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Procoagulant Activity in Diseases of The Kidney
Published in Gary A. Levy, Edward H. Cole, Procoagulant Activity in Health and Disease, 2019
Stephen Holdsworth, Peter Tipping
A number of studies have demonstrated that fibrin deposition is most prominent in those forms of glomerulonephritis with the worst prognosis and the greatest evidence of glomerular injury.27,28 Clinicopathological correlations in a variety of diseases with glomerulonephritis clearly show that prominent glomerular fibrin deposition is associated with crescent formation and renal failure. These include a variety of systemic diseases with prominent immune-complex deposition, e.g., systemic lupus erythematosis29,30 and cryoglobulinemia.31 However, fibrin deposition is not limited to immune complex-associated diseases. Anti-GBM disease is an autoimmune disease in which the immune response is directed at components of the glomerular basement membrane. This results in linear deposition of immunoglobulin along the glomerular basement membrane and is commonly associated with severe proliferative crescentic glomerulonephritis with prominent fibrin deposition.32
Noninfectious Pulmonary Manifestations of Renal Disease In Children
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
Stephen T. Lawless, H. Jorge Baluarte
Evidence of anti-GBM antibody formation may be obtained in several ways. The first is by the demonstration of linear, ribbon-like deposits of IgG along the glomerular capillary walls by immunofluorescent study of renal biopsy material. These deposits are frequently (80%), but not necessarily, accompanied by C3, usually in an irregular or interrupted linear fashion. The association of these deposits with proliferative glomerulonephritis, particularly of the crescentric variety, can confidently be assumed to represent in vivo fixation of anti-GBM antibody. Second, circulating anti-GBM antibody may be demonstrated by precipitation in-gel, indirect immunofluorescence, passive hemagglutination, or radioimmunoassay procedures.19 Third, if sufficient renal or lung tissue is available, elution studies may be carried out to demonstrate directly the antibody nature and specificity of deposited IgG.
Pulmonary vasculitis and alveolar haemorrhage syndromes
Published in Muhunthan Thillai, David R Moller, Keith C Meyer, Clinical Handbook of Interstitial Lung Disease, 2017
Rebecca C Keith, Stephen K Frankel
Anti-GBM (glomerular basement membrane) disease may present as with pulmonary-renal syndrome, isolated glomerulonephritis or isolated DAH. Anti-GBM disease is characterized by serum autoantibodies to the collagen IV alpha-3 constituents of the basement membrane (27). Pulmonary haemorrhage occurs in approximately 50% of patients when antibodies react to the pulmonary basement membrane (27). It is a rare disease, occurring in one person per million (28). Isolated lung disease is uncommon but does occur (29). Diagnosis can be made by the identification of serum anti-GBM antibodies, but not all patients will have positive serologies. A renal biopsy is often performed to confirm the diagnosis.
Clinical and pathological features of anti-glomerular basement membrane disease associated with membranous nephropathy: an observational study
Published in Renal Failure, 2022
Shasha Zhang, Chaofan Li, Jing Huang, Yan Zhou, Caifeng Gao, Mengyao Sun, Rong Wang, Bing Chen
Anti-GBM disease was diagnosed by circulating or renal anti-GBM antibody positivity. Furthermore, the key diagnostic criteria of anti-GBM GN was a large proportion of crescents at similar stages, with linear staining of IgG along the GBM [14]. Patients who tested positive for ANCAs were enrolled in the anti-GBM + ANCA group. All the ANCA-positive patients in our study were anti-MPO positive. Renal samples with anti-GBM disease were involved in the anti-GBM + MN group if they also displayed glomerular capillary wall thickening, granular deposition of IgG and C3 along the capillary wall, and glomerular subepithelial electron-dense deposition. The causes which could be associated with secondary MN, such as hepatitis B/C virus infection, lupus, malignancy, drugs, and heavy metal poisoning, were not found in our anti-GBM + MN cases. Considering extracorporeal shock wave lithotripsy (ESWL) may be associated with anti-GBM pathogenesis, we focused on the history of renal stones and ESWL in the medical investigation [15]. No subjects had renal calculi or accepted ESWL prior to the diagnosis. In addition, no stones were found by renal ultrasound at the time of diagnosis.
Anti-glomerular basement membrane disease mediated by IgG and IgA: a case report
Published in Renal Failure, 2021
Guming Zou, Haitao Lu, Li Zhuo, Wanzhong Zou, Wenge Li
Anti-GBM disease is a rare autoimmune disorder with an estimated incidence of <1 per million population [2]. The major class of anti-GBM autoantibody deposited along the GBM on kidney biopsies is IgG, with exceptionally rare reports of IgA or IgM classified as atypical anti-GBM disease [4]. Collagen IV is the main constituent of all basement membranes, a specialized form of extracellular matrix, which supports tissue integrity and plays roles in a number of key functions, including cell signaling, morphogenesis, and tissue regeneration [15]. Commonly, IgG anti-GBM antibodies bind the α3 subunit of the NC1 domain of type IV collagen (α3(IV)NC1). In addition to the ubiquitous autoantibodies against α3 NC1, distinct antibodies specific for the α5 NC1 domain have also been detected [16]. We performed a further analysis of the IgA and IgG antibodies capable of binding to α3(IV)NC1 in our patient.
Comparison of the performance of a chemiluminescence assay and an ELISA for detection of anti-GBM antibodies
Published in Renal Failure, 2020
Ying Tan, Wei Pang, Xiaoyu Jia, Ming-hui Zhao
Anti–glomerular basement membrane (anti-GBM) disease is a rare autoimmune disorder that is characterized by the production of autoantibodies directed to the GBM, rapidly progressive glomerulonephritis, and a high risk for alveolar hemorrhage [1]. Anti-GBM disease is a rare disease with a yearly incidence of 0.5–1 cases per million inhabitants, which can result in a rapid deterioration in renal function. The pathogenic role of anti-GBM antibodies has been demonstrated by their ability to transfer the disease to monkeys and by the recurrence of disease in human kidney allografts [2]. Early and accurate identification of anti-GBM antibodies are essential for renal prognosis since patients showed poor outcomes when the initial serum creatinine level was over 6.8mg/dL [3].