Takayasu's arteritis; aortic arch syndrome; pulseless disease
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Introduction
Inflammatory & stenotic disease of medium & large sized arteries with strong predilection for the aortic arch & its branches. The pulmonary artery may also be involved.
Etiology
- autoimmune suspected, circulating immune complexes detected
- unknown
Epidemiology
- uncommon, much less common than temporal arteritis
- most common in adolescent girls & young women
- more common in Asians (young Asian women)
Pathology
- chronic nonspecific granulomatous vasculitis affecting aorta & its main branches, coronary & pulmonary arteries[4][5][10]
- panarteritis with mononuclear infiltrates & occasional giant cells
- marked intimal proliferation & fibrosis
- scarring & vascularization of the media
- disruption & degeneration of the elastic lamina
- narrowing of the lumen with or without thrombosis
- involvement of vaso vasorium
- systemic effects secondary to compromised blood flow
- pulmonary abnormalities begin in upper lobes, with middle & lower lobe involvement in later stages of the disease
- ischemia may occur distal to stenotic vessel[4]
Genetics
Clinical manifestations
- general
- non-specific symptoms may be present months prior to manifestations of arterial involvement
- pain over involved vessel
- arterial bruit over involved vessel
- abdominal bruit (renal artery stenosis)[4]
- disease may be fulminant, slowly progressive or may stabilize or remit
- vascular bruits & diminished pulses are the most reliable signs
- seldom causes skin disease[5]
- case report of woman with skin lesions[11]
- manifestations secondary to specific artery involvement
- subclavian artery (93%)
- Raynaud's phenomenon
- claudication of arm
- asymmetric blood pressure[5]
- common carotid artery (58%)
- abdominal aorta (aortitis) (47%)
- generally asymptomatic
- abdominal pain
- nausea/vomiting
- renal artery stenosis (38%)
- aortic arch or aortic root aortitis (35%)
- vertebral artery (35%)
- celiac artery or superior mesenteric artery (18%)
- generally asymptomatic
- abdominal pain
- nausea/vomiting
- iliac artery (17%)
- pulmonary artery (10-40%[2], >50%[3])
- coronary arteries (<10%)
- subclavian artery (93%)
Diagnostic criteria
- age of onset < 40 years of age
- claudication of the extremities, especially the arms
- decreased pulsation in one or both brachial arteries
- difference of at least 10 mm Hg systolic blood pressure between the two arms
- bruit over one or both subclavian arteries or over the abdominal aorta
- arteriography (angiography) showing narrowing or occlusion of the entire aorta, its primary branches or large arteries of the extremities (brachial artery, femoral artery) not due to atherosclerosis or fibromuscular dysplasia[4]
Laboratory
- elevated erythrocyte sedimentation rate (ESR)
- elevated C-reactive protein (CRP)
- complete blood count
- anemia (mild)
- thrombocytosis
- case report with elevated serum alkaline phosphatase
- serum protein electrophoresis: polyclonal gammopathy
- see ARUP consult[6]
Radiology
- angiography (arteriography), CT angiography or MRI angiography showing stenosis & dilatation of the aorta & or its branches (aortography)
- MRI & ultrasound may show thickened aortic wall prior to angiographic changes
- pulmonary angiography reveals arterial occlusion in 86% of patients[3]
Complications
- aortic aneurysm
- aortic regurgitation
- pulmonary artery stenosis or aneurysm
- renovascular hypertension
- death generally occurs from congestive heart failure or stroke
Management
- prognosis
- course of disease variable, spontaneous remissions occur
- mortality 10-75%
- general measures
- correct hypertension
- improve blood flow to affected organs
- pharmacologic agents
- glucocorticoids
- prednisone 40-60 mg QD
- generally symptomatic response
- no evidence glucocorticoids improve survival
- methotrexate
- in patients refractory to glucocorticoids
- may be an effective corticosteroid-sparing agent
- up to 25 mg q week
- azathioprine as an alternative to methotrexate[4]
- TNF inhibitors have been used in refractory cases[10]
- aspirin may be useful to manage symptoms of vascular insufficiency once inflammatory phase has been controlled by glucocorticoids[4]
- glucocorticoids
- angioplasty alleviates renal artery stenosis in 50% of cases
- vascular surgery
- vascular bypass
- aortic valve replacement if needed
More general terms
References
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1677
- ↑ 2.0 2.1 Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 1918
- ↑ 3.0 3.1 3.2 Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 783
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018.
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - ↑ 5.0 5.1 5.2 5.3 5.4 5.5 Khasnis A and Langford CA. Update on vasculitis. J Allergy Clin Immunol 2009 Jun; 123:1226. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19501230
- ↑ 6.0 6.1 ARUP Consult: Takayasu Arteritis The Physician's Guide to Laboratory Test Selection & Interpretation https://arupconsult.com/content/takayasu-arteritis
- ↑ Mason JC. Takayasu arteritis--advances in diagnosis and management. Nat Rev Rheumatol. 2010 Jul;6(7):406-15 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20596053
- ↑ Andrews J, Mason JC. Takayasu's arteritis--recent advances in imaging offer promise. Rheumatology (Oxford). 2007 Jan;46(1):6-15 PMID: https://www.ncbi.nlm.nih.gov/pubmed/17043053
- ↑ Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007 Mar;56(3):1000-9. PMID: https://www.ncbi.nlm.nih.gov/pubmed/17328078
- ↑ 10.0 10.1 10.2 Wen D, Du X, Ma CS. Takayasu arteritis: diagnosis, treatment and prognosis. Int Rev Immunol. 2012 Dec;31(6):462-73. PMID: https://www.ncbi.nlm.nih.gov/pubmed/23215768
- ↑ 11.0 11.1 Mansour MK, Letourneau AR, Wallace ZS et al Case 15-2017 - A 27-Year-Old Woman with Anemia, Thrombocytosis, and Skin Lesions after Travel Abroad. N Engl J Med 2017; 376:1973-1981. May 18, 2017 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28514603 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMcpc1616396
- ↑ de Souza AW, de Carvalho JF. Diagnostic and classification criteria of Takayasu arteritis. J Autoimmun. 2014 Feb-Mar;48-49:79-83. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24461381