{"id":9326,"date":"2021-12-19T00:23:58","date_gmt":"2021-12-18T22:23:58","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/immunology\/large-vessel-vasculitides-polymyalgia-rheumatica-classification-patomechanism-symp-toms-treatment\/"},"modified":"2021-12-24T10:40:23","modified_gmt":"2021-12-24T08:40:23","slug":"large-vessel-vasculitides-polymyalgia-rheumatica-classification-pathomechanism-symptoms-treatment","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/immunology\/large-vessel-vasculitides-polymyalgia-rheumatica-classification-pathomechanism-symptoms-treatment\/","title":{"rendered":"Large vessel vasculitides, Polymyalgia rheumatica (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\" class=\"wp-block-heading\" 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\" class=\"wp-block-heading\" class=\"title_collection title1\">Large vessel vasculitides<\/h2>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<p class=\"wp-block-paragraph\">Both giant cell and Takayasu arteritis are associated with lymphocytic migration and proliferation in the adventitia of vessels, cytokine secretion, macrophage recruitment, and formation of granulomas resulting in the destruction of the vessel wall and its occlusion. <\/p>\n\n\n<\/span><span class=\"block-heading\" id=\"header_3\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Giant cell (temporal) arteritis<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<p class=\"wp-block-paragraph\">Giant cell arteritis (also known as <strong>temporal arteritis<\/strong> or <strong>Horton disease<\/strong>) affects large arteries in the head and neck, typically the occipital or temporal arteries.<\/p>\n\n\n\n<ul class=\"wp-block-list\" id=\"block-108efa30-d5f6-4c85-a902-4d578d20a60d\"><li>May affect other arteries as well (thoracic aorta, branches of the aortic arch, coronaries).<\/li><li>Intracranial involvement may lead to stroke. Ophthalmic artery involvement may lead to blindness. Thoracic aorta involvement may lead to anyeurysms and aortic dissection.<\/li><li>Most common in young females.<\/li><li>Usually comes together with <strong>polymyalgia rheumatica<\/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 title3\">Clinical presentation<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<ul class=\"wp-block-list\"><li>Presents with a severe, throbbing headache<\/li><li>Generalized symptoms (fever, malaise, fatigue, weight-loss, night-sweats)<\/li><li>Jaw claudication<\/li><li>Tongue ulcers<\/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 title3\">Diagnosis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_5\">\n\n\n<ul class=\"wp-block-list\"><li>Physical examination<ul><li>The affected vessels are enlarged, stiffened, tender, sometimes nodular, and &#8220;roll&#8221; under the finger of the examiner (rather than collapsing).<\/li><\/ul><ul><li>Bruits in the large arteries of the limbs and carotids.<\/li><\/ul><\/li><li>Blood panel (ESR, CRP, CBC)<\/li><li>Biopsy (temporal\/occipital artery; should be cut length-wise)<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_6\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title3\">Treatment<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_6\">\n\n\n<ul class=\"wp-block-list\"><li>Corticosteroids<\/li><li>Aspirin<\/li><li>Tocilizumab<\/li><\/ul>\n\n\n\n<p class=\"wp-block-paragraph\">In case of suspicion of giant cell arteritis, the treatment should be started as soon as possible, even before the result of the biopsy.<\/p>\n\n\n<\/span><span class=\"block-heading\" id=\"header_7\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Takayasu arteritis<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_7\">\n\n\n<p class=\"wp-block-paragraph\">Takayasu arteritis affects the aorta, its branches, and the pulmonary arteries.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Characterized by marked stenosis of the affected arteries. The stenosed arteries may occlude or undergo anyeurysm formation.<\/li><li>The T-cell activity is against specific antigens in the wall of the aorta, thought to arise due to molecular mimicry.<\/li><li>Most common in young females of Asian descent.<\/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 title3\">Symptoms<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_8\">\n\n\n<ul class=\"wp-block-list\"><li>Generalized symptoms <\/li><li>Hypoperfusion of the limb will lead to weakness, pain, and fatigue, as well as claudication and diminished pulse of the limb<\/li><li>Pulmonary HTN (in pulmonary artery involvement)<\/li><li>Renovascular HTN (in renal artery involvement)<\/li><li>Angina\/MI (in cardiac involvement)<\/li><li>Subclavian steal syndrome (the bloodflow is &#8220;stolen&#8221; from the vertebral artery into the subclavian artery when raising the arm, due to a stenotic lesion)<\/li><li>Aortic regurgitation and heart failure (in ascending aorta involvement)<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_9\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title3\">Diagnosis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_9\">\n\n\n<ul class=\"wp-block-list\"><li>Physical examination<ul><li>BP measurement of all limbs<\/li><li>Bruits can be heard in large arteries<\/li><li><strong>Ankle-brachial pulse index<\/strong>, normal value 1.0-1.3<\/li><\/ul><\/li><li>CT-angio<\/li><li>MRI<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_10\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title3\">Treatment<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_10\">\n\n\n<ul class=\"wp-block-list\"><li>Corticosteroids (prenisone PO 1mg\/kg\/day for 1-3 months)<\/li><li><span style=\"color: initial;\">DMARDs<\/span><\/li><li>Tocilizumab&nbsp;<\/li><li>Bypass surgeries in severe ischemia<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_11\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Polymyalgia rheumatica<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_11\">\n\n\n<p class=\"wp-block-paragraph\">Polymyalgia rheumatica is an inflammatory syndrome presenting with bilateral hip, thighs, shoulder, and neck pain and stiffness, usually being worst early in the morning.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>The etiology and pathomechanism of polymyalgia rheumaica are unknown.<\/li><li>It may be associated with giant cell arteritis; the conditons often appear together.<\/li><li>Typically appears in older females.<\/li><li><strong>Diagnosis <\/strong>is based on the symptoms, and inflammatory markers:<ul><li>Patient may complain of a difficulty getting up in the morning, bilateral pain and stiffness in the upper &amp; lower limb when trying to dress up and perform daily activities, especially early in the morning.<\/li><li>ESR, CRP may be elevated.<\/li><\/ul><\/li><li><strong>Treatment<\/strong> involves corticosteroids (oral prednisolone 15-20mg\/day); NSAIDs are ineffective.<\/li><\/ul>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h2 class=\"wp-block-heading\" class=\"wp-block-heading\">Mechanisms<\/h2><h2 class=\"wp-block-heading\" class=\"wp-block-heading\">Large vessel vasculitides<\/h2><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Giant cell (temporal) arteritis<\/h3><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Clinical presentation<\/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\">Takayasu arteritis<\/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\">Polymyalgia rheumatica<\/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":9,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-9326","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>Large vessel vasculitides, Polymyalgia rheumatica (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\/large-vessel-vasculitides-polymyalgia-rheumatica-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\\\/large-vessel-vasculitides-polymyalgia-rheumatica-classification-pathomechanism-symptoms-treatment\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/immunology\\\/large-vessel-vasculitides-polymyalgia-rheumatica-classification-pathomechanism-symptoms-treatment\\\/\",\"name\":\"Large vessel vasculitides, Polymyalgia rheumatica (classification, pathomechanism, symptoms, treatment) &#8211; 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