Atypical anti-glomerular basement membrane disease with anti-GBM antibody negativity and ANCA positivity: a case report

Guo, Na; Yin, Qudong; Song, Lei; He, Yanjun; Fu, Ping

doi:10.1186/s12882-021-02232-1

Cited by 10 publications

(8 citation statements)

References 11 publications

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“…Despite revealing typical immunoglobulin deposition along the GBM lineage in kidney immunofluorescence, the serum tests negative for anti-GBM antibodies. Furthermore, the clinical manifestations, serologic and pathologic features, and prognosis of atypical anti-GBM disease differ from those of the typical variant, suggesting the possibility of distinct underlying pathogenic mechanisms [ 5 , 6 , 11 ].…”

Section: Discussionmentioning

confidence: 99%

“… linear deposition Negative NA 79/M [ 28 ] NA IgG (+); IgA (+); C3 (+); and lambda (+); fibrinogen (+). linear deposition Negative NA 48/F [ 11 ] Some globally sclerotic glomeruli, cellular and fibric crescents, Bowman’s capsules and segmental sclerotic glomeruli, tubular atrophy accompanied by interstitial fibrosis, interstitial infiltration of lymphocytes, monocytes, and plasmocytes IgG (+) linear deposition Negative No electron-dense deposits and presence of foot process fusion. 37/M [ 29 ] diffuse proliferation in the glomeruli, with increased mesangial proliferation; crescents IgG (+);C3 (+).…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

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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“…Anti-GBM disease is an organ-specific autoimmune disease that involves the lung and kidneys and leads to rapid glomerulonephritis progression, with or without diffuse alveolar hemorrhage, and even respiratory failure. Classic cases of anti-GBM disease are diagnosed based on the presence of the anti-GBM antibody in serum samples and kidney or lung biopsy tissue samples, [ 1 ] the estimated incidence of anti-GBM disease is <2 cases per million population per annum, it is even lower in Asian populations. [ 2 , 3 ] In this case, the patient was admitted with clinical manifestations of rapidly progressive glomerulonephritis.…”

Section: Discussion/conclusionmentioning

confidence: 99%

“…Anti-glomerular basement membrane (anti-GBM) disease classically presents with aggressive necrotizing and crescentic glomerulonephritis, often with pulmonary hemorrhage. Classic cases are diagnosed based on the presence of the anti-GBM antibody in serum samples and kidney or lung biopsy tissue samples, [1] the estimated incidence of anti-GBM disease is <2 cases per million population per annum, it is even lower in Asian populations [2,3] . The primary therapeutic aim is to render the anti-GBM antibody titer negative as quickly as possible combining plasmapheresis and immunosuppression [4] .…”

Section: Introductionmentioning

confidence: 99%

Pneumocystis pneumonia secondary to intensive immunosuppression treatment for anti-GBM disease complicated with IgA nephropathy

et al. 2021

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Rationale:The estimated incidence of anti-glomerular basement membrane (anti-GBM) disease complicated with immunoglobulin A (IgA) nephropathy is minimal, there have only been 15 cases (including this case) reported in the literature, and only 5 (33.33%) of them showed significant improvement in renal function after treatment. Pneumocystis pneumonia is a severe opportunistic pulmonary infection of pneumocystis jiroveci in immunocompromised patients. Here, we report a case of pneumocystis pneumonia secondary to intensive immunosuppression treatment for anti-GBM disease complicated with IgA nephropathy, with no similar reports or studies published before to our knowledge.Patient concerns:The patient was admitted to our hospital with a 1-week diagnosis of crescent glomerulonephritis who had been suffered from hematuria and foamy urine for more than 1 month. Before admission, the patient received pulse dose intravenous methylprednisolone and immunosuppression with rituximab, but the renal function and titer of pathogenic antibody did not improve significantly.Diagnosis:Crescentic glomerulonephritis, anti-glomerular basal membrane disease complicated with IgA nephropathy (Type I+II) was pathologically confirmed by renal biopsy. Secondary pneumocystis pneumonia was diagnosed by acute progressive respiratory failure, chest computed tomography and metagenomic next-generation sequencing of transbronchoscopic bronchoalveolar lavage fluid.Interventions:The key to successful treatment was to make the pathogenic antibody turn negative quickly by combining pulse dose intravenous methylprednisolone, immunosuppression with rituximab, and plasma exchange therapy. Early identification of pneumocystis pneumonia, accurate etiological identification, and active anti-infective treatment were also crucial.Outcomes:The patient was discharged after 16 days of anti-infection with secondary infection controlled and dialysis catheter removed. Up to now, the patient has been followed for a period of 28 weeks, results showed renal function had been repaired even hematuria and proteinuria were basically alleviated.Lessons:Our case provided experience in the treatment of anti-GBM disease complicated with IgA nephropathy, further proposed the potential therapeutic effects of rituximab, also illustrated low dose hormone combined with tacrolimus can be used as sequential therapy after plasma exchange and intensive immunosuppression. Our research also suggested that resulting in severe immune suppression, a high risk of secondary pneumocystis opportunistic infection should be aware of. metagenomic next-generation sequencing might increase the detection rate of the pathogen.

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“… 11 1 58 5.8 AKI after immune checkpoint inhibitor (nivolumab) Focal crescentic and proliferative IgG dominant (subclass NA) Negative Steroids/CYC ESKD Guo et al. 21 1 38 4.35 RPGN Diffuse crescentic, IFTA: 30% IgG dominant Negative Steroids/PLEX Improved serum creatinine Tamura et al. 22 1 43 1.44 Episodic macrohematuria Focal crescents, endocapillary hypercellularity IgG1 dominant Negative by ELISA, positive by IIF Steroids/CYC/PLEX Normalized serum creatinine Javaugue et al.…”

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confidence: 99%