{"id":7883,"date":"2021-10-29T19:58:32","date_gmt":"2021-10-29T17:58:32","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/nephrology\/rapid-progressive-glomerulonephritis-anca-associated-vasculitis-goodpasture-syndrome\/"},"modified":"2021-12-09T23:58:47","modified_gmt":"2021-12-09T21:58:47","slug":"rapid-progressive-glomerulonephritis-anca-associated-vasculitis-goodpasture-syndrome","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/nephrology\/rapid-progressive-glomerulonephritis-anca-associated-vasculitis-goodpasture-syndrome\/","title":{"rendered":"Rapid progressive glomerulonephritis (ANCA-associated vasculitis, Goodpasture syndrome)"},"content":{"rendered":"\n<p class=\"wp-block-paragraph\">As its name suggests, is a type of glomerulonephritis that quickly progresses to renal failure (within weeks to months).<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">The classic lesions include <strong>crescent formations<\/strong> which contain a cellular proliferation that fill up the Bowman&#8217;s space.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">RPGN can be primary (idiopathic) or secondary to another disease.<\/p>\n\n\n<span class=\"block-heading\" id=\"header_1\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Classification<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<p class=\"wp-block-paragraph\">RPGN is classified into 5 entities based on the etiology, determined by histopathological findings, immunofluorescence, and serological tests for the presence of specific antibodies.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Anti-GBM antibody mediated (RPGN type I)<ul><li><strong>Linear<\/strong> deposition of IgG along the GBM (and often C3)<\/li><\/ul><ul><li>Includes the <strong>anti-GBM GN<\/strong> and <strong>Goodpasture syndrome<\/strong>, which are the same, except Goodpasture has lung participation (hemorrhage) as well.<\/li><li>10% of cases<\/li><\/ul><\/li><li>Immune-complex mediated (RPGN type II)<ul><li><strong>Diffuse<\/strong> and irregular deposition of IgG and C3 along the GBM<\/li><li>Postinfectious\/SLE\/IgA nephropathy\/membranoproliferative GN<\/li><li>40% of cases<\/li><\/ul><\/li><li>Pauci-immune (RPGN type III and IV)<ul><li>Autoantibodies or immune deposits are <strong>not detected<\/strong><\/li><li>Can be ANCA-positive (RPGN type III) or ANCA-negative (RPGN type IV)<\/li><li><strong>Vasculitidies<\/strong> including <strong>Granulomatosis with polyangiitis, microscopic polyangiitis, <\/strong>and <strong>idiopathic crescentic GN<\/strong> (which is basically vasculitis that is limited to the kidney)<\/li><li>50% of cases<\/li><\/ul><\/li><li>Double-positive (RPGN type V)<ul><li>Both ANCA and anti-GBM antibodies are present<\/li><li>Same characteristics as anti-GBM<\/li><\/ul><\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_2\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">ANCA-associated GN<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<p class=\"wp-block-paragraph\">Entities include:<\/p>\n\n\n\n<ol class=\"wp-block-list\" type=\"1\"><li><strong>Granulomatosis with Polyangiitis<\/strong> (Wegner&#8217;s); medium+small vessel vasculitis, with granuloma formation, anti-PR3 ANCA; involvement of the upper respiratory tract + kidneys.<\/li><li><strong>Microscopic polyangiitis<\/strong>; small vessel vasculitis, anti-MPO ANCA, involves kidneys, lungs, nerves, skin, joints.<\/li><li><strong>Idiopathic crescentic GN<\/strong> (vasculitis isolated to the kidneys)<\/li><\/ol>\n\n\n\n<p class=\"wp-block-paragraph\">Basically, ANCA antibodies (anti-MPO, and anti-protease-3) bind to antigens presented by neutrophils, causing their excessive activation.<\/p>\n\n\n<\/span><span class=\"block-heading\" id=\"header_3\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Symptoms<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<ul class=\"wp-block-list\"><li>Purpura<\/li><li><strong>Upper airway symptoms (Wegner\u2019s) with hemoptysis requiring hospitalization<\/strong><\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_4\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Diagnosis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<ul class=\"wp-block-list\"><li>Serology (ANCAs)<\/li><li>Biopsy (crescents)<\/li><li>Nephritic signs<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_5\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Treatment<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_5\">\n\n\n<ul class=\"wp-block-list\"><li>Prednisone<\/li><li>Cyclophosphamide<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_6\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Goodpausture syndrome<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_6\">\n\n\n<p class=\"wp-block-paragraph\">A triad of <strong>glomerulonephritis<\/strong> with <strong>pulmonary hemorrhage<\/strong> and <strong>anti-GBM antibodies.<\/strong><\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Usually appears after <strong>RTI<\/strong> with symptoms of <strong>hemoptysis, dyspnea, hematuria<\/strong><\/li><li>Associated with smoking<\/li><li>Appears with a <strong>crescentic proliferation<\/strong> of the glomeruli<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_7\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Diagnosis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_7\">\n\n\n<ul class=\"wp-block-list\"><li>Anti GBM antibodies (serology)<\/li><li>Renal biopsy shows linear immunofluorescence pattern<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_8\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Treatment<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_8\">\n\n\n<ul class=\"wp-block-list\"><li>Plasma exchange<\/li><li>Prednisone<\/li><li>Cyclophosphamide<\/li><\/ul>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Classification<\/h3><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">ANCA-associated GN<\/h3><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Symptoms<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Diagnosis<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Treatment<\/h4><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Goodpausture syndrome<\/h3><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Diagnosis<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Treatment<\/h4><\/div>","protected":false},"excerpt":{"rendered":"<p>As its name suggests, is a type of glomerulonephritis that quickly progresses to renal failure (within weeks to months). The classic lesions include crescent formations which contain a cellular proliferation that fill up the Bowman&#8217;s space. RPGN can be primary (idiopathic) or secondary to another disease. Classification RPGN is classified into 5 entities based on [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":685,"menu_order":6,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-7883","page","type-page","status-publish","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.9 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Rapid progressive glomerulonephritis (ANCA-associated vasculitis, Goodpasture syndrome) &#8211; Meddists<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/nephrology\/rapid-progressive-glomerulonephritis-anca-associated-vasculitis-goodpasture-syndrome\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"2 minutes\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/nephrology\\\/rapid-progressive-glomerulonephritis-anca-associated-vasculitis-goodpasture-syndrome\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/nephrology\\\/rapid-progressive-glomerulonephritis-anca-associated-vasculitis-goodpasture-syndrome\\\/\",\"name\":\"Rapid progressive glomerulonephritis (ANCA-associated vasculitis, Goodpasture syndrome) &#8211; 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