Takayasu Arteritis



Takayasu Arteritis


Surya V. Seshan, MD





Key Facts


Terminology



  • Granulomatous inflammation of aorta and its major branches


Etiology/Pathogenesis



  • Cell-mediated immune mechanism


Clinical Issues



  • Common in Asia (6/1,000) compared with USA (1-3/1,000)


  • Patients usually < 30 years (90%)


  • Pain of involved vessels and bruits


  • Renovascular hypertension


  • Steroid therapy as primary form


  • Complications mainly due to hypertension & stroke


Image Findings



  • Disease classified by abnormalities of aorta or main branches using angiography (“gold standard”)


  • Aneurysmal dilatation may alternate with stenosis


Microscopic Pathology



  • Granulomatous panarteritis with variable number of multinucleated giant cells


  • Multifocal disruption of the medial elastic fibers


  • Adventitial and marked intimal thickening


Top Differential Diagnoses



  • Isolated granulomatous aortitis


  • Giant cell arteritis


  • Atherosclerosis


  • Fibromuscular dysplasia







Segment of aortic wall in Takayasu arteritis (TA) shows adventitia image and a band of medial granulomatous inflammatory infiltrate image.






Coronal MR angiogram of the great vessels shows occlusion of the proximal subclavian artery image and irregular diffuse narrowing image of the left common carotid artery.


TERMINOLOGY


Abbreviations



  • Takayasu arteritis (TA)


Synonyms



  • Takayasu disease/syndrome


  • Pulseless disease


  • Obliterative arteritis


  • Aortoarteritis


  • Idiopathic medial aortopathy and arteriopathy


Definitions



  • Chapel Hill Consensus Conference



    • TA is granulomatous inflammation of aorta and its major branches


    • Usually occurs in patients < 50 years of age


  • American College of Rheumatology criteria



    • Age ≤ 40 years


    • Claudication of an extremity


    • Decreased brachial artery pulse


    • Systolic blood pressure difference > 10 mmHg (between arms)


    • Bruit over subclavian arteries or aorta


    • Angiographic abnormalities (narrowing or occlusion)


    • Any 3 of the above fulfills requirement for diagnosis of TA with high sensitivity and specificity


  • Definitive diagnosis may be problematic and delayed due to slow evolution and low activity of the disease


ETIOLOGY/PATHOGENESIS


Etiology



  • Unknown, autoimmune disease


Genetic



  • Possible link to various HLA subtypes, particularly HLA-B22


  • HLA-BW52 antigen (associated with Crohn disease) is observed in 44% of Japanese patients with TA


Pathogenesis



  • Cell-mediated immune mechanism


  • Not associated with autoantibodies or immune complexes


  • T-lymphocytes, macrophages, antigen presenting dendritic cells, and B-lymphocytes


  • Inflammation causes aortic and arterial wall damage


  • Crohn disease shows similar mural granulomatous inflammation


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare: 1-3/106 per year in USA and UK


    • Said to be more common in Asia but may just be more commonly recognized


    • Japan: Higher aortic arch involvement


    • India: Higher thoracic and abdominal aorta


    • USA: Higher great vessel involvement


  • Age



    • Patients usually < 30 years (90%)


    • Common in 2nd and 3rd decades of life


  • Gender



    • Predominantly female (M:F = 1:8)


  • Ethnicity



    • Pattern of disease varies by geographic area


Presentation



  • 2 phases of disease



    • Early inflammatory phase



      • Constitutional symptoms of fever, myalgias, arthralgias, weight loss, anemia


      • Pain of involved vessels


      • Hypertension


      • Bruits over great vessels


      • Aortic valve insufficiency



      • Ischemic effects: Stroke, claudication, mesenteric ischemia


    • Late occlusive or pulseless phase


    • May coexist with other autoimmune diseases (≤ 10%)



      • Rheumatoid arthritis


      • Systemic lupus erythematosus


      • Inflammatory bowel disease (Crohn disease)


    • Initial nonspecific symptomatology may delay definitive diagnosis for months or years


    • Renal



      • Renovascular hypertension (60%) due to obstructive abdominal aortic disease involving renal artery ostia


      • Renal artery stenosis


      • Evidence of glomerular disease (hematuria, proteinuria), anecdotal


      • Progressive renal insufficiency


Treatment



  • Steroid therapy as primary form


  • Addition of other immunosuppressants or methotrexate in severe/refractory cases or relapse


  • Angioplasty or surgical bypass when disease activity is quiescent


  • Renal lesions according to the type and frequency of specific lesion


Prognosis

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Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Takayasu Arteritis

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