{"id":9330,"date":"2021-12-19T00:23:58","date_gmt":"2021-12-18T22:23:58","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/immunology\/small-vessel-vasculitides-classification-patomechanism-symptoms-treatment\/"},"modified":"2022-10-10T10:09:22","modified_gmt":"2022-10-10T08:09:22","slug":"small-vessel-vasculitides-classification-pathomechanism-symptoms-treatment","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/immunology\/small-vessel-vasculitides-classification-pathomechanism-symptoms-treatment\/","title":{"rendered":"Small vessel vasculitides (classification, pathomechanism, symptoms, treatment)"},"content":{"rendered":"\n<p class=\"wp-block-paragraph\">Vasculitis is essentially an inflammation of the vessel wall. It can be either <strong>infectious<\/strong> or <strong>non-infectious<\/strong> (autoimmune).<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Generally, &#8220;vasculitis&#8221; is often mentioned on its own, referring to the latter (non-infectious) form. It is classified based on the vessel size (large vessels such as the aorta and its branches, medium vessels, and small vessels).<\/p>\n\n\n<span class=\"block-heading\" id=\"header_1\">\n<h2 class=\"wp-block-heading\" id=\"mechanisms\" class=\"wp-block-heading\" id=\"mechanisms\" class=\"title_collection title1\">Mechanisms<\/h2>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<ul class=\"wp-block-list\"><li>Immune complex deposition<\/li><li>Neutrophil-mediated (ANCAs; pauci-immune)<\/li><li>Anti-endothelial activity<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_2\">\n<h2 class=\"wp-block-heading\" id=\"small-vessel-vasculitides\" class=\"wp-block-heading\" id=\"small-vessel-vasculitides\" class=\"title_collection title1\">Small vessel vasculitides<\/h2>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<p class=\"wp-block-paragraph\">Small vessel vasculitides include:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>ANCA-associated vasculitides:<ul><li>Granulomatosis with polyangiitis (Wegner&#8217;s granulomatosis)<\/li><li>Eosinophilic granulomatosis with polyangiitis (Churg-strauss syndrome)<\/li><\/ul><ul><li>Microscopic polyangiitis<\/li><\/ul><\/li><li>Immune-complex vasculitides:<ul><li>IgA vasculitis (Henoch-Sch\u00f6nlein purpura)<\/li><li>Goodpasture syndrome<\/li><li>Essential cryoglobulinemic vasculitis<\/li><\/ul><\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_3\">\n<h3 class=\"wp-block-heading\" id=\"anca-associated-vasculitides\" class=\"wp-block-heading\" id=\"anca-associated-vasculitides\" class=\"title_collection title2\">ANCA-associated vasculitides<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<p class=\"wp-block-paragraph\">These are vasculitides without the presence of immune complexes, but rather they are neutrophil-mediated, due to antineutrophil cytoplasm antibodies (ANCAs) directed against proteinase-3 (c-ANCA) and MPO (p-ANCA).<\/p>\n\n\n<\/span><span class=\"block-heading\" id=\"header_4\">\n<h4 class=\"wp-block-heading\" id=\"granulomatosis-with-polyangiitis-gpa\" class=\"wp-block-heading\" id=\"granulomatosis-with-polyangiitis-gpa\" class=\"title_collection title3\">Granulomatosis with polyangiitis (GPA)<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<p class=\"wp-block-paragraph\">Granulomatosis with polyangiitis (previously known as Wegner&#8217;s) presents with a triad of upper respiratory tract, lower respiratory tract, and kidney hemorrhages (although any organ can be affected).<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>The lesions are characterized by necrotizing granulomatous inflammation.<\/li><li>Symptoms:<ul><li>Usually affects the upper and lower respiratory tract first (epistaxis, damage to the nose and its collapse, hemoptysis, hemorrhage, pulmonary nodules and cavitation)<\/li><li>Kidneys (glomerulonephritis, may progress to CKD and ESRD)<\/li><li>Other tissues are affected later (myalgia, mononeuritis multiplex, hearing loss, inflammation of the eye and vision loss)<\/li><\/ul><\/li><li>Diagnosis:<ul><li>GPA should be suspected in patients with recurrent upper respiratory and middle ear symptoms, and glomerulonephritis.<\/li><li>Labwork (ESR, CRP), urinalysis<\/li><li>Chest x-ray<\/li><li>Serology (<strong>c-ANCA<\/strong>)<\/li><li>Biopsy<\/li><\/ul><\/li><li>Treatment:<ul><li>Treated with high-dose corticosteroids with methotrexate, and if severe, add cyclophosphamide or rituximab.<\/li><li>Treatment of the glomerulonephritis (ACEi\/ARB), kidney transplantation if needed.<\/li><\/ul><\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_5\">\n<h4 class=\"wp-block-heading\" id=\"eosinophilic-granulomatosis-with-polyangiitis-egpa\" class=\"wp-block-heading\" id=\"eosinophilic-granulomatosis-with-polyangiitis-egpa\" class=\"title_collection title3\">Eosinophilic granulomatosis with polyangiitis (EGPA)<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_5\">\n\n\n<p class=\"wp-block-paragraph\">Eosinophilic granulomatosis with polyangiitis is characterized by necrotizing granulomatous vascular lesions rich in eosinophils (although eosinophils are not always present), typically appearing in patients with a history of allergies, asthma, allergic rhinitis, and polyposis.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Characterized by three phases:<ul><li>Prodromal: asthma, allergic rhinitis, or polyposis.<\/li><li>2nd phase: tissue eosinophilia (pulmonary eosinophilia, esophageal eosinophilia)<\/li><li>3rd phase: generalized symptoms and systemic vasculitis.<\/li><\/ul><\/li><li>Any organ can be affected, with respiratory tract involvement beign typical (alveolar hemorrhage, hemoptysis), along with:<ul><li>Mononeuritis multiplex (very common)<\/li><li>Cutanous lesions (palpable cutanous purpura)<\/li><li>GI (pain and bleeding)<\/li><li>The kidneys are less commonly affected<\/li><\/ul><\/li><li>Compared with other small-vessel vasculitides, cardiac involvement is common, and is the most common cause of death.<\/li><li>Diagnosis: <ul><li>EULAR\/ACR scoring system based on the symptoms.<\/li><li>Labwork (ESR, CRP), urinalysis<\/li><\/ul><ul><li>ECG and echocardiography<\/li><li>Serology (<strong>p-ANCA<\/strong>)<\/li><li>Biopsy<\/li><\/ul><\/li><li>Treatment: Mostly corticosteroids; DMARDs may be added.<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_6\">\n<h4 class=\"wp-block-heading\" id=\"microscopic-polyangiitis\" class=\"wp-block-heading\" id=\"microscopic-polyangiitis\" class=\"title_collection title3\">Microscopic polyangiitis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_6\">\n\n\n<p class=\"wp-block-paragraph\">Microscopic polyangiitis resembles granulomatosis with polyangiitis, except that there are no granulomatous lesions and there&#8217;s less upper-respiratory-tract involvement.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>All of the lesions are of the same age (unline PAN).<\/li><li>Initially, general symptoms (fever, malaise); the affected organs are:<ul><li>Kidneys (hematuria and proteinuria)<\/li><li>Cutanous lesions (palpable cutanous purpura)<\/li><li>Alveolar hemorrhage and hemoptysis (although less than in granulomatosis with polyangiitis)<\/li><li>GI (pain and bleeding)<\/li><li>Mononeuritis multiplex<\/li><\/ul><\/li><li>The diagnosis is based on the clinical findings and labwork (ESR, CRP), urinalysis, serology (<strong>p-ANCA<\/strong>), and biopsy.<\/li><li>Treated with high-dose corticosteroids with methotrexate, and if severe, add cyclophosphamide or rituximab.<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_7\">\n<h3 class=\"wp-block-heading\" id=\"immune-complex-vasculitis\" class=\"wp-block-heading\" id=\"immune-complex-vasculitis\" class=\"title_collection title2\">Immune complex vasculitis<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_7\">\n\n\n<p class=\"wp-block-paragraph\">Appears in case of immune complex deposition within the vessel walls.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li><strong>IgA vasculitis (Henoch-Sch\u00f6nlein purpura) <\/strong>occurs due to <strong>IgA1 deposition<\/strong> in vessels.<\/li><li><strong>Goodpasture syndrome<\/strong> is a triad of glomerulonephritis with pulmonary hemorrhage and <strong>anti-GBM antibodies<\/strong> typically appearing after a RTI. <\/li><li><strong>Essential cryoglobulinemic vasculitis <\/strong>occurs in case of abnormal production of antibodies known as cryoglobulins <\/li><\/ul>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h2 class=\"wp-block-heading\" id=\"mechanisms\" class=\"wp-block-heading\" id=\"mechanisms\">Mechanisms<\/h2><h2 class=\"wp-block-heading\" id=\"small-vessel-vasculitides\" class=\"wp-block-heading\" id=\"small-vessel-vasculitides\">Small vessel vasculitides<\/h2><h3 class=\"wp-block-heading\" id=\"anca-associated-vasculitides\" class=\"wp-block-heading\" id=\"anca-associated-vasculitides\">ANCA-associated vasculitides<\/h3><h4 class=\"wp-block-heading\" id=\"granulomatosis-with-polyangiitis-gpa\" class=\"wp-block-heading\" id=\"granulomatosis-with-polyangiitis-gpa\">Granulomatosis with polyangiitis (GPA)<\/h4><h4 class=\"wp-block-heading\" id=\"eosinophilic-granulomatosis-with-polyangiitis-egpa\" class=\"wp-block-heading\" id=\"eosinophilic-granulomatosis-with-polyangiitis-egpa\">Eosinophilic granulomatosis with polyangiitis (EGPA)<\/h4><h4 class=\"wp-block-heading\" id=\"microscopic-polyangiitis\" class=\"wp-block-heading\" id=\"microscopic-polyangiitis\">Microscopic polyangiitis<\/h4><h3 class=\"wp-block-heading\" id=\"immune-complex-vasculitis\" class=\"wp-block-heading\" id=\"immune-complex-vasculitis\">Immune complex vasculitis<\/h3><\/div>","protected":false},"excerpt":{"rendered":"<p>Vasculitis is essentially an inflammation of the vessel wall. It can be either infectious or non-infectious (autoimmune). Generally, &#8220;vasculitis&#8221; is often mentioned on its own, referring to the latter (non-infectious) form. It is classified based on the vessel size (large vessels such as the aorta and its branches, medium vessels, and small vessels). Mechanisms Immune [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":9306,"menu_order":11,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-9330","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>Small vessel vasculitides (classification, pathomechanism, symptoms, treatment) &#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\/immunology\/small-vessel-vasculitides-classification-pathomechanism-symptoms-treatment\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"3 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\\\/immunology\\\/small-vessel-vasculitides-classification-pathomechanism-symptoms-treatment\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/immunology\\\/small-vessel-vasculitides-classification-pathomechanism-symptoms-treatment\\\/\",\"name\":\"Small vessel vasculitides (classification, pathomechanism, symptoms, treatment) &#8211; 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