Takayasu arteritis M31.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 02.04.2022

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inflammatory aortic arch syndrome; pulseless disease; Takayasu Disease; Takayasu-Ohnishi Syndrome; Takayasu`s arteritis; Takayasu Syndrome

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Yamamoto 1830; Takayasu 1908

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Takayasu's arteritis is a rare, recurrent, inflammatory large vessel vasculitis ("large vessel" vasculitis; aorta and its branches), which in histological examination has the characteristics of a giant cell arteritis.

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Worldwide distribution with emphasis on East Asia (Japan, China, Korea and also India). Incidence: 2.6/1,000,000 people.

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Unknown, autoimmune disease? Association with rheumatoid arthritis and other autoimmune diseases.

Polymorphisms with the IL12B gene are associated with this arteritis.

Unresolved are associations with sarcoidosis and systemic scleroderma.

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Mainly occurring in women aged 15-35 years. w:m=9:1. In Asian ethnic groups the disease is one of the third most common forms of vasculitis in children.

Incidence in Europe/North America:<1/100,000 per year.

Clinical features
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Onset with non-specific general symptoms such as headache, night sweats, weight loss, recurrent fever, weight loss, myalgias, arthralgias or arthritides.

Signs of disease progression are circulatory disturbances in the upper half of the body (RR differences between right and left). Involvement of carotids (40%), subclavian artery (85%), ophthalmic artery (visual disturbances 50%), renal artery (renovascular hypertension) and arteries of the lower extremities (10%). Involvement of the extrastremity arteries leads to Raynaud's syndrome.

Although manifestation as "large vessel" vasculitis, dermatologic symptoms of"small vessel" vasculitis occur in 15-20% of cases and should be interpreted as partial manifestation of the disease (see Rocha LK et al. 2013).

In the early stage of the disease:

In later stages:

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Color duplex ultrasonography: Concentric, echo-poor wall thickening with halo ("macaroni phenomenon") in arterial cross-section.

CT or MR angiography; possibly PET with fluorodecyglucose to assess disease activity.

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Non-specific inflammatory constellation (acute phase reaction: increased BSG > 50, increase in CRP, fibrinogen, gamma globulins) thrombocytosis, anemia, leukocytosis. ANA and ANCA are negative!

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Takayasu Arteriitis: ACR Classification Criteria (1990)

  • Age at onset of disease <40 years
  • Claudicatio intermittens of the upper/lower extremity
  • Pulse weakening/pulselessness of the brachial artery (pulseless disease)
  • blood pressure difference >10mm Hg between both arms
  • Vascular sounds over other vessels (e.g. A.Subclavia, aorta)
  • Pathological angiogram without signs of arteriosclerosis or fibromuscular dysplasia
  • The occurrence of =/>3 criteria has a specificity of 98% for the diagnosis TA.

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  • The therapeutic goal is the reduction of vascular wall inflammation. The humoral symptoms of inflammation are considered as indicators.
  • Glucocorticoids: Prednisone equivalents in an initial dosage of 1.0-1.5 mg/kg bw for 7-14 days, then reduction by 10 mg/day up to a dosage of 25-40 mg/day (duration 4 weeks). Further reduction by 5 mg/week up to a maintenance dose of < 10 mg/day p.o. Total therapy duration: 1 year. Subsequent therapy depending on the clinic (acute-phase reaction as an indicator of inflammatory symptoms).
  • Non-steroidal anti-inflammatory drugs: Supplementary to therapy with glucocorticoids NSAIDs in medium dosage.
  • If this therapy regime is not sufficient (recurrences during treatment), an additive therapy with cyclophosphamide (endoxane) 2 mg/day/kg bw according to the standard Fauci scheme is necessary. About 4% (!) of glucocorticoid-resistant patients can still benefit from this therapy.
  • Alternative: Intensified Fauci scheme: prednisolone 1 mg/kg bw/day every 8 hours in combination with cyclophosphamide (endoxane) 1-2 mg/kg bw/day.
  • In severe cases: Austin scheme (pulse therapy): 1 g prednisolone i.v. and 15 mg cyclophosphamide/kg bw i.v. every 2 weeks for 6 weeks, later every 4 weeks. Therapy period over 9-12 months.
  • Once the chronic scar stage is reached, the question of lumen-opening therapy measures must be discussed according to the individual case.

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Quoad vitam unfavorable. Five-year mortality rate is about 50%. Most frequent causes of death: neurological (insult) or cardiac (valve insufficiency, coronary heart disease, heart attack) complications.

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  1. Dagan O et al (1995) Pyoderma gangrenosum and sterile multifocal osteomyelitis preceding the appearance of Takayasu arteritis. Pediatric Dermatol 12: 39-42
  2. Ohta Y et al (2003) Inflammatory diseases associated with Takayasu's arteritis. Angiology 54: 339-344
  3. Pascual-Lopez M et al (2004) Takayasu's Disease with Cutaneous Involvement. Dermatology 208: 10-15
  4. Reichman N et al (1998) Purpura--an unusual presentation of Takayasu arteritis. Harefuah 135: 197-198
  5. Rocha LK et al (2013) The Journal of Rheumatology 40: 734-738
  6. Savory WS (1856) Case of a young woman in whom the main arteries of both upper extremities and of the left side of the neck were throughout completely obliterated. Med Chir Trans London 39: 205-219
  7. Savory WS (1856) Royal Medical and Chirurgical Society on a case of young woman in whom the main arteries of both upper extremities and of the left side of the neck were throughout completely obliterated. Lancet 5: 373
  8. Schapiro JM et al (1994) Sarcoidosis as the initial manifestation of Takayasu's arteritis. J Med 25: 121-128
  9. Skaria AM et al (2000) Takayasu arteritis and cutaneous necrotizing vasculitis. Dermatology 200: 139-143
  10. Takayasu M (1908) A case of strange anastomosis of the central vessels of the retina. J Jap Ophthalm Soc 12: 554
  11. Yago T et al (2002) A case of systemic sclerosis complicated by Takayasu's arteritis. Ryumachi 42: 605-609
  12. Yamamoto R (1830) Kitsuo-Idan


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Last updated on: 02.04.2022