Anti-glomerular basement membrane disease: A clinicomorphological study of 16 cases

Vavilapalli, Srinivasrao; Madireddy, Nishika; Uppin, Megha S; Kalidindi, Karthik; Swarnalatha, G; Taduri, Gangadhar; Raju, Sree Bhushan

doi:10.4103/ijpm.ijpm_712_18

Cited by 3 publications

(1 citation statement)

References 13 publications

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“…The presence of circulating anti-GBM antibodies and deposition of antibodies on the GBM, which is usually revealed by immunofluorescence (IF) staining of the renal biopsy specimen, are diagnostic of the condition. [ 2 3 ] Global incidence varies from 0.5–1 case per million per year. Here, we present a case of a 68-year-old female who was initially diagnosed as decompensated chronic liver disease (DCLD) and later developed gross hematuria and alveolar hemorrhage which turned out to be Goodpasture syndrome.…”

Section: Introductionmentioning

confidence: 99%

An unusual presentation of Goodpasture syndrome

Kurhekar

George

Rajarathinam

et al. 2023

Saudi Journal of Anaesthesia

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Goodpasture disease is an anti–glomerular basement membrane (anti-GBM) disease that manifests as progressive glomerulopathy and alveolar hemorrhage. Our case was a 68-year-old female who presented with decompensated liver disease (DCLD) with no prior history suggestive of liver disease. She had dyspnea, bilateral pitting edema, icterus with normal renal parameters, and elevated liver enzymes. Ultrasonogram revealed shrunken liver, ascites, and portal hypertension with normal renal cortex echogenicity. Over the next three days, she developed progressive oliguria, hematuria, and breathlessness, with arterial blood gas showing hypoxia with acidosis and hyperkalemia. Bronchoscopic alveolar lavage was suggestive of hemorrhagic fluid. The patient succumbed to the disease in 24 hours. Anti-GBM antibodies came positive by immunofluorescence assay which confirmed the diagnosis of Goodpasture syndrome. Unusual presentation of DCLD in our case resulted in a delay in diagnosis that could have been crucial in altering the outcome.

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Section: Introductionmentioning

confidence: 99%

An unusual presentation of Goodpasture syndrome

Kurhekar

George

Rajarathinam

et al. 2023

Saudi Journal of Anaesthesia

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Accuracy of Anti-GBM Antibodies in Diagnosing Anti-Glomerular Basement Membrane Disease: A Systematic Review and Meta-Analysis

et al. 2021

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Background: The sensitivity and specificity of anti-glomerular basement membrane (GBM) antibodies have not been systematically analyzed. In this systematic review, we aimed to evaluate the diagnostic accuracy of anti-GBM antibodies for anti-GBM disease. Summary: Potential studies were searched using MEDLINE, Embase, the Cochrane Library, and the International Clinical Trials Registry Platform based on the index test and target condition. The inclusion criteria were prospective or retrospective cohort studies or case-control studies assessing the sensitivity and specificity of anti-GBM antibodies, and the reference standard was clinical diagnosis including biopsy results. The exclusion criteria were review articles, case reports, animal studies, and in vitro studies. Quality assessment was conducted based on the Quality Assessment of Diagnostic Accuracy Studies-2. The pooled estimates of sensitivity and specificity were calculated using a bivariate random-effects model. The overall quality was evaluated using the Grades of Recommendation, Assessment, Development, and Evaluation. Six studies (1,691 patients) and 11 index tests were included in our systematic review. A high risk of bias and concerns regarding the applicability of patient selection were noted because of the case-control design in 67% of the included studies. The pooled sensitivity and specificity were 93% (95% CI: 84–97%) and 97% (95% CI: 94–99%), respectively. The certainty of evidence was low because of the high risk of bias and indirectness. Key Messages: Anti-GBM antibodies may exhibit high sensitivity and specificity in the diagnosis of anti-GBM disease. Further cohort studies are needed to confirm their precise diagnostic accuracy and compare diagnostic accuracies among different immunoassays.

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The Immunobiological Agents for Treatment of Antiglomerular Basement Membrane Disease

Yamashita,

Takayasu,

Maruyama

et al. 2023

Medicina

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Combination therapy with glucocorticoids, cyclophosphamide, and plasmapheresis is recommended as the standard treatment for anti-glomerular basement membrane (anti-GBM) disease, but the prognosis of this disease remains poor. Several immunobiological agents have been administered or are expected to be useful for anti-GBM disease in light of refractory disease or the standard treatments’ tolerability. Many data regarding the use of biologic agents for anti-GBM disease have accumulated, verifying the effectiveness and potential of biologic agents as a new treatment option for anti-GBM disease. Tumor necrosis factor (TNF) inhibitors were shown to be useful in animal studies, but these agents have no clinical use and were even shown to induce anti-GBM disease in several cases. Although the efficacy of the TNF-receptor antagonist has been observed in animal models, there are no published case reports of its clinical use. There are also no published reports of animal or clinical studies of anti-B-cell-activating factor, which is a member of the TNF family of agents. Anti-interleukin (IL)-6 antibodies have been demonstrated to have no effect on or to exacerbate nephritis in animal models. Anti-C5 inhibitor was observed to be useful in a few anti-GBM disease cases. Among the several immunobiological agents, only rituximab has been demonstrated to be useful in refractory or poor-tolerance patients or small uncontrolled studies. Rituximab is usually used in combination with steroids and plasma exchange and is used primarily as an alternative to cyclophosphamide, but there is insufficient evidence regarding the efficacy of rituximab for anti-GBM disease, and thus, randomized controlled studies are required.

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Anti-glomerular basement membrane disease: A clinicomorphological study of 16 cases

Cited by 3 publications

References 13 publications

An unusual presentation of Goodpasture syndrome

An unusual presentation of Goodpasture syndrome

Accuracy of Anti-GBM Antibodies in Diagnosing Anti-Glomerular Basement Membrane Disease: A Systematic Review and Meta-Analysis

The Immunobiological Agents for Treatment of Antiglomerular Basement Membrane Disease

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