Monoclonal immunoglobulin G1 κ-type atypical antiglomerular basement membrane disease accompanied by necrotizing glomerulonephritis

Tsuji, Takayuki; Ohashi, Naro; Sato, Taichi; Goto, Daiki; Nagata, Soichiro; Matsuyama, Takashi; Naito, Yasuhisa; Tsuji, Naoko; Isobe, Shinsuke; Fujikura, Tomoyuki; Kato, Akihiko; Fujigaki, Yoshihide; Shimizu, Akira; Yasuda, Hideo

doi:10.5414/cn109889

Cited by 4 publications

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“… linear deposition Negative No electron-dense deposits. 64/M [ 30 ] Necrotizing glomerulonephritis, cellular crescent, and diffuse segmental endocapillary proliferation. IgG1-κ (+).…”

Section: Discussionmentioning

confidence: 99%

Atypical anti-glomerular basement membrane disease with membranous hyperplasia: diagnostic challenges and treatment variability

Tong,

Luo,

Zhong

et al. 2024

BMC Nephrol

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This case report presents a detailed analysis of a 31-year-old male patient who presented with a complex array of clinical symptoms, including proteinuria, hematuria, edema, and kidney insufficiency. Despite undergoing multiple tests, the results for anti-glomerular basement membrane antibodies yielded negative findings. Subsequently, kidney biopsy pathology revealed a distinct diagnosis of atypical anti-glomerular basement membrane (anti-GBM) disease with membrane hyperplasia. Treatment was initiated with a comprehensive approach involving high doses of corticosteroids therapy and cyclophosphamide (CTX). However, contrary to expectations, the patient’s kidney function exhibited rapid deterioration following this therapeutic regimen. The culmination of these complications necessitated a pivotal transition to maintenance hemodialysis. This case underscores the intricate challenges associated with diagnosing and managing rare and atypical presentations of kidney disorders. The negative anti-GBM antibody results and subsequent identification of atypical anti-GBM nephropathy highlight the need for tailored diagnostic strategies to discern subtle nuances within complex clinical scenarios. Additionally, the unexpected response to the treatment regimen emphasizes the potential variability in individual patient responses, underlining the necessity for vigilant monitoring and adaptable treatment strategies. This case report contributes to the evolving understanding of atypical kidney pathologies and the complexities involved in their management.

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“… linear deposition Negative No electron-dense deposits. 64/M [ 30 ] Necrotizing glomerulonephritis, cellular crescent, and diffuse segmental endocapillary proliferation. IgG1-κ (+).…”

Section: Discussionmentioning

confidence: 99%

Atypical anti-glomerular basement membrane disease with membranous hyperplasia: diagnostic challenges and treatment variability

Tong,

Luo,

Zhong

et al. 2024

BMC Nephrol

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“…Atypical anti-GBM progresses slowly, symptoms of pulmonary involvement such as hemoptysis are rare [42] , and most of them have a better prognosis than patients with classical anti-GBM, but the prognosis is poor when atypical anti-GBM is combined with crescent bodies. There is no standard treatment regimen, and usually the vast majority of patients are treated conservatively (e.g., angiotensin-converting enzyme inhibitors/angiotensin receptor blockers) and/or immune suppressively [43][44][45][46][47][48][49] , with the addition of plasma exchange therapy in a few patients when other types of antibodies are detected [50] , and in a few patients with the addition of rituximab in the event of a poor conventional outcome or in the presence of a rapid deterioration in renal function [51][52] .…”

Section: Atypical Anti-gbmmentioning

confidence: 99%

Clinicopathological characterization of subgroups in anti-glomerular basement membrane nephritis patients From a Single Center in China: A 12-Year observational study

Fang,

Wang,

Ding

et al. 2024

Preprint

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Background Despite the previous reports of patients with combined anti-glomerular basement membrane (anti-GBM) disease occurring sequentially or simultaneously with other nephropathies, most of them have been reported seperately. The complication of these diseases is rare and the mechanism is not clear, and their immuno-antibodies, microscopic picture, clinical presentation, treatment and prognosis are different, therefore, we presented the collection of anti-GBM with combined disease such as membranous nephropathy (MN), anti-neutrophil cytoplasmic antibody (ANCA), IgA nephropathy and atypical anti-GBM to systematically characterized the epidemiological features, clinical manifestations, pathological features and herapeutic outcomes through a summative review. Method We retrospectively a case series of 39 anti-GBM diseases from a single center in Northwest China from 2011–2023. Results A total of 39 patients with anti-GBM disease including 19 males and 20 females were collected with a mean age of 50.0 ± 14.6 years. Among them there were 22 cases (22/39,56.4%) of anti-GBM alone, 6 cases (6/39,15.4%) of combined ANCA, 6 cases (6/39,15.4%) of combined MN, 2 cases (2/39,5.1%) of combined IgAN, and 3 cases (3/39,7.7%) of atypical anti-GBM nephropathy. The mean duration of the disease was 2.6 ± 6.2 months. Clinical symptoms were dominated by fever 68.2% (15/22), oliguria/anuria 63.6% (14/22), and microscopic haematuria 88.9% (16/22,) in the anti-GBM alone group, and nausea and vomiting 50% (3/6) in the anti-GBM + MN group, and edema 83.3% (5/6) in the anti-GBM + MN group. The proportion of patients requiring hemodialysis (HD) at the first visit was 79.5% (31/39) in all patients, 56.4% (22/39) in the anti-GBM alone group, 66.7% (4/6) in the anti-GBM + ANCA group, 66.7% (4/6) in the anti-GBM + MN group, and 100% (2/2) in the anti-GBM + IgAN group. Among them, 56.4% had more than 85% glomerular involvement. The proportion of sclerotic glomeruli was higher in the anti-GBM and anti-GBM + ANCA groups. The highest percentage of cellular crescents was found in the anti-GBM group as long with fibrous crescents in the anti-GBM + ANCA group. Immunofluorescence staining demonstrated positive IgG and C3 staining in all subgroups. Conclusions We concluded that the complication of other nephritis is another potential risk factor for anti-GBM, which is directly attributable to the adverse effects of the different immune depositions and pathological features on renal function, as timely intervention in patients with different pathological features is crucial.

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Non-typical anti-GBM disease with intraglomerular granulomatous reaction and anti-PLA2R-negative membranous nephropathy in the context of IgM/κ paraproteinemia

Zhao

Yang

et al. 2022

Int Urol Nephrol

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Monoclonal immunoglobulin G1 κ-type atypical antiglomerular basement membrane disease accompanied by necrotizing glomerulonephritis

Cited by 4 publications

References 0 publications

Atypical anti-glomerular basement membrane disease with membranous hyperplasia: diagnostic challenges and treatment variability

Atypical anti-glomerular basement membrane disease with membranous hyperplasia: diagnostic challenges and treatment variability

Clinicopathological characterization of subgroups in anti-glomerular basement membrane nephritis patients From a Single Center in China: A 12-Year observational study

Non-typical anti-GBM disease with intraglomerular granulomatous reaction and anti-PLA2R-negative membranous nephropathy in the context of IgM/κ paraproteinemia

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