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.
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:
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 (11)Autoimmune diseases; Eosinophilic granulomatosis with polyangiitis; Giant cell arteritis; IL12B Gene; MICA gene; Renovascular hypertension; Takayasu disease; Takayasu-ohnishi syndrome; Takayasu`s arteritis; Takayasu syndrome; ... Show all
Outgoing links (13)Cyclophosphamide; Erythema nodosum; Giant cell arteritis; Glucocorticosteroids; IL12B Gene; Nodular vasculitis; Non steroidal anti-inflammatory drugs; Pyoderma gangraenosum; Raynaud's syndrome; Sarcoidosis of the skin; ... Show all
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