Anti-brush border antibody (ABBA)-associated renal disease

Campbell, Ruth E; Uhlenhopp, Dustin; Shaw, Michael; Dai, Dao-Fu; Sekar, Arjun

doi:10.1093/qjmed/hcaa015

Cited by 6 publications

(13 citation statements)

References 4 publications

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“…Any morphologically atypical appearing lymphoid populations in the biopsy should be further evaluated immunohistochemically for the presence of neoplasia. Anti-LRP2 nephropathy and primary kidney lym-phoma are both exceedingly rare, and our report may help to further shed light on this unusual association [1][2][3][4][5][6][7][8][9][10]16].…”

Section: Discussionmentioning

confidence: 98%

Anti-LDL Receptor-Related Protein 2 Nephropathy with Synchronous Primary Kidney Extranodal Marginal Zone Lymphoma

Ng¹,

Ruíz-Cordero²,

Caza³

et al. 2021

Glomerular Dis

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Introduction: Anti-LDL receptor-related protein 2 (anti-LRP2) nephropathy is a rare but progressive form of autoimmune-mediated tubulointerstitial nephritis and glomerular disease, characterized by a classic pattern of immune complex deposition in the kidney. A theoretic link between autoimmune disease and lymphoproliferative diseases exists, and therefore, in some cases autoimmune-mediated inflammation and lymphoproliferative neoplasm can co-exist in the same site. Case Presentation: An elderly man presented with 6 months of weight loss and fatigue. Subsequent workup showed an elevated serum creatinine and subnephrotic range proteinuria. Kidney biopsy was performed which revealed anti-LRP2 nephropathy with concurrent primary kidney extranodal marginal zone lymphoma. He was subsequently treated with rituximab but remains dialysis-dependent (12 months after his initial diagnosis, at time of publication of this report). Conclusion: We discuss the bidirectional relationship between autoimmune disease and lymphoma in the kidney, along with a brief review of the literature pertaining to these rare lesions. Our case report highlights the diagnostic difficulties faced by pathologists when encountering this entity. We also attempt to spread awareness about the co-existence of tubulointerstitial inflammation and lymphoproliferative disorder, which may be under-recognized.

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Section: Discussionmentioning

confidence: 98%

Anti-LDL Receptor-Related Protein 2 Nephropathy with Synchronous Primary Kidney Extranodal Marginal Zone Lymphoma

Ng¹,

Ruíz-Cordero²,

Caza³

et al. 2021

Glomerular Dis

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“…1 In the same series, 3 patients showed serologic positivity for antinuclear antibody, but none met diagnostic criteria for systemic lupus erythematosus. 1 All other known case reports of ABBAD are in patients 59 years of age or older, [6][7][8][9] and none carried an association with systemic lupus erythematosus or lupus nephritis. Therefore, our patient is a highly unusual case of ABBAD in a relatively young individual associated with another concurrent autoimmune nephritis.…”

Section: Discussionmentioning

confidence: 99%

“…5 Renewed interest in this disease arose only recently with a case report of an immune-complex tubulointerstitial nephritis with antibodies to the proximal tubular brush border in 2016 8 and discovery of LRP2 as the target antigen in 2017, confirmed in a small series of 10 patients. 1 Since then, only 2 case reports of ABBAD have emerged, 6,7 and no large case series have been published.…”

Section: Discussionmentioning

confidence: 99%

Anti–Brush Border Antibody Disease (Anti-LRP2 Nephropathy) Associated With Lupus Nephritis

Dvanajscak

Murphy

Larsen

et al. 2020

Kidney International Reports

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“…There are few reports of anti-LRP2 nephropathy with confirmed serum ABBA (anti-LRP2 antibody) [1][2][3][4][5][6][7][8]. In 2016, a case report described a patient with tubulointersitial nephritis with immune complexes in the TBM and occasional epimembranous deposits.…”

Section: Discussionmentioning

confidence: 99%

“…Anti-brush border antibody (ABBA) disease is a disorder characterized by acute tubulointerstitial injury associated with circulating antibodies reactive to kidney proximal tubular brush border. ABBA was first described in 1981 and a total of 30 cases have been reported in the literature [1][2][3][4][5][6][7][8]. Circulating antibodies reactive to normal human kidney proximal tubular low-density lipoprotein (LDL) receptor-related protein 2 (LRP2), also known as megalin, have been identified as being responsible for the disorder [4].…”

Section: Introductionmentioning

confidence: 99%

Complete remission of nephrotic syndrome in a young woman with anti-LRP2 nephropathy after immunosuppressive therapy

Zhu

Liu

et al. 2020

BMC Nephrol

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Background: Anti-low density lipoprotein receptor-related protein 2 (LRP2) nephropathy/anti-brush border antibody (ABBA) disease is a disorder characterized by acute tubulointerstitial injury associated with circulating antibodies to kidney proximal tubular brush border protein LRP2/megalin. Patients are typically elderly and present with acute kidney injury and subnephrotic proteinuria. They progress to end-stage renal disease with poor response to immunosuppressive therapies. Case presentation: We report a case of a 29-year-old Chinese woman, who presented with nephrotic syndrome with normal kidney function. Kidney biopsy showed no obvious tubular injury or interstitial inflammation. Positive immunoglobulin G (IgG) staining was revealed along the brush border of proximal tubular cells. Anti-LRP2 antibody was identified in serum, consistent with a diagnosis of anti-LRP2 nephropathy. The patient achieved complete remission after receiving prednisone and cyclophosphamide. Conclusions: Anti-LRP2 nephropathy can also present as nephrotic syndrome in young patients and complete remission from nephrotic syndrome may be achieved after immunosuppressive therapy.

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Anti-brush border antibody (ABBA)-associated renal disease

Cited by 6 publications

References 4 publications

Anti-LDL Receptor-Related Protein 2 Nephropathy with Synchronous Primary Kidney Extranodal Marginal Zone Lymphoma

Anti-LDL Receptor-Related Protein 2 Nephropathy with Synchronous Primary Kidney Extranodal Marginal Zone Lymphoma

Anti–Brush Border Antibody Disease (Anti-LRP2 Nephropathy) Associated With Lupus Nephritis

Complete remission of nephrotic syndrome in a young woman with anti-LRP2 nephropathy after immunosuppressive therapy

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