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A. Characteristics [3] navigator

  1. Chronic inflammatory disease primarily of aorta and its branches
  2. Granulomatous changes in media and adventitia of vessels
  3. Progression to sclerosis over time
    1. Intimal hyperplasia
    2. Medial degeneration
    3. Adventitial fibrosis
    4. Stenoses more common than frank occlusion
  4. Aorta and its major branches have segmental narrowings
  5. Weakened vessels may also dilate to form aneurysms
  6. Therefore, most patients have both segmental narrowings (stenoses) and aneurysms
  7. Epidemiology
    1. Female : Male >10:1
    2. More common in Asian patients than other races
    3. Most patients are ages 10-40 (mean ~25 years)
    4. Incidence in USA is 2.6 cases per million annually
  8. May be related to chronic periaortitis [8]

B. Criteria for Classification navigator

  1. Age of disease onset <40 years
  2. Extremity claudication - upper usually more common tha lower extremities
  3. Brachial artery pulse(s) decreased
  4. BP difference >10mm Hg between arms
  5. Bruit over subclavian arteries or abdominal aorta
  6. Arteriographic narrowing or occlusion (usually segmental)
    1. Entire aorta and/or Primary Branches of the Aorta
    2. Large arteries in the proximal extremities (upper or lower)
    3. Rule out atherosclerotic or fibromuscular dysplasia
  7. At least 3 of the above 6 criteria must be present; Sensitivity 90%, Specificity 98% [4]
  8. Radiographic Classification
    1. Type I: Pulseless, stenosis of aortic arch and its branches
    2. Type II: Mixed, stenosis of aortic arch, its branches, and thoracoabdominal aorta
    3. Type III: Atypical coarctation, stenosis of descending thoracic and abdominal aortas
    4. Type IV: Dilated, aneurysmal dilatation of vessels

C. Presentationnavigator

  1. Arthralgias, Mild/Transient Arthritis (50% of patients)
  2. Headache
  3. Systemic (<30%)
    1. Fatigue
    2. Weight Loss
    3. Low Grade Fever
  4. Pulseless Phase
    1. Vascular insufficiency, late disease
    2. Tenderness with decreased pulses and the presence of bruits
    3. Cold extremities
  5. Claudication is common due to involvement of aorta or major branches (>90% of cases)
  6. Cranial nerve involvement
  7. Hypertension (~50% of patients)
  8. Patients may present with (premature) coronary artery disease [5,6]
  9. ~20% of patients do not require therapy, that is, they have self limiting disease

D. Laboratory Datanavigator

  1. ESR >50mm/hr in 57% of patients (~37% specific for diagnosis)
  2. Arteriographic data
  3. Thrombocytosis
  4. Leukocytosis
  5. Decreased Albumin
  6. Increased Globulin and Fibrinogen
  7. Radiographic classification as above

E. Treatment navigator

  1. Salicylates in high dose may improve symptoms
  2. High dose Glucocorticoids
    1. Pulse 125-1000mg iv qd methylprednisolone for initial improvement
    2. Then prednisone 45-60mg po qd
    3. Low dose, long term (qod dosing not tested)
    4. Disease resistant to high dose glucocorticoids requires second line agents
    5. Response to glucocorticoids usually within days
  3. Second Line Agents
    1. Methotrexate - usually require 15-25mg/wk as steroid sparing agent
    2. Cyclophosphamide -usually 2mg/kg po qd
    3. Azathioprine - 2-4mg/kg as steroid sparing agent
    4. Cyclosporine
    5. Mycophenolate - 1gm po bid showed efficacy in 3 patients resistant to other drugs [7]
  4. Surgery: vascular bypass [6]
  5. Response rate
    1. ~75% with medical therapy
    2. ~50% of responders relapsing later
  6. Side Effects of Medical Therapy
    1. Infections - especially Varicella Zoster
    2. Cushingoid Appearance
    3. Cyclophosphamide induced hemorrhagic cystitis
    4. Methotrexate associated hepatotoxicity with increased transaminase
    5. All cytotoxic agents can induce neutropenia, anemia, thrombocytopenia

References navigator

  1. Rigby WFC, Fan CM, Mark EJ. 2002. NEJM. 347(25):2057 (Case Record) abstract
  2. Weyand CM and Goronzy JJ. 2003. NEJM. 349(2):160 abstract
  3. Kerr GS, Hallahan CW, Giordano J, et al. 1994. Ann Intern Med. 120(11):919 abstract
  4. Arend WP, Michel BA, Bloch DA, et al. 1990. Arthritis Rheum. 33(8):1129 abstract
  5. Vlahakes GJ, Hanna GJ, Marks EJ. 1998. NEJM. 338(13):897 (Case Record)
  6. Levitsky S and Nickeleit V. 1995. NEJM. 332(6):380
  7. Daina E, Schieppati A, Remuzzi G. 1999. Ann Intern Med. 130(5):422 abstract
  8. Vaglio A, Corradi D, Manenti L, et al. 2003. Am J Med. 114(6):454 abstract