{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T00:32:01Z","timestamp":1792110721681,"version":"4.4.0"},"reference-count":101,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"7","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["CJASN"],"published-print":{"date-parts":[[2017,7]]},"abstract":"<jats:p>\n                    Anti\u2013glomerular basement membrane (anti-GBM) disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs. It is an archetypic autoimmune disease, caused by the development of directly pathogenic autoantibodies targeting a well characterized autoantigen expressed in the basement membranes of these organs, although the inciting events that induce the autoimmune response are not fully understood. The recent confirmation of spatial and temporal clustering of cases suggests that environmental factors, including infection, may trigger disease in genetically susceptible individuals. The majority of patients develop widespread glomerular crescent formation, presenting with features of rapidly progressive GN, and 40%\u201360% will have concurrent alveolar hemorrhage. Treatment aims to rapidly remove pathogenic autoantibody, typically with the use of plasma exchange, along with steroids and cytotoxic therapy to prevent ongoing autoantibody production and tissue inflammation. Retrospective cohort studies suggest that when this combination of treatment is started early, the majority of patients will have good renal outcome, although presentation with oligoanuria, a high proportion of glomerular crescents, or kidney failure requiring dialysis augur badly for renal prognosis. Relapse and recurrent disease after kidney transplantation are both uncommon, although\n                    <jats:italic toggle=\"yes\">de novo<\/jats:italic>\n                    anti-GBM disease after transplantation for Alport syndrome is a recognized phenomenon. Copresentation with other kidney diseases such as ANCA-associated vasculitis and membranous nephropathy seems to occur at a higher frequency than would be expected by chance alone, and in addition atypical presentations of anti-GBM disease are increasingly reported. These observations highlight the need for future work to further delineate the immunopathogenic mechanisms of anti-GBM disease, and how to better refine and improve treatments, particularly for patients presenting with adverse prognostic factors.\n                  <\/jats:p>","DOI":"10.2215\/cjn.01380217","type":"journal-article","created":{"date-parts":[[2017,5,17]],"date-time":"2017-05-17T20:20:15Z","timestamp":1495052415000},"page":"1162-1172","source":"Crossref","is-referenced-by-count":380,"title":["Anti-Glomerular Basement Membrane Disease"],"prefix":"10.2215","volume":"12","author":[{"given":"Stephen P.","family":"McAdoo","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles D.","family":"Pusey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2017,5,17]]},"reference":[{"key":"B1-20230813","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1002\/art.37715","article-title":"2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides","volume":"65","author":"Jennette","year":"2013","journal-title":"Arthritis Rheum"},{"key":"B2-20230813","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1111\/imj.1958.7.2.132","article-title":"Goodpasture\u2019s syndrome (pulmonary haemorrhage associated with glomerulonephritis)","volume":"7","author":"Stanton","year":"1958","journal-title":"Australas Ann Med"},{"key":"B3-20230813","doi-asserted-by":"crossref","first-page":"863","DOI":"10.1097\/00000441-191911000-00012","article-title":"The significance of certain pulmonary lesions in relation to the etiology of influenza","volume":"158","author":"Goodpasture","year":"1919","journal-title":"Am J Med Sci"},{"key":"B4-20230813","doi-asserted-by":"crossref","first-page":"1009","DOI":"10.7326\/0003-4819-60-6-1009","article-title":"Immune aspects of the glomerulonephritis associated with pulmonary hemorrhage","volume":"60","author":"Scheer","year":"1964","journal-title":"Ann Intern Med"},{"key":"B5-20230813","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1084\/jem.126.6.989","article-title":"The role of anti-glomerular basement membrane antibody in the pathogenesis of human glomerulonephritis","volume":"126","author":"Lerner","year":"1967","journal-title":"J Exp Med"},{"key":"B6-20230813","doi-asserted-by":"crossref","first-page":"1168","DOI":"10.4049\/jimmunol.103.6.1168","article-title":"The presence of anti-glomerular basement membrane antibodies in peripheral blood","volume":"103","author":"McPhaul","year":"1969","journal-title":"J Immunol"},{"key":"B7-20230813","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1038\/ki.1973.14","article-title":"Anti-glomerular basement membrane antibody-induced glomerulonephritis","volume":"3","author":"Wilson","year":"1973","journal-title":"Kidney Int"},{"key":"B8-20230813","doi-asserted-by":"crossref","first-page":"1392","DOI":"10.2215\/CJN.13591215","article-title":"Spatial and temporal clustering of anti-glomerular basement membrane disease","volume":"11","author":"Canney","year":"2016","journal-title":"Clin J Am Soc Nephrol"},{"key":"B9-20230813","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1172\/JCI108286","article-title":"Glomerulonephritis mediated by antibody to glomerular basement membrane. 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