{"id":10510,"date":"2009-08-12T16:06:25","date_gmt":"2009-08-12T13:06:25","guid":{"rendered":"https:\/\/www.synevo.ro\/afectiuni\/profil-vasculite-si-boala-cu-anticorpi-anti-membrana-bazala-glomerulara\/"},"modified":"2020-07-10T11:48:26","modified_gmt":"2020-07-10T08:48:26","slug":"profil-vasculite-si-boala-cu-anticorpi-anti-membrana-bazala-glomerulara","status":"publish","type":"synevo_diseases","link":"https:\/\/old.synevo.md\/ru\/afectiuni\/profil-vasculite-si-boala-cu-anticorpi-anti-membrana-bazala-glomerulara\/","title":{"rendered":"Profil vasculite \u015fi boala cu anticorpi anti-membran\u0103 bazal\u0103 glomerular\u0103"},"content":{"rendered":"<p style=\"text-align: justify;\"><span style=\"color: #00539f;\"><strong>Vasculitele sistemice <\/strong><\/span>descrise prima oar\u0103 ca entitate \u00een 1866, sunt considerate afec\u0163iuni rare, inciden\u0163a lor anual\u0103 fiind apreciat\u0103 la ~1\/100000 locuitori, exist\u00e2nd diferen\u0163e semnificative \u00eentre anumite tipuri de vasculite sau grupe popula\u0163ionale. <!--more-->Se caracterizeaz\u0103 prin semne inflamatorii tipice la nivelul peretelului vascular, inflama\u0163ia put\u00e2nd afecta arterele de orice dimensiune \u015fi de asemenea venele. Exist\u0103 diferite tipuri de clasific\u0103ri ale vasculitelor, \u00een func\u0163ie de criteriile utilizate: m\u0103rimea vasului afectat, etiologia, anatomopatologia, mecanismul patogenic sau prezen\u0163a unor markeri imuni \u2013 ANCA.<\/p>\n<hr \/>\n<p style=\"text-align: justify;\"><strong>Clinic<\/strong>, simptomele generale (oboseala, febra, sc\u0103derea \u00een greutate) alc\u0103tuiesc adesea tabloul clinic de debut, ulterior manifest\u0103rile clinice variaz\u0103 \u00een func\u0163ie de patternul vaselor afectate (mici, mijlocii sau mari). Astfel afectarea vaselor mici se poate manifesta prin: purpur\u0103, polineurit\u0103, episclerit\u0103, hemoptizii sau microhematurie, afectarea vaselor mijlocii poate conduce la infarcte \u00een diferite organe cum ar fi cord, rinichi, intestine sau extremita\u0163i, dar \u015fi ale vaselor cerebrale (cu apari\u0163ia \u201cstroke\u201d), iar afectarea vaselor mari se manifest\u0103 ca sindrom de arc aortic sau ocluzie venoas\u0103 trombotic\u0103.<\/p>\n<p style=\"text-align: justify;\"><strong>Paraclinic<\/strong>, vasculitele se caracterizeaz\u0103 prin sindrom inflamator foarte intens, modific\u0103ri imunologice nespecifice \u015fi uneori markeri imuni cu o oarecare specificitate reprezenta\u0163i de autoanticorpii anticitoplasma neutrofile (ANCA). Odat\u0103 cu descrierea ANCA s-a \u00eencercat utilizarea acestora drept criteriu pentru clasificarea vasculitelor descriindu-se o subclas\u0103 de vasculite ANCA-pozitive, categorie \u00een care sunt incluse: granulomatoz\u0103 Wegener, sindromul Churg-Strauss \u015fi poliangeita microscopic\u0103, entit\u0103\u0163i care mai au \u00een comun afectarea arterelor medii \u015fi mici, riscul crescut de interesare renal\u0103 \u015fi lipsa de implicare a complexelor imune \u00een patogenia bolii.<sup>1,2<\/sup><\/p>\n<hr \/>\n<p style=\"text-align: justify;\"><span style=\"color: #00539f;\"><strong>Boala cu anticorpi anti-membran\u0103 bazal\u0103 glomerular\u0103 (anti-GBM) <\/strong><\/span>este o afec\u0163iune autoimun\u0103 rar\u0103 cu manifest\u0103ri renale \u015fi pulmonare, caracterizat\u0103 prin distruc\u0163ia mediat\u0103 prin autoanticorpi a membranelor bazale alveolare \u015fi glomerulare. Acest model de glomerulonefrit\u0103 rapid progresiv\u0103 \/insuficien\u0163\u0103 renal\u0103 \u015fi hemoragie pulmonar\u0103 este adesea \u00eent\u00e2lnit sub numele de sindromul Goodpasture \u015fi poate fi cauzat de diferite afec\u0163iuni; boala cu anti-GMB reprezint\u0103 o cauz\u0103 bine definit\u0103; alte cauze posibile vasculite sistemice (granulomatoza Wegener, poliangeita microscopica, sindromul Churg-Strauss, LES), alte vasculite(boala Behcet, crioglobulinemia, post-medicamentoase: penicilamina, hidralazina) etc.<\/p>\n<p style=\"text-align: justify;\"><span style=\"color: #00539f;\"><strong>Boala cu anticorpi anti-membran\u0103 bazal\u0103 glomerular\u0103 <\/strong><\/span>are o inciden\u0163a mai mare \u00een popula\u0163ia caucazian\u0103 \u015fi anume de 1caz \/2mil.\/an; reprezint\u0103 1-5% din toate tipurile de glomerulonefrite si 10-20% dintre glomerulonefritele progresive.<sup>3<\/sup><\/p>\n<p style=\"text-align: justify;\">Principala protein\u0103 a membranei bazale glomerulare este colagenul tip IV care prezint\u0103 propriet\u0103\u0163i autoagregante \u015fi formeaz\u0103 o matrice \u00een care sunt integrate moleculele altor membrane bazale. Colagenul tip IV, la r\u00e2ndul s\u0103u, este alc\u0103tuit din 6 lan\u0163uri \u03b1 diferite, \u00eens\u0103 antigenul \u0163int\u0103 pentru anti-GBM este reprezentat doar de un mic fragment al lan\u0163ului \u03b13 denumit domeniul NC1. Prin imunofluorescen\u0163\u0103 pot fi identificate la nivelul membranei bazale depozite lineare alc\u0103tuite din complexe antigen-anticorp, ce constituie elementele histopatologice specifice sindromului.<sup>4,6<\/sup><\/p>\n<p style=\"text-align: justify;\">\u00centruc\u00e2t epitopii antigenici sunt ascun\u015fi \u00een triplul helix al colagenului, este posibil s\u0103 fie necesar\u0103 prezen\u0163a unor factori de mediu (ex. fumat, expunere la hidrocarburi) pentru prezentarea antigenelor criptice Goodpasture sistemului imun al organismului; au fost raportate cazuri de boala anti-GBM \u015fi dup\u0103 litotri\u0163ie \u015fi obstruc\u0163ie ureteric\u0103, suger\u00e2nd c\u0103 antigenul eliberat prin afectarea mecanic\u0103 renal\u0103 poate ini\u0163ia boala la indivizii susceptibili.<sup>3,5<\/sup><\/p>\n<p style=\"text-align: justify;\"><strong>Clinic, manifest\u0103rile principale sunt:<\/strong><\/p>\n<ul>\n<li style=\"text-align: justify;\"><strong>hemoragia pulmonar\u0103<\/strong> \u2013 cea mai obi\u015fnuit\u0103 form\u0103 de prezentare ( 70% din cazuri), adesea precede debutul bolii renale cu luni sau ani; apare aproape exclusiv la fum\u0103tori, factori precipitan\u0163i \u2013 infec\u0163iile intercurente \u015fi supra\u00eencarcarea volemic\u0103; poate fi episodic\u0103 \u015fi la mul\u0163i pacien\u0163i se rezolv\u0103 spontan, la al\u0163ii poate progresa conduc\u00e2nd la insuficien\u0163\u0103 respiratorie, principala cauz\u0103 de moarte precoce la pacien\u0163ii cu anti-GBM, ce face imperativ diagnosticul precoce al bolii;<\/li>\n<li style=\"text-align: justify;\"><strong>afectarea renal\u0103<\/strong> \u2013 apare izolat sau \u00een asociere cu manifest\u0103rile pulmonare; \u00een general, odat\u0103 ce a ap\u0103rut o leziune semnificativ\u0103 progreseaz\u0103 rapid spre insuficien\u0163\u0103 renal\u0103 \u015fi rareori se rezolv\u0103 spontan.<sup>3<\/sup><\/li>\n<\/ul>\n<hr \/>\n<p style=\"text-align: justify;\"><strong>Diagnosticul<\/strong> este stabilit pe baza triadei:<\/p>\n<ul>\n<li style=\"text-align: justify;\">hemoragie pulmonar\u0103 difuz\u0103;<\/li>\n<li style=\"text-align: justify;\">glomerulonefrit\u0103;<\/li>\n<li style=\"text-align: justify;\">existen\u0163a de anticorpi circulan\u0163i anti-GBM.<\/li>\n<\/ul>\n<p style=\"text-align: justify;\">Cel mai adesea, cele 3 componente ale sindromului sunt prezente simultan \u00een perioada de activitate a bolii, de\u015fi au fost descrise cazuri de afectare renal\u0103 sau pulmonar\u0103 izolate.<sup>6<\/sup><\/p>\n<p style=\"text-align: justify;\"><strong>Diagnosticul de sindrom Goodpasture<\/strong> sau de boal\u0103 cu anti-GBM prezen\u0163i este dependent de detec\u0163ia acestor autoanticorpi. Afec\u0163iunea apare tipic la barba\u0163ii tineri; dac\u0103 nu este depistat\u0103 \u00een stadiu precoce are o evolu\u0163ie rapid-progresiv\u0103, cu poten\u0163ial letal.<\/p>\n<p style=\"text-align: justify;\"><strong>Anti-GBM<\/strong> pot fi detecta\u0163i \u00eentr-un procent de 8-10% la pacien\u0163ii cu manifest\u0103ri reno-pulmonare asociate cu prezen\u0163a autoanticorpilor de tip ANCA. \u00cen func\u0163ie de titrul ANCA \u015fi anti-GBM, tabloul clinic al afec\u0163iunii renale include predominant simptome\/semne de tip vasculitic sau respectiv de tip boal\u0103 cu anti-GBM prezen\u0163i. Aceast\u0103 distinc\u0163ie este important\u0103 deoarece atitudinea terapeutic\u0103 este mai agresiv\u0103 \u00een cazul bolii cu anti-GBM prezen\u0163i (include \u015fi plasmaferez\u0103).<sup>4<\/sup><\/p><div class=\"synevo-shop-products\">\r\n    <div class=\"sysprod\">\r\n        \r\n        <div class=\"sysprod-products\">\r\n                            <div class=\"sysprod-product\">\r\n                    <a class=\"sysprod-product-title\" href=\"https:\/\/old.synevo.md\/ru\/shop\/anticorpi-anti-citoplasma-neutrofile-anca-imunofluorescenta\/\">Anticorpi anti-citoplasma neutrofile (ANCA) &#8211; imunofluorescenta<\/a>\r\n                    <div class=\"sysprod-product-price\">\r\n                        <span>Pre\u021b:<\/span>\r\n                        <span>1120 lei<\/span>\r\n                    <\/div>\r\n                                            <div class=\"sysprod-product-addcart\">\r\n                            <a\r\n                                href=\"?add-to-cart=9145\"\r\n                                data-quantity=\"1\"\r\n                                class=\"addToCart button product_type_simple add_to_cart_button ajax_add_to_cart \"\r\n                                data-product_id=\"9145\"\r\n                                data-product_sku=\"IM45\"\r\n                                rel=\"nofollow\"\r\n                            ><div><\/div><\/a>\r\n                        <\/div>\r\n                                    <\/div>\r\n                            <div class=\"sysprod-product\">\r\n                    <a class=\"sysprod-product-title\" href=\"https:\/\/old.synevo.md\/ru\/shop\/anticorpi-anti-membrana-bazala-glomerulara\/\">Anticorpi anti-membran\u0103 bazal\u0103 glomerular\u0103<\/a>\r\n                    <div class=\"sysprod-product-price\">\r\n                        <span>Pre\u021b:<\/span>\r\n                        <span>380.00 lei<\/span>\r\n                    <\/div>\r\n                                            <div class=\"sysprod-product-addcart\">\r\n                            <a\r\n                                href=\"?add-to-cart=9151\"\r\n                                data-quantity=\"1\"\r\n                                class=\"addToCart button product_type_simple add_to_cart_button ajax_add_to_cart \"\r\n                                data-product_id=\"9151\"\r\n                                data-product_sku=\"IM83\"\r\n                                rel=\"nofollow\"\r\n                            ><div><\/div><\/a>\r\n                        <\/div>\r\n                                    <\/div>\r\n                    <\/div>\r\n        \r\n    <\/div>\r\n<\/div>\r\n\r\n<style>\r\n.synevo-shop-products {\r\n    padding: 24px 0;\r\n    margin: 24px 0;\r\n    border-radius: 10px;\r\n    background: #F6F7F9;\r\n}\r\n.synevo-shop-products .sysprod-product {\r\n    position: relative;\r\n    margin: 0 24px;\r\n    margin-bottom: 24px;\r\n    border-radius: 10px;\r\n    background: #fff;\r\n    display: flex;\r\n    flex-direction: row;\r\n    align-items: center;\r\n}\r\n.synevo-shop-products .sysprod-product:last-child {\r\n    margin-bottom: 0;\r\n}\r\n.synevo-shop-products .sysprod-product-title {\r\n    flex-grow: 1;\r\n    padding: 15px;\r\n    margin-left: 14px;\r\n    font-family: Poppins,sans-serif;\r\n    font-weight: 600;\r\n    font-size: 16px;\r\n    color: #29263F;\r\n}\r\n.synevo-shop-products .sysprod-product-price {\r\n    flex-shrink: 0;\r\n    font-family: Poppins,sans-serif;\r\n    font-weight: 600;\r\n    font-size: 18px;\r\n    color: #FDB813;\r\n}\r\n.synevo-shop-products .sysprod-product-addcart {\r\n    flex-shrink: 0;\r\n    position: relative;\r\n    width: 38px;\r\n    height: 38px;\r\n    margin-left: 24px;\r\n    margin-right: 24px;\r\n}\r\n.synevo-shop-products .sysprod-product-addcart .button {\r\n    top: unset;\r\n    right: unset;\r\n}\r\n<\/style><div class=\"wp-block-columns is-layout-flex wp-container-core-columns-is-layout-1 wp-block-columns-is-layout-flex\"><div class=\"wp-block-column is-vertically-aligned-center is-layout-flow wp-block-column-is-layout-flow\" style=\"flex-basis:33.33%\"><p><strong>Analize medicale disponibile \u00een laboratoarele Synevo:<\/strong><\/p><\/div>\n\n<div class=\"wp-block-column is-layout-flow wp-block-column-is-layout-flow\" style=\"flex-basis:66.66%\"><div class=\"synevo-shop-products\">\r\n    <div class=\"sysprod\">\r\n        \r\n        <div class=\"sysprod-products\">\r\n                            <div class=\"sysprod-product\">\r\n                    <a class=\"sysprod-product-title\" href=\"https:\/\/old.synevo.md\/ru\/shop\/anticorpi-anti-citoplasma-neutrofile-anca-imunofluorescenta\/\">Anticorpi anti-citoplasma neutrofile (ANCA) &#8211; imunofluorescenta<\/a>\r\n                    <div class=\"sysprod-product-price\">\r\n                        <span>Pre\u021b:<\/span>\r\n                        <span>1120 lei<\/span>\r\n                    <\/div>\r\n                                            <div class=\"sysprod-product-addcart\">\r\n                            <a\r\n                                href=\"?add-to-cart=9145\"\r\n                                data-quantity=\"1\"\r\n                                class=\"addToCart button product_type_simple add_to_cart_button ajax_add_to_cart \"\r\n                                data-product_id=\"9145\"\r\n                                data-product_sku=\"IM45\"\r\n                                rel=\"nofollow\"\r\n                            ><div><\/div><\/a>\r\n                        <\/div>\r\n                                    <\/div>\r\n                            <div class=\"sysprod-product\">\r\n                    <a class=\"sysprod-product-title\" href=\"https:\/\/old.synevo.md\/ru\/shop\/anticorpi-anti-membrana-bazala-glomerulara\/\">Anticorpi anti-membran\u0103 bazal\u0103 glomerular\u0103<\/a>\r\n                    <div class=\"sysprod-product-price\">\r\n                        <span>Pre\u021b:<\/span>\r\n                        <span>380.00 lei<\/span>\r\n                    <\/div>\r\n                                            <div class=\"sysprod-product-addcart\">\r\n                            <a\r\n                                href=\"?add-to-cart=9151\"\r\n                                data-quantity=\"1\"\r\n                                class=\"addToCart button product_type_simple add_to_cart_button ajax_add_to_cart \"\r\n                                data-product_id=\"9151\"\r\n                                data-product_sku=\"IM83\"\r\n                                rel=\"nofollow\"\r\n                            ><div><\/div><\/a>\r\n                        <\/div>\r\n                                    <\/div>\r\n                    <\/div>\r\n        \r\n    <\/div>\r\n<\/div>\r\n\r\n<style>\r\n.synevo-shop-products {\r\n    padding: 24px 0;\r\n    margin: 24px 0;\r\n    border-radius: 10px;\r\n    background: #F6F7F9;\r\n}\r\n.synevo-shop-products .sysprod-product {\r\n    position: relative;\r\n    margin: 0 24px;\r\n    margin-bottom: 24px;\r\n    border-radius: 10px;\r\n    background: #fff;\r\n    display: flex;\r\n    flex-direction: row;\r\n    align-items: center;\r\n}\r\n.synevo-shop-products .sysprod-product:last-child {\r\n    margin-bottom: 0;\r\n}\r\n.synevo-shop-products .sysprod-product-title {\r\n    flex-grow: 1;\r\n    padding: 15px;\r\n    margin-left: 14px;\r\n    font-family: Poppins,sans-serif;\r\n    font-weight: 600;\r\n    font-size: 16px;\r\n    color: #29263F;\r\n}\r\n.synevo-shop-products .sysprod-product-price {\r\n    flex-shrink: 0;\r\n    font-family: Poppins,sans-serif;\r\n    font-weight: 600;\r\n    font-size: 18px;\r\n    color: #FDB813;\r\n}\r\n.synevo-shop-products .sysprod-product-addcart {\r\n    flex-shrink: 0;\r\n    position: relative;\r\n    width: 38px;\r\n    height: 38px;\r\n    margin-left: 24px;\r\n    margin-right: 24px;\r\n}\r\n.synevo-shop-products .sysprod-product-addcart .button {\r\n    top: unset;\r\n    right: unset;\r\n}\r\n<\/style><\/div><\/div><p><small><strong>Bibliografie:<\/strong><\/small><\/p><p><small>1. Werner J.Mayet, Autoantibodies in systemic vasculitis, \u00een Clinical Laboratory Diagnostics-Use and Assessment of Clinical Laboratory Results. Lothar Thomas. TH-Books Verlagsgesellschaft mbH, Frankfurt \/Main, Germany, 1 Ed., 1998, 841-8452. Andra Balanescu, Vasculitele sistemice, \u00een Esentialul in reumatologie, coordonator Ruxandra Ionescu, Ed. a 2-a, 2007, 315-3383. David C. Kluth, Andrew J. Rees, Anti-Glomerular Basement Membrane Disease, Journal American Society of Nephrology 10;2446-2453, 1999<br>4. Laborator Synevo. Referin\u0163ele specifice tehnologiei de lucru utilizate 2006. Ref Type: Catalog<br>5. Ramesh Saxena, MD, PhD, Antiglomerular Basement Membrane Disease, www.emedicine, 2006<br>6.Pina Joseph, Goodpasture Syndrome: A Case of Delayed Appearance of Autoantibodies and Renal Disease, In Military Medicine, Sept.2001<\/small><\/p><hr class=\"wp-block-separator\"\/><p class=\"has-normal-font-size\">Mai multe informa\u021bii despre testele disponibile \u00een laboratoarele Synevo: <\/p><p style=\"font-size:18px\"><a href=\"\/shop\/anticorpi-anti-citoplasma-neutrofile-imunofluorescenta\/\" class=\"rank-math-link\"><small>Anticorpi anti-citoplasm\u0103 neutrofile (ANCA)<\/small><\/a><\/p><p style=\"font-size:18px\"><small><a href=\"\/shop\/anticorpi-anti-membrana-bazala-glomerulara\/\" class=\"rank-math-link\">Anticorpi anti-GBM<\/a><\/small><\/p>","protected":false},"featured_media":0,"template":"","synevo_diseases_category":[902],"class_list":["post-10510","synevo_diseases","type-synevo_diseases","status-publish","hentry","synevo_diseases_category-teste-alergologie"],"_links":{"self":[{"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/synevo_diseases\/10510","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/synevo_diseases"}],"about":[{"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/types\/synevo_diseases"}],"wp:attachment":[{"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/media?parent=10510"}],"wp:term":[{"taxonomy":"synevo_diseases_category","embeddable":true,"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/synevo_diseases_category?post=10510"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}