HistoryThis section has been translated automatically.
Yamamoto 1830; Takayasu 1908
DefinitionThis section has been translated automatically.
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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Occurrence/EpidemiologyThis section has been translated automatically.
EtiopathogenesisThis section has been translated automatically.
ManifestationThis section has been translated automatically.
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 featuresThis section has been translated automatically.
- Beginning with unspecific general symptoms such as headaches, night sweats, weight loss, recurrent fever, weight loss, myalgias, arthralgias or arthritis.
- Signs of disease progression are circulatory disorders in the upper half of the body (RR differences between right and left). Infection of carotides (40%), A. subclavia (85%), A. ophthalmica (visual disorders 50%), A. renalis (renovascular hypertension) and arteries of the lower extremities (10%). Infestation of the extremity arteries leads to Raynaud's syndrome.
- Although manifestation as "large vessel" vasculitis, dermatological symptoms of"small vessel" vasculitis occur in 15-20% of cases, which can be interpreted as a partial manifestation of the disease (see Rocha LK et al. 2013).
- In the early stages of the disease:
- In later stages:
ImagingThis section has been translated automatically.
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.
LaboratoryThis section has been translated automatically.
DiagnosisThis section has been translated automatically.
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.
TherapyThis section has been translated automatically.
- 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.
Progression/forecastThis section has been translated automatically.
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.
LiteratureThis section has been translated automatically.
- Dagan O et al (1995) Pyoderma gangrenosum and sterile multifocal osteomyelitis preceding the appearance of Takayasu arteritis. Pediatric Dermatol 12: 39-42
- Ohta Y et al (2003) Inflammatory diseases associated with Takayasu's arteritis. Angiology 54: 339-344
- Pascual-Lopez M et al (2004) Takayasu's Disease with Cutaneous Involvement. Dermatology 208: 10-15
- Reichman N et al (1998) Purpura--an unusual presentation of Takayasu arteritis. Harefuah 135: 197-198
- Rocha LK et al (2013) The Journal of Rheumatology 40: 734-738
- 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
- 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
- Schapiro JM et al (1994) Sarcoidosis as the initial manifestation of Takayasu's arteritis. J Med 25: 121-128
- Skaria AM et al (2000) Takayasu arteritis and cutaneous necrotizing vasculitis. Dermatology 200: 139-143
- Takayasu M (1908) A case of strange anastomosis of the central vessels of the retina. J Jap Ophthalm Soc 12: 554
- Yago T et al (2002) A case of systemic sclerosis complicated by Takayasu's arteritis. Ryumachi 42: 605-609
- Yamamoto R (1830) Kitsuo-Idan
Incoming links (8)Autoimmune diseases; Eosinophilic granulomatosis with polyangiitis; Giant cell arteritis; Renovascular hypertension; Takayasu disease; Takayasu-ohnishi syndrome; Takayasu`s arteritis; Takayasu syndrome;
Outgoing links (12)Cyclophosphamide; Erythema nodosum; Giant cell arteritis; Glucocorticosteroids; Nodular vasculitis; Non steroidal anti-inflammatory drugs; Pyoderma gangraenosum; Raynaud's syndrome; Sarcoidosis of the skin; Scleroderma systemic; ... Show all
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