1955
An inflammatory large blood vessel vasculitis of unknown origin.
Affects primarily the aorta and its branches.
It can occur worldwide, but it is most commonly observed in Asian women.
It is the most common large vessel vasculitis affecting the aorta among patients younger than 50 years, and it typically occurs in women younger than 40 years of age.
Dermatologic manifestations include erythema nodosum, a lupus like photosensitive malar rash can occur in approximately 20% of patients.
With disease progression symptoms of large vessel vasculitis predominate with carotid artery tenderness, arterial bruits, hypertension, discrepant blood pressure, G.I. symptoms, angina, visual disturbances, and neurologic symptoms, depending on arterial inflammation and stenosis distribution.
In all stages of TA, especially in the late stenotic phase, distant pulses are often asymmetrically diminished or even absent: pulseless disease.
Rare entity in North America with an estimated incidence 1 of 2.6 per million.
in Japan, the prevalence is 40 cases per million.
Inflammation of the blood vessels leads to arterial stenosis, occlusion or dilatation.
A chronic inflammatory disease of the aorta and branch arteries of unknown cause.
Onset is generally subacute or insidious, and disease severity ranges from asymptomatic to catastrophic.
Approximately 1/3 of patience have constitutional or muscular signs and symptoms such as fatigue, fever, weight, loss, myalgias, and arthralgias.
2/3 of patients present with manifestations, associated with arterial involvement, most commonly in the aorta and the subclavian, carotid, and renal arteries, and less often in the vertebral, pulmonary, coronary, mesenteric, and iliofemoral arteries.
Patient often have arm clarification.
Auscultation of the anterior neck and supraclavicular region can reveal bruits, reflecting, arterial stenosis.
It is characterized by a transmural, granulomatous infiltrate.
Cardiac disease is a major cause of complications and death in TA.
There is some degree of aortic regurgitation, in 15 to 50% of patients in primarily occur as a consequence of the separation of the aortic valve leaflets due to aortic root dilatation.
Most patients with TA have non-specific lab abnormalities, reflecting systemic inflammation, including: anemia, elevated ESR, and C reactive protein, and thrombocytosis.
Diagnosis is often delayed and patients present with established vascular damage.
Starts with nonspecific symptoms such as:
Localized joint pain
Fever
Fatigue
Headaches
Rashes
Weight loss
Diagnosis usually does not happen until the blockage causes deficient blood flow to the extremities or to a stroke.
Tissue from large arteries is obtained from only a minority of patients undergoing vascular surgery, therefore vascular imaging is the most frequent test used to establish the diagnosis.
Classification criteria:
Age when disease starts is under 50
Decreased brachial artery pulse
Systolic blood pressure differs by more than 10mmHg between arms
Cramping caused by exercise in the extremities
Abnormal sounds over subclavian arteries or abdominal aorta
A narrowing or blockage in the aorta, its primary branches, or large arteries as seen through a angiogram of the arteries.
The ascending aorta and vessels of the aorta are classically affected, but five categories of aortic involvement have been established, based on angiographic distribution.
Long segments of contiguous involvement, as well as multiple areas of stenosis of frank vessel occlusion are typical findings along aneurysmal formation.
Patients often present with symptoms related to distal hypoperfusion, including arm claudication, syncope, stroke, or diminished or absent pulses on physical examination.
Aortic root aneurysm with secondary aortic regurgitation is a classic complication of TA.
Diagnostic delay is especially present in patients older than 40 years.
Patients often present initially with nonspecific symptoms and laboratory abnormalities.
Arthralgias, fatigue, low-grade fever and elevated inflammatory markers are part of presentation.
The clinical course is of progressive , relapsing and remitting nature despite treatment.
Medical management is to control the inflammation, but surgery may be needed to correct arterial damage.
Also known as aortic arch syndrome, nonspecific aortoarteritis and the pulseless disease
A form of large vessel granulomatous vasculitis with massive intimal fibrosis and vascular narrowing, affecting often young or middle-aged women of Asian descent.
Mainly affects the aorta and its branches, as well as the pulmonary arteries.
Females are about 8–9 times more likely to be affected than males.
Onset of disease symptoms usually between 15 and 30 years of age: often manifests before 40 years of age.
The constitutional symptoms of fatigue, low-grade fever, night sweats, and weight loss are frequent manifestations, occurring up to 65% of patients.
Synovitis is rare, but arthralgias a common affecting approximately 40% of patients.
Prevalence varies widely from 0.9 cases per million in the United States to 40 cases per million in Japan.
Autopsy studies in Japan indicated prevalence as high as one in 3000 suggesting substantial underdiagnosis.
Due to obstruction of the main branches of the aorta, including the left common carotid artery, the brachiocephalic artery, and the left subclavian artery, can present as pulseless upper extremities.
Initially, some patients present with an inflammatory phase characterized by systemic illness with malaise, fever, night sweats, weight loss, arthralgia, and fatigue, anemia and marked elevation of the ESR or C-reactive protein.
Following the systemic symptoms the pulseless phase characterized by vascular insufficiency from intimal narrowing of the vessels manifesting as arm or leg claudication, renal artery stenosis causing hypertension, and neurological manifestations due to decreased blood flow to the brain may occur.
Characterized by cell mediated auto immunity with infiltration of gamma/lambda lymphocytes, dendritic and granulocytes.
Its pathogenesis probably involves an interaction between genetics and environment.
no single variant in the HLA region has been identified
The development of renal artery stenosis can cause hypertension by the increased secretion of renin, and high blood levels of aldosterone.
The neurological symptoms of the disease can range from lightheadedness, to seizures in severe cases.
Ocular involvement rarely can occur in form of visual field defects, vision loss, or retinal hemorrhage.
Some patients present with only late vascular changes, without a pre existing systemic illness.
Late disease may give rise to localized aneurysms.
Raynaud’s phenomenon is common.
Etiology is unknown.
Characterized by segmental and patchy granulomatous inflammation of the aorta and its major branches.
Inflammation leads to arterial stenosis, thrombosis, and aneurysms.
Associated with fibrosis of the blood vessels due to chronic vasculitis, leading to intimal fibrosis.
Narrowing secondary to inflammation, granuloma, and fibrosis may be seen in arterial studies.
Genetic relationship supported by the association with HLA-B∗52.
There are multiple additional susceptibility loci for this disease across the genome.
The blood vessel malformations that occur in the retina are an angiogenic response to the arterial narrowings in the neck, and that the absence of pulses noted in some patients occurs because of narrowings of the blood vessels to the arms.
The eye findings are rarely seen in patients from North America.
Diagnostic criteria: include the presence of at least three of six features- age of onset of less than 40 years, claudication, decreased brachial artery pulse, blood pressure difference of more than 10 mmHg between alarms, subclavian for aortic bruit, any abnormal aortic imaging.
The criteria is reported to be 90.5% sensitive and 97.8% specific for Takayasu’s arteritis.
About 13 to 17.5% of patients receive a diagnosis after age of 40 years.
Aortic regurgitation complicates approximately 25% of cases, probably due to malcoptation due the dilitation of the aortic root.
Aortic regurgitation may have other findings include in bounding pulse with rapid systolic rising and diastolic collapse, barbing of the head, repeated flushing and blanching of the capillary nail beds.
Aortic regurgitation, if left untreated, results in less ventricular dilation, systolic heart failure, ultimately death.
Treatment:
Majority patients respond to steroids.
Surgical options exist for patients who do not respond to steroids and include reconstructive surgery.
Surgical procedures are performed in approximately 50% of patients, and can be life-saving with severe, aortic regurgitation, aortic 23\aneurysms, or aortic dissection.
Other agents include azothioprine, lefunomide, methotrexate, mycophenolate mofetil, or cyclophosphamide.
Tocilizumab, a monoclonal antibody against interleukin-6 receptor has proven efficacious.
In Takayasu’s arteritis it is vital to combine drug treatments often with low-dose aspirin or a statin.