Takayasu arteritis

Updated: May 6, 2025

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Background

Takayasu arteritis, also known as “pulseless disease,” is a rare condition that causes long-term inflammation in large and medium-sized arteries, especially those branching off the aorta. It often starts with general symptoms like fever, tiredness, weight loss, and loss of appetite. As the disease progresses, it can cause problems like high blood pressure, weakness or pain in the arms during activity, and symptoms related to reduced blood flow to the brain. These occur due to narrowing or blockage of affected arteries. Diagnosing and treating Takayasu arteritis requires a team approach, involving doctors from different specialties to help manage the condition and improve outcomes.

Epidemiology

Takayasu arteritis is quite uncommon, affecting only 1 to 2 people per million worldwide. It is seen much more often in women than men, with about 90% of cases occurring in females. Most patients are between 40 and 50 years old, though the disease can appear in children and older adults as well. It tends to be more frequent in people of Asian or Mexican origin and is rarely seen in North America.

Anatomy

Pathophysiology

Although the exact cause is not fully understood, Takayasu arteritis is known to involve inflammation in the walls of large arteries. Over time, this inflammation can damage all layers of the artery wall, causing it to thicken, scar, or even form weak spots (aneurysms). These changes can reduce or block blood flow, leading to symptoms in different parts of the body depending on which arteries are involved. The immune system particularly certain white blood cells like CD4+ and CD8+ T cells appears to play a major role. These cells may help form granulomas (small areas of inflammation) and trigger other immune responses that contribute to ongoing inflammation and scarring. Some genetic factors, such as specific HLA types, may increase the risk, but this connection is still being studied.

Etiology

The exact reason why someone develops Takayasu arteritis isn’t known. It is believed to be an autoimmune condition, where the body’s immune system mistakenly attacks its own blood vessels. This immune response leads to inflammation and thickening of the artery walls, which can restrict blood flow and create areas of damage, including bulging sections called pseudoaneurysms.

Genetics

Prognostic Factors

Ongoing Inflammation

If inflammation continues or comes back even after treatment, the chances of complications increase.
People who still have general symptoms like fever or fatigue or show high levels of inflammatory markers in blood tests (like ESR or CRP), are more likely to have a more active and challenging form of the disease.

Blood Vessel Involvement

When the disease affects many arteries or key vessels such as those supplying the heart, kidneys, or brain, it raises the risk of serious health problems like heart failure, stroke, or kidney damage.
If arteries become severely narrowed or balloon out into aneurysms, there’s a higher chance of life-threatening events like ruptures or blocked blood flow.

Late Diagnosis

Delays in diagnosis often mean worse outcomes because some of the changes become permanent and harder to treat.

Treatment Response

People who improve with standard treatments like steroids or immune-suppressing medications usually have a better outlook.
However, those who do not respond well, or whose symptoms return often, may need stronger medications and usually have a more difficult disease course.

Clinical History

In the early stages of Takayasu arteritis, the condition often presents with vague symptoms such as fever, fatigue, muscle pain, loss of appetite, and unintended weight loss. These signs reflect a general inflammatory response and are not specific to the disease, which can make early diagnosis difficult. Most individuals do not seek medical attention until they experience symptoms caused by reduced blood flow, marking the so-called “pulseless” phase. These later signs may include high blood pressure due to narrowing of the renal arteries, neurological issues from reduced blood supply to the brain, or arm pain during activity due to narrowing of arteries in the upper limbs. Because early symptoms are non-specific, diagnosis is frequently delayed. In more advanced stages, patients may develop severe circulation problems in the arms or legs, leading to weak or absent pulses hence the term “pulseless disease” and sometimes a whooshing sound over the affected arteries (vascular bruits). Other later complications can include rapid progression of atherosclerosis or even heart failure.

Age group

Takayasu arteritis most commonly affects young women, typically between the ages of 10 and 40. The highest incidence is observed during the second and third decades of life, it often begins in the teenage years through the 30s. Although the condition can occur in any population, it is more frequently seen in females and is particularly prevalent among individuals of Asian descent.

Physical Examination

General Examination

Neurological Exam

Cardiovascular Exam

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Takayasu arteritis can have a variable acuity of presentation. It often begins with a nonspecific systemic phase (fever, fatigue, weight loss, arthralgia), which may last weeks to months, this is the early or “pre-pulseless” phase. The disease may then progress to a more chronic phase characterized by vascular insufficiency symptoms (e.g., limb claudication, absent pulses, bruits, hypertension), due to stenosis or occlusion of large vessels. Some patients may present acutely with vascular complications, such as stroke, myocardial infarction, or critical limb ischemia, though this is less common.

Differential Diagnoses

Sarcoidosis

Rheumatoid arthritis

Kawasaki disease

Ig-G4 related disease

Giant cell arteritis

Behcet disease

Atherosclerosis

Aortic coarctation

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

The initial treatment for symptomatic Takayasu arteritis typically involves corticosteroids. In some cases, immunosuppressive drugs may be used alone or alongside corticosteroids. However, research has not consistently shown a clear benefit of these medications over corticosteroids alone.

Revascularization procedures are generally acknowledged as effective for addressing vascular issues caused by this disease. However, due to the uncertain nature of Takayasu arteritis, there has historically been hesitation to adopt primary surgical interventions. Surgery has traditionally been reserved for cases where arterial blockages do not respond to corticosteroids or immunosuppressive treatments. Recently, however, there has been a shift in this approach as the effectiveness of medical treatments in halting the progression of the disease has come into question. A recent study indicated that 50% of individuals with Takayasu arteritis will experience a relapse and face vascular complications within 10 years of diagnosis. Men and patients with elevated C-reactive protein levels are at higher risk of relapse.

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

lifestyle-modifications-in-treating-takayasu-arteritis

Lifestyle Adjustments

Exercise: Encourage moderate activity to maintain heart health, avoiding overexertion, especially during flare-ups.

Diet: Eat a heart-healthy, low-salt, low-fat diet with plenty of fruits, vegetables, and lean proteins to manage blood pressure and inflammation.

Smoking: Avoid smoking to improve vascular health and reduce complications.

Managing Stress

Stress Reduction: Promote relaxation through mindfulness, yoga, or counselling to manage chronic stress and reduce inflammation.

Medical Environment

Regular Check-ups: Schedule visits with healthcare providers to monitor disease progression and adjust treatment.

Immunization: Ensure proper vaccinations for patients on immunosuppressive medications to prevent infections.

Environmental Safety

Avoid Extreme Temperatures: Protect from extreme cold or heat to avoid vascular complications.

Air Quality: Maintain a clean air environment to prevent respiratory issues.

Social and Emotional Support

Support Networks: Encourage a positive, supportive social environment to help with emotional well-being.

use-of-corticosteroids-in-treating-takayasu-arteritis

Prednisone: Prednisone is a medication commonly used to treat autoimmune disorders. It helps reduce inflammation by making blood vessels less leaky and lowering the activity of certain immune cells, helping to manage the disease.

effectiveness-of-immunosuppressants-in-treating-takayasu-arteritis

Rheumatology

Azathioprine: This medicine may be added if steroids aren’t working or if the patient has been using steroids for a long time. Azathioprine works by interfering with the body’s ability to make new DNA and proteins, reducing the number of immune cells that cause inflammation.

Mycophenolate: Mycophenolate works by stopping the growth of immune cells, which helps reduce inflammation. It also prevents the production of antibodies that attack the body. There are two versions of this drug:

Mycophenolate mofetil (CellCept), which turns into the active form inside the body.

Mycophenolic acid (Myfortic), which is coated to protect it from being broken down too quickly in the stomach.

role-of-calcium-channel-blockers-in-treating-takayasu-arteritis

Nifedipine: This drug is often used to treat high blood pressure caused by arteritis (inflammation of the arteries).

use-of-dmards-disease-modifying-anti-rheumatic-drugs-and-tnf-inhibitors-in-treating-takayasu-arteritis

Etanercept: If steroids and other immune-suppressing drugs aren’t working, etanercept may be added. It works by blocking a substance in the body (TNF) that causes inflammation, helping to reduce the immune system’s attack on the body.

role-of-intervention-with-procedure-in-treating-takayasu-arteritis

Surgical revascularization is becoming a more common treatment for Takayasu arteritis. In the past, angioplasty (a procedure to widen arteries) was studied, but it is not very effective for this condition. Because the arteries in Takayasu arteritis become stiff and thickened, angioplasty doesn’t offer long-term benefits.

A study at the Cleveland Clinic found that 78% of patients who had angioplasty experienced restenosis (narrowing of the arteries again) within 3 years, and 93% needed additional procedures later. So, angioplasty should mainly be used as a short-term solution to relieve symptoms, especially if other medical needs need to be addressed first.

Another surgical option is arterial reconstruction, which depends on where the artery damage is located and the patient’s individual anatomy. For major artery repairs, materials like Dacron or PTFE are typically used, while veins from the patient’s leg (saphenous vein) are used for smaller arteries in the arms or kidneys. However, using the patient’s own vein can sometimes lead to complications like aneurysms (swelling of the artery). It’s also important that the areas where the surgery is done are not affected by inflammation, as this can lead to the failure of the graft. A study at the University of Southern California found that kidney artery procedures were the most common, but other surgeries included repairs of the aorta or procedures to improve blood flow to the brain or upper limbs.

role-of-management-in-treating-takayasu-arteritis

Acute Phase: Focuses on controlling inflammation with high-dose corticosteroids and immunosuppressive medications. Biologics may be used for severe cases.

Chronic Phase: Aims to maintain remission with low-dose corticosteroids and continued use of immunosuppressives or biologics. Regular monitoring of disease activity is essential.

Surgical Phase: Involves revascularization procedures (e.g., bypass surgery or angioplasty) for severe arterial blockages or ischemia not controlled by medication.

Long-Term Follow-up: Ongoing monitoring for relapses and complications. Immunosuppressive therapy may be continued, and regular imaging is used to check for vascular issues.

Medication

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Takayasu arteritis

Updated : May 6, 2025

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Takayasu arteritis, also known as “pulseless disease,” is a rare condition that causes long-term inflammation in large and medium-sized arteries, especially those branching off the aorta. It often starts with general symptoms like fever, tiredness, weight loss, and loss of appetite. As the disease progresses, it can cause problems like high blood pressure, weakness or pain in the arms during activity, and symptoms related to reduced blood flow to the brain. These occur due to narrowing or blockage of affected arteries. Diagnosing and treating Takayasu arteritis requires a team approach, involving doctors from different specialties to help manage the condition and improve outcomes.

Takayasu arteritis is quite uncommon, affecting only 1 to 2 people per million worldwide. It is seen much more often in women than men, with about 90% of cases occurring in females. Most patients are between 40 and 50 years old, though the disease can appear in children and older adults as well. It tends to be more frequent in people of Asian or Mexican origin and is rarely seen in North America.

Although the exact cause is not fully understood, Takayasu arteritis is known to involve inflammation in the walls of large arteries. Over time, this inflammation can damage all layers of the artery wall, causing it to thicken, scar, or even form weak spots (aneurysms). These changes can reduce or block blood flow, leading to symptoms in different parts of the body depending on which arteries are involved. The immune system particularly certain white blood cells like CD4+ and CD8+ T cells appears to play a major role. These cells may help form granulomas (small areas of inflammation) and trigger other immune responses that contribute to ongoing inflammation and scarring. Some genetic factors, such as specific HLA types, may increase the risk, but this connection is still being studied.

The exact reason why someone develops Takayasu arteritis isn’t known. It is believed to be an autoimmune condition, where the body’s immune system mistakenly attacks its own blood vessels. This immune response leads to inflammation and thickening of the artery walls, which can restrict blood flow and create areas of damage, including bulging sections called pseudoaneurysms.

Ongoing Inflammation

If inflammation continues or comes back even after treatment, the chances of complications increase.
People who still have general symptoms like fever or fatigue or show high levels of inflammatory markers in blood tests (like ESR or CRP), are more likely to have a more active and challenging form of the disease.

Blood Vessel Involvement

When the disease affects many arteries or key vessels such as those supplying the heart, kidneys, or brain, it raises the risk of serious health problems like heart failure, stroke, or kidney damage.
If arteries become severely narrowed or balloon out into aneurysms, there’s a higher chance of life-threatening events like ruptures or blocked blood flow.

Late Diagnosis

Delays in diagnosis often mean worse outcomes because some of the changes become permanent and harder to treat.

Treatment Response

People who improve with standard treatments like steroids or immune-suppressing medications usually have a better outlook.
However, those who do not respond well, or whose symptoms return often, may need stronger medications and usually have a more difficult disease course.

In the early stages of Takayasu arteritis, the condition often presents with vague symptoms such as fever, fatigue, muscle pain, loss of appetite, and unintended weight loss. These signs reflect a general inflammatory response and are not specific to the disease, which can make early diagnosis difficult. Most individuals do not seek medical attention until they experience symptoms caused by reduced blood flow, marking the so-called “pulseless” phase. These later signs may include high blood pressure due to narrowing of the renal arteries, neurological issues from reduced blood supply to the brain, or arm pain during activity due to narrowing of arteries in the upper limbs. Because early symptoms are non-specific, diagnosis is frequently delayed. In more advanced stages, patients may develop severe circulation problems in the arms or legs, leading to weak or absent pulses hence the term “pulseless disease” and sometimes a whooshing sound over the affected arteries (vascular bruits). Other later complications can include rapid progression of atherosclerosis or even heart failure.

Age group

Takayasu arteritis most commonly affects young women, typically between the ages of 10 and 40. The highest incidence is observed during the second and third decades of life, it often begins in the teenage years through the 30s. Although the condition can occur in any population, it is more frequently seen in females and is particularly prevalent among individuals of Asian descent.

General Examination

Neurological Exam

Cardiovascular Exam

Takayasu arteritis can have a variable acuity of presentation. It often begins with a nonspecific systemic phase (fever, fatigue, weight loss, arthralgia), which may last weeks to months, this is the early or “pre-pulseless” phase. The disease may then progress to a more chronic phase characterized by vascular insufficiency symptoms (e.g., limb claudication, absent pulses, bruits, hypertension), due to stenosis or occlusion of large vessels. Some patients may present acutely with vascular complications, such as stroke, myocardial infarction, or critical limb ischemia, though this is less common.

Sarcoidosis

Rheumatoid arthritis

Kawasaki disease

Ig-G4 related disease

Giant cell arteritis

Behcet disease

Atherosclerosis

Aortic coarctation

The initial treatment for symptomatic Takayasu arteritis typically involves corticosteroids. In some cases, immunosuppressive drugs may be used alone or alongside corticosteroids. However, research has not consistently shown a clear benefit of these medications over corticosteroids alone.

Revascularization procedures are generally acknowledged as effective for addressing vascular issues caused by this disease. However, due to the uncertain nature of Takayasu arteritis, there has historically been hesitation to adopt primary surgical interventions. Surgery has traditionally been reserved for cases where arterial blockages do not respond to corticosteroids or immunosuppressive treatments. Recently, however, there has been a shift in this approach as the effectiveness of medical treatments in halting the progression of the disease has come into question. A recent study indicated that 50% of individuals with Takayasu arteritis will experience a relapse and face vascular complications within 10 years of diagnosis. Men and patients with elevated C-reactive protein levels are at higher risk of relapse.

Rheumatology

Lifestyle Adjustments

Exercise: Encourage moderate activity to maintain heart health, avoiding overexertion, especially during flare-ups.

Diet: Eat a heart-healthy, low-salt, low-fat diet with plenty of fruits, vegetables, and lean proteins to manage blood pressure and inflammation.

Smoking: Avoid smoking to improve vascular health and reduce complications.

Managing Stress

Stress Reduction: Promote relaxation through mindfulness, yoga, or counselling to manage chronic stress and reduce inflammation.

Medical Environment

Regular Check-ups: Schedule visits with healthcare providers to monitor disease progression and adjust treatment.

Immunization: Ensure proper vaccinations for patients on immunosuppressive medications to prevent infections.

Environmental Safety

Avoid Extreme Temperatures: Protect from extreme cold or heat to avoid vascular complications.

Air Quality: Maintain a clean air environment to prevent respiratory issues.

Social and Emotional Support

Support Networks: Encourage a positive, supportive social environment to help with emotional well-being.

Rheumatology

Prednisone: Prednisone is a medication commonly used to treat autoimmune disorders. It helps reduce inflammation by making blood vessels less leaky and lowering the activity of certain immune cells, helping to manage the disease.

Rheumatology

Rheumatology

Azathioprine: This medicine may be added if steroids aren’t working or if the patient has been using steroids for a long time. Azathioprine works by interfering with the body’s ability to make new DNA and proteins, reducing the number of immune cells that cause inflammation.

Mycophenolate: Mycophenolate works by stopping the growth of immune cells, which helps reduce inflammation. It also prevents the production of antibodies that attack the body. There are two versions of this drug:

Mycophenolate mofetil (CellCept), which turns into the active form inside the body.

Mycophenolic acid (Myfortic), which is coated to protect it from being broken down too quickly in the stomach.

Rheumatology

Nifedipine: This drug is often used to treat high blood pressure caused by arteritis (inflammation of the arteries).

Rheumatology

Etanercept: If steroids and other immune-suppressing drugs aren’t working, etanercept may be added. It works by blocking a substance in the body (TNF) that causes inflammation, helping to reduce the immune system’s attack on the body.

Rheumatology

Surgical revascularization is becoming a more common treatment for Takayasu arteritis. In the past, angioplasty (a procedure to widen arteries) was studied, but it is not very effective for this condition. Because the arteries in Takayasu arteritis become stiff and thickened, angioplasty doesn’t offer long-term benefits.

A study at the Cleveland Clinic found that 78% of patients who had angioplasty experienced restenosis (narrowing of the arteries again) within 3 years, and 93% needed additional procedures later. So, angioplasty should mainly be used as a short-term solution to relieve symptoms, especially if other medical needs need to be addressed first.

Another surgical option is arterial reconstruction, which depends on where the artery damage is located and the patient’s individual anatomy. For major artery repairs, materials like Dacron or PTFE are typically used, while veins from the patient’s leg (saphenous vein) are used for smaller arteries in the arms or kidneys. However, using the patient’s own vein can sometimes lead to complications like aneurysms (swelling of the artery). It’s also important that the areas where the surgery is done are not affected by inflammation, as this can lead to the failure of the graft. A study at the University of Southern California found that kidney artery procedures were the most common, but other surgeries included repairs of the aorta or procedures to improve blood flow to the brain or upper limbs.

Rheumatology

Acute Phase: Focuses on controlling inflammation with high-dose corticosteroids and immunosuppressive medications. Biologics may be used for severe cases.

Chronic Phase: Aims to maintain remission with low-dose corticosteroids and continued use of immunosuppressives or biologics. Regular monitoring of disease activity is essential.

Surgical Phase: Involves revascularization procedures (e.g., bypass surgery or angioplasty) for severe arterial blockages or ischemia not controlled by medication.

Long-Term Follow-up: Ongoing monitoring for relapses and complications. Immunosuppressive therapy may be continued, and regular imaging is used to check for vascular issues.

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