8% required dialysis or were deceased. Untreated patients were at the highest risk. Conclusion: ABBA is a rare autoimmune kidney disease that often occurs with other kidney diseases. Although the overall prognosis of ABBA is poor, there is potential benefit from immunosuppression. |
| format | Article |
| id | doaj-art-c41d93ed6ad24307a0dcb967b20fda8e |
| institution | DOAJ |
| issn | 2468-0249 |
| language | English |
| publishDate | 2024-02-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Kidney International Reports |
| spelling | doaj-art-c41d93ed6ad24307a0dcb967b20fda8e2025-08-20T03:20:30ZengElsevierKidney International Reports2468-02492024-02-019237038210.1016/j.ekir.2023.11.008Clinicopathologic Features of Antibrush Border Antibody DiseaseJoel D. Murphy0Tiffany N. Caza1Clarissa A. Cassol2Aaron Storey3Josephine M. Ambruzs4Christie Boils5Patrick D. Walker6Shree Sharma7Nidia Messias8Randolph Hennigar9Nicole K. Andeen10Christine VanBeek11Matthew Palmer12Lakshna Sankar13Pooja Sanghi14Kumar Dinesh15Lance Dicker16Anatoly Urisman17Christopher P. Larsen18Arkana Laboratories, Little Rock, Arkansas, USAArkana Laboratories, Little Rock, Arkansas, USA; Correspondence: Tiffany Caza, Arkana Laboratories, 10810 Executive Center Drive #100, Little Rock, Arkansas 72211, USA.Arkana Laboratories, Little Rock, Arkansas, USAArkana Laboratories, Little Rock, Arkansas, USAArkana Laboratories, Little Rock, Arkansas, USAArkana Laboratories, Little Rock, Arkansas, USAArkana Laboratories, Little Rock, Arkansas, USAArkana Laboratories, Little Rock, Arkansas, USADepartment of Pathology, Washington University, St. Louis, Missouri, USADepartment of Pathology, State University of New York at Stony Brook, Stony Brook, New York, USADepartment of Pathology, Oregon Health and Science University, Portland, Oregon, USAAmeripath Laboratories, Oklahoma City, Oklahoma, USADepartment of Pathology, University of Pennsylvania, Philadelphia, Pennsylvania, USADivision of Nephrology, Geisinger Medical Center, Danville, Pennsylvania, USARenal Care Consultants, Medford, Oregon, USARenal Care Consultants, Medford, Oregon, USAKidney Care Physicians, Salem, Oregon, USADepartment of Pathology, University of California - San Francisco, San Francisco, California, USAArkana Laboratories, Little Rock, Arkansas, USAIntroduction: Antibrush border antibody disease (ABBA) is an autoimmune tubulointerstitial kidney disease that primarily affects older individuals and results in progressive kidney failure. It is rare with only 20 reported cases. Here, we describe a case series to further define the clinicopathologic spectrum and natural history, and to inform management. Methods: We identified 67 patients with ABBA who underwent kidney biopsy, including 65 native and 2 transplants. Demographics, clinical findings, and laboratory data were obtained. Histopathologic data included light microscopy, immunofluorescence, electron microscopy and immunostaining for LRP2, CUBN, and AMN. Follow-up data, including treatment(s), laboratory values, and outcomes, were available from 51 patients. Results: Patients with ABBA were predominantly male with a median age of 72 years. Median serum creatinine was 2.7 mg/dl, proteinuria was 2.8 g/day, and hematuria was present in two-thirds of the patients. Tubular injury with LRP2-positive tubular basement membrane (TBM) deposits were seen in 94.2% of patients. Thirty-eight patients (56.7%) had a second kidney disease, commonly glomerular diseases with high-grade proteinuria. These diseases included podocytopathies, membranous nephropathy (MN), IgA nephropathy, diabetic glomerulopathy, lupus nephritis (LN), crescentic glomerulonephritis (GN), tubulointerstitial nephritis, and involvement by lymphoma. The majority of patients were treated with immunosuppression. Of those patients with follow-up, 29.4% achieved remission, 70.6% had no response, and 52.8% required dialysis or were deceased. Untreated patients were at the highest risk. Conclusion: ABBA is a rare autoimmune kidney disease that often occurs with other kidney diseases. Although the overall prognosis of ABBA is poor, there is potential benefit from immunosuppression.http://www.sciencedirect.com/science/article/pii/S2468024923015966ABBAAMNantibrush border antibody diseaseCUBNLRP2kidney biopsy |
| spellingShingle | Joel D. Murphy Tiffany N. Caza Clarissa A. Cassol Aaron Storey Josephine M. Ambruzs Christie Boils Patrick D. Walker Shree Sharma Nidia Messias Randolph Hennigar Nicole K. Andeen Christine VanBeek Matthew Palmer Lakshna Sankar Pooja Sanghi Kumar Dinesh Lance Dicker Anatoly Urisman Christopher P. Larsen Clinicopathologic Features of Antibrush Border Antibody Disease Kidney International Reports ABBA AMN antibrush border antibody disease CUBN LRP2 kidney biopsy |
| title | Clinicopathologic Features of Antibrush Border Antibody Disease |
| title_full | Clinicopathologic Features of Antibrush Border Antibody Disease |
| title_fullStr | Clinicopathologic Features of Antibrush Border Antibody Disease |
| title_full_unstemmed | Clinicopathologic Features of Antibrush Border Antibody Disease |
| title_short | Clinicopathologic Features of Antibrush Border Antibody Disease |
| title_sort | clinicopathologic features of antibrush border antibody disease |
| topic | ABBA AMN antibrush border antibody disease CUBN LRP2 kidney biopsy |
| url | http://www.sciencedirect.com/science/article/pii/S2468024923015966 |
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