Takayasu arteritis (TA) is a rare, chronic, inflammatory condition that affects the large arteries of the body, particularly the aorta and its main branches. It is named after Dr. Mikito Takayasu, a Japanese ophthalmologist who first described the disease in 1908. Takayasu arteritis primarily affects young to middle-aged women but can also occur in men, children, and the elderly.
The exact cause of the disease is not well understood, but it is believed to involve an abnormal immune response leading to inflammation of the arterial walls. Genetics and environmental factors might also play a role. The disease often progresses slowly, and its symptoms can vary depending on the affected arteries and the extent of inflammation. Early symptoms may be nonspecific, making diagnosis challenging.
Epidemiology
Takayasu arteritis is relatively higher in Asian countries, especially in Japan, India, and Korea. It is less common in Western countries, including the United States and Europe. Takayasu arteritis usually affects young to middle-aged individuals, with most cases diagnosed between 15 and 40. The disease primarily affects females, with a female-to-male ratio of around 8:1, indicating a strong female predisposition.
Certain ethnic groups, particularly individuals of Asian descent, have a higher risk of developing Takayasu arteritis than other racial or ethnic groups. The disease is more prevalent in certain regions with a higher incidence reported in Southeast Asia, South Asia, East Asia, and Latin America. Despite being more common in specific regions, TA is still considered a rare disease worldwide, with an estimated incidence of 1 to 2 cases per million people per year.
Anatomy
Pathophysiology
The pathophysiology of Takayasu arteritis involves chronic inflammation of the large and medium-sized arteries, leading to various changes in the arterial walls. The exact cause is not fully understood, but it is believed to be an autoimmune-mediated process. In TA, the arterial walls become infiltrated with inflammatory cells, including lymphocytes, macrophages, and giant cells.
These inflammatory cells release various cytokines and mediators that perpetuate the inflammatory response. The intima undergoes thickening due to chronic inflammation. This process is known as intimal hyperplasia, narrowing the arterial lumen and reducing blood flow. The middle layer of the artery, the media, is affected by fibrosis. Medial fibrosis can lead to arterial stiffness and compromise the elasticity of the artery.
The reduced blood flow to various organs and tissues can lead to ischemia, resulting in organ dysfunction and damage. The severity of organ involvement varies depending on the affected arteries and the extent of the disease. In response to chronic inflammation and arterial changes, the body may attempt to remodel the affected arteries, leading to a thickened, narrowed, or dilated appearance.
Etiology
Genetic Predisposition: Evidence suggests a genetic component in the development of Takayasu arteritis. Some studies have found associations between specific human leukocyte antigen (HLA) alleles, particularly HLA-B52, and an increased risk of developing TA in certain populations. However, the genetic factors involved in TA are likely complex and involve multiple genes.
Environmental Triggers: Environmental factors may play a role in triggering or exacerbating the disease in individuals with a genetic predisposition. Infections have been proposed as potential triggers for the immune system’s abnormal response, leading to the initiation of TA in susceptible individuals. However, specific infectious agents or environmental triggers have not been consistently identified across all cases.
Hormonal Factors: The female predominance in Takayasu arteritis suggests that hormonal factors may play a role in the disease’s development.
Genetics
Prognostic Factors
Overall, the long-term prognosis for patients with TA has improved over the years with advances in medical management and early detection. With appropriate and timely treatment, many individuals can achieve remission and have a good quality of life.
Clinical History
Clinical History
Takayasu arteritis is a chronic disease, and its onset can be insidious, meaning that symptoms may develop gradually over time. The age of onset is typically younger, with most cases diagnosed in individuals between 15 and 40 years old, although it can occur in people of all ages. Females are affected more commonly than males. The symptoms of Takayasu arteritis can vary widely among individuals and depend on the arteries affected and the extent of inflammation.
Common symptoms and clinical manifestations of TA include fatigue, fever, unexplained weight loss, loss of appetite, muscle and joint pain, headaches, dizziness or fainting spells, hypertension, weak or absent pulses in the limbs, skin changes, such as rashes, nodules, or ulcers, visual disturbances or blindness (if the arteries supplying the eyes are affected). The duration of symptoms can vary greatly from person to person. Some individuals may experience a relatively rapid onset of symptoms.
In contrast, others may have a more indolent course with mild or intermittent symptoms for an extended period before a definitive diagnosis is made. Early symptoms are often nonspecific and can be mistaken for other conditions, leading to delays in diagnosis. Given the chronic and often fluctuating nature of the disease, individuals with Takayasu arteritis may experience periods of remission and disease flare-ups.
Physical Examination
Physical Examination
In Takayasu arteritis, there may be a significant difference in blood pressure readings between the arms. This difference can occur due to the narrowing or occlusion of the arteries supplying the arms. Auscultation of the blood vessels, particularly in the neck, may reveal abnormal sounds called bruits.
Bruits are caused by turbulent blood flow in narrowed arteries and can be a sign of arterial stenosis. Hypertension is common in Takayasu arteritis, particularly if the renal arteries are affected. The femoral arteries in the groin area may have a diminished or weak pulse due to involvement by the disease.
The pulses in the legs, particularly in the popliteal and dorsalis pedis arteries, may be weak or absent. Skin findings can include skin rashes, ulcers, nodules, or color changes (such as livedo reticularis) due to impaired blood flow. Ophthalmic involvement may lead to visual disturbances, such as decreased vision or blindness, and an ophthalmologist may detect changes during an eye examination.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Differential Diagnoses
Atherosclerosis
Aortic coarctation
Behcet disease
Kawasaki disease
Sarcoidosis
Rheumatoid arthritis
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Corticosteroids and Immunosuppressants
The primary goal of treatment is to suppress inflammation, prevent disease progression, and manage complications. Medications such as corticosteroids and immunosuppressants (e.g., methotrexate, azathioprine, or biologic agents) are commonly used to achieve these objectives. In some cases, interventional procedures or surgery may be required to address arterial stenosis or aneurysms.
Surgical Revascularization
Surgical revascularization is emerging as a primary treatment option for Takayasu arteritis, but the role of transluminal angioplasty in managing the condition remains limited. Previous studies have shown that arterial obliteration’s fibrous nature hinders long-term angioplasty benefits. A recent study conducted at the Cleveland Clinic reported that 78% of their patient cohort treated with angioplasty at different arterial sites experienced restenosis over a median 3-year follow-up period. Additionally, 93% of these patients required further reintervention at some point. As a result, percutaneous transluminal angioplasty should be considered a short-term strategy primarily aimed at providing symptomatic relief, especially if other pressing medical needs take precedence in a particular patient.
Arterial Reconstruction
Arterial reconstruction stands as an alternative surgical option for patients diagnosed with Takayasu arteritis. The choice of reconstruction method depends on the location of known lesions and the patient’s individual surgical anatomy. For major aortic reconstructions, Dacron or polytetrafluoroethylene (PTFE) grafts are typically used. In contrast, autogenous saphenous vein grafts are employed for extremity or isolated renal or mesenteric revascularization. However, caution should be exercised when using the saphenous vein due to its known tendency for aneurysmal degeneration.
In the surgical procedure, it is crucial to ensure that both the proximal and distal anastomotic sites are free of any inflammatory involvement. Grafting onto segments of arteries affected by inflammation can lead to early graft failure. A study conducted at the University of Southern California, which involved 40 patients with TA, revealed that renovascular procedures were the most common. Other procedures included various aortic reconstructions, aorta-carotid bypasses for cerebrovascular insufficiency, and bypasses to distal subclavian, axillary, or brachial arteries to address upper extremity ischemia.
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Takayasu arteritis (TA) is a rare, chronic, inflammatory condition that affects the large arteries of the body, particularly the aorta and its main branches. It is named after Dr. Mikito Takayasu, a Japanese ophthalmologist who first described the disease in 1908. Takayasu arteritis primarily affects young to middle-aged women but can also occur in men, children, and the elderly.
The exact cause of the disease is not well understood, but it is believed to involve an abnormal immune response leading to inflammation of the arterial walls. Genetics and environmental factors might also play a role. The disease often progresses slowly, and its symptoms can vary depending on the affected arteries and the extent of inflammation. Early symptoms may be nonspecific, making diagnosis challenging.
Takayasu arteritis is relatively higher in Asian countries, especially in Japan, India, and Korea. It is less common in Western countries, including the United States and Europe. Takayasu arteritis usually affects young to middle-aged individuals, with most cases diagnosed between 15 and 40. The disease primarily affects females, with a female-to-male ratio of around 8:1, indicating a strong female predisposition.
Certain ethnic groups, particularly individuals of Asian descent, have a higher risk of developing Takayasu arteritis than other racial or ethnic groups. The disease is more prevalent in certain regions with a higher incidence reported in Southeast Asia, South Asia, East Asia, and Latin America. Despite being more common in specific regions, TA is still considered a rare disease worldwide, with an estimated incidence of 1 to 2 cases per million people per year.
The pathophysiology of Takayasu arteritis involves chronic inflammation of the large and medium-sized arteries, leading to various changes in the arterial walls. The exact cause is not fully understood, but it is believed to be an autoimmune-mediated process. In TA, the arterial walls become infiltrated with inflammatory cells, including lymphocytes, macrophages, and giant cells.
These inflammatory cells release various cytokines and mediators that perpetuate the inflammatory response. The intima undergoes thickening due to chronic inflammation. This process is known as intimal hyperplasia, narrowing the arterial lumen and reducing blood flow. The middle layer of the artery, the media, is affected by fibrosis. Medial fibrosis can lead to arterial stiffness and compromise the elasticity of the artery.
The reduced blood flow to various organs and tissues can lead to ischemia, resulting in organ dysfunction and damage. The severity of organ involvement varies depending on the affected arteries and the extent of the disease. In response to chronic inflammation and arterial changes, the body may attempt to remodel the affected arteries, leading to a thickened, narrowed, or dilated appearance.
Genetic Predisposition: Evidence suggests a genetic component in the development of Takayasu arteritis. Some studies have found associations between specific human leukocyte antigen (HLA) alleles, particularly HLA-B52, and an increased risk of developing TA in certain populations. However, the genetic factors involved in TA are likely complex and involve multiple genes.
Environmental Triggers: Environmental factors may play a role in triggering or exacerbating the disease in individuals with a genetic predisposition. Infections have been proposed as potential triggers for the immune system’s abnormal response, leading to the initiation of TA in susceptible individuals. However, specific infectious agents or environmental triggers have not been consistently identified across all cases.
Hormonal Factors: The female predominance in Takayasu arteritis suggests that hormonal factors may play a role in the disease’s development.
Overall, the long-term prognosis for patients with TA has improved over the years with advances in medical management and early detection. With appropriate and timely treatment, many individuals can achieve remission and have a good quality of life.
Clinical History
Takayasu arteritis is a chronic disease, and its onset can be insidious, meaning that symptoms may develop gradually over time. The age of onset is typically younger, with most cases diagnosed in individuals between 15 and 40 years old, although it can occur in people of all ages. Females are affected more commonly than males. The symptoms of Takayasu arteritis can vary widely among individuals and depend on the arteries affected and the extent of inflammation.
Common symptoms and clinical manifestations of TA include fatigue, fever, unexplained weight loss, loss of appetite, muscle and joint pain, headaches, dizziness or fainting spells, hypertension, weak or absent pulses in the limbs, skin changes, such as rashes, nodules, or ulcers, visual disturbances or blindness (if the arteries supplying the eyes are affected). The duration of symptoms can vary greatly from person to person. Some individuals may experience a relatively rapid onset of symptoms.
In contrast, others may have a more indolent course with mild or intermittent symptoms for an extended period before a definitive diagnosis is made. Early symptoms are often nonspecific and can be mistaken for other conditions, leading to delays in diagnosis. Given the chronic and often fluctuating nature of the disease, individuals with Takayasu arteritis may experience periods of remission and disease flare-ups.
Physical Examination
In Takayasu arteritis, there may be a significant difference in blood pressure readings between the arms. This difference can occur due to the narrowing or occlusion of the arteries supplying the arms. Auscultation of the blood vessels, particularly in the neck, may reveal abnormal sounds called bruits.
Bruits are caused by turbulent blood flow in narrowed arteries and can be a sign of arterial stenosis. Hypertension is common in Takayasu arteritis, particularly if the renal arteries are affected. The femoral arteries in the groin area may have a diminished or weak pulse due to involvement by the disease.
The pulses in the legs, particularly in the popliteal and dorsalis pedis arteries, may be weak or absent. Skin findings can include skin rashes, ulcers, nodules, or color changes (such as livedo reticularis) due to impaired blood flow. Ophthalmic involvement may lead to visual disturbances, such as decreased vision or blindness, and an ophthalmologist may detect changes during an eye examination.
Differential Diagnoses
Atherosclerosis
Aortic coarctation
Behcet disease
Kawasaki disease
Sarcoidosis
Rheumatoid arthritis
Corticosteroids and Immunosuppressants
The primary goal of treatment is to suppress inflammation, prevent disease progression, and manage complications. Medications such as corticosteroids and immunosuppressants (e.g., methotrexate, azathioprine, or biologic agents) are commonly used to achieve these objectives. In some cases, interventional procedures or surgery may be required to address arterial stenosis or aneurysms.
Surgical Revascularization
Surgical revascularization is emerging as a primary treatment option for Takayasu arteritis, but the role of transluminal angioplasty in managing the condition remains limited. Previous studies have shown that arterial obliteration’s fibrous nature hinders long-term angioplasty benefits. A recent study conducted at the Cleveland Clinic reported that 78% of their patient cohort treated with angioplasty at different arterial sites experienced restenosis over a median 3-year follow-up period. Additionally, 93% of these patients required further reintervention at some point. As a result, percutaneous transluminal angioplasty should be considered a short-term strategy primarily aimed at providing symptomatic relief, especially if other pressing medical needs take precedence in a particular patient.
Arterial Reconstruction
Arterial reconstruction stands as an alternative surgical option for patients diagnosed with Takayasu arteritis. The choice of reconstruction method depends on the location of known lesions and the patient’s individual surgical anatomy. For major aortic reconstructions, Dacron or polytetrafluoroethylene (PTFE) grafts are typically used. In contrast, autogenous saphenous vein grafts are employed for extremity or isolated renal or mesenteric revascularization. However, caution should be exercised when using the saphenous vein due to its known tendency for aneurysmal degeneration.
In the surgical procedure, it is crucial to ensure that both the proximal and distal anastomotic sites are free of any inflammatory involvement. Grafting onto segments of arteries affected by inflammation can lead to early graft failure. A study conducted at the University of Southern California, which involved 40 patients with TA, revealed that renovascular procedures were the most common. Other procedures included various aortic reconstructions, aorta-carotid bypasses for cerebrovascular insufficiency, and bypasses to distal subclavian, axillary, or brachial arteries to address upper extremity ischemia.
medtigo
Takayasu arteritis
Updated :
September 5, 2023
Takayasu arteritis (TA) is a rare, chronic, inflammatory condition that affects the large arteries of the body, particularly the aorta and its main branches. It is named after Dr. Mikito Takayasu, a Japanese ophthalmologist who first described the disease in 1908. Takayasu arteritis primarily affects young to middle-aged women but can also occur in men, children, and the elderly.
The exact cause of the disease is not well understood, but it is believed to involve an abnormal immune response leading to inflammation of the arterial walls. Genetics and environmental factors might also play a role. The disease often progresses slowly, and its symptoms can vary depending on the affected arteries and the extent of inflammation. Early symptoms may be nonspecific, making diagnosis challenging.
Takayasu arteritis is relatively higher in Asian countries, especially in Japan, India, and Korea. It is less common in Western countries, including the United States and Europe. Takayasu arteritis usually affects young to middle-aged individuals, with most cases diagnosed between 15 and 40. The disease primarily affects females, with a female-to-male ratio of around 8:1, indicating a strong female predisposition.
Certain ethnic groups, particularly individuals of Asian descent, have a higher risk of developing Takayasu arteritis than other racial or ethnic groups. The disease is more prevalent in certain regions with a higher incidence reported in Southeast Asia, South Asia, East Asia, and Latin America. Despite being more common in specific regions, TA is still considered a rare disease worldwide, with an estimated incidence of 1 to 2 cases per million people per year.
The pathophysiology of Takayasu arteritis involves chronic inflammation of the large and medium-sized arteries, leading to various changes in the arterial walls. The exact cause is not fully understood, but it is believed to be an autoimmune-mediated process. In TA, the arterial walls become infiltrated with inflammatory cells, including lymphocytes, macrophages, and giant cells.
These inflammatory cells release various cytokines and mediators that perpetuate the inflammatory response. The intima undergoes thickening due to chronic inflammation. This process is known as intimal hyperplasia, narrowing the arterial lumen and reducing blood flow. The middle layer of the artery, the media, is affected by fibrosis. Medial fibrosis can lead to arterial stiffness and compromise the elasticity of the artery.
The reduced blood flow to various organs and tissues can lead to ischemia, resulting in organ dysfunction and damage. The severity of organ involvement varies depending on the affected arteries and the extent of the disease. In response to chronic inflammation and arterial changes, the body may attempt to remodel the affected arteries, leading to a thickened, narrowed, or dilated appearance.
Genetic Predisposition: Evidence suggests a genetic component in the development of Takayasu arteritis. Some studies have found associations between specific human leukocyte antigen (HLA) alleles, particularly HLA-B52, and an increased risk of developing TA in certain populations. However, the genetic factors involved in TA are likely complex and involve multiple genes.
Environmental Triggers: Environmental factors may play a role in triggering or exacerbating the disease in individuals with a genetic predisposition. Infections have been proposed as potential triggers for the immune system’s abnormal response, leading to the initiation of TA in susceptible individuals. However, specific infectious agents or environmental triggers have not been consistently identified across all cases.
Hormonal Factors: The female predominance in Takayasu arteritis suggests that hormonal factors may play a role in the disease’s development.
Overall, the long-term prognosis for patients with TA has improved over the years with advances in medical management and early detection. With appropriate and timely treatment, many individuals can achieve remission and have a good quality of life.
Clinical History
Takayasu arteritis is a chronic disease, and its onset can be insidious, meaning that symptoms may develop gradually over time. The age of onset is typically younger, with most cases diagnosed in individuals between 15 and 40 years old, although it can occur in people of all ages. Females are affected more commonly than males. The symptoms of Takayasu arteritis can vary widely among individuals and depend on the arteries affected and the extent of inflammation.
Common symptoms and clinical manifestations of TA include fatigue, fever, unexplained weight loss, loss of appetite, muscle and joint pain, headaches, dizziness or fainting spells, hypertension, weak or absent pulses in the limbs, skin changes, such as rashes, nodules, or ulcers, visual disturbances or blindness (if the arteries supplying the eyes are affected). The duration of symptoms can vary greatly from person to person. Some individuals may experience a relatively rapid onset of symptoms.
In contrast, others may have a more indolent course with mild or intermittent symptoms for an extended period before a definitive diagnosis is made. Early symptoms are often nonspecific and can be mistaken for other conditions, leading to delays in diagnosis. Given the chronic and often fluctuating nature of the disease, individuals with Takayasu arteritis may experience periods of remission and disease flare-ups.
Physical Examination
In Takayasu arteritis, there may be a significant difference in blood pressure readings between the arms. This difference can occur due to the narrowing or occlusion of the arteries supplying the arms. Auscultation of the blood vessels, particularly in the neck, may reveal abnormal sounds called bruits.
Bruits are caused by turbulent blood flow in narrowed arteries and can be a sign of arterial stenosis. Hypertension is common in Takayasu arteritis, particularly if the renal arteries are affected. The femoral arteries in the groin area may have a diminished or weak pulse due to involvement by the disease.
The pulses in the legs, particularly in the popliteal and dorsalis pedis arteries, may be weak or absent. Skin findings can include skin rashes, ulcers, nodules, or color changes (such as livedo reticularis) due to impaired blood flow. Ophthalmic involvement may lead to visual disturbances, such as decreased vision or blindness, and an ophthalmologist may detect changes during an eye examination.
Differential Diagnoses
Atherosclerosis
Aortic coarctation
Behcet disease
Kawasaki disease
Sarcoidosis
Rheumatoid arthritis
Corticosteroids and Immunosuppressants
The primary goal of treatment is to suppress inflammation, prevent disease progression, and manage complications. Medications such as corticosteroids and immunosuppressants (e.g., methotrexate, azathioprine, or biologic agents) are commonly used to achieve these objectives. In some cases, interventional procedures or surgery may be required to address arterial stenosis or aneurysms.
Surgical Revascularization
Surgical revascularization is emerging as a primary treatment option for Takayasu arteritis, but the role of transluminal angioplasty in managing the condition remains limited. Previous studies have shown that arterial obliteration’s fibrous nature hinders long-term angioplasty benefits. A recent study conducted at the Cleveland Clinic reported that 78% of their patient cohort treated with angioplasty at different arterial sites experienced restenosis over a median 3-year follow-up period. Additionally, 93% of these patients required further reintervention at some point. As a result, percutaneous transluminal angioplasty should be considered a short-term strategy primarily aimed at providing symptomatic relief, especially if other pressing medical needs take precedence in a particular patient.
Arterial Reconstruction
Arterial reconstruction stands as an alternative surgical option for patients diagnosed with Takayasu arteritis. The choice of reconstruction method depends on the location of known lesions and the patient’s individual surgical anatomy. For major aortic reconstructions, Dacron or polytetrafluoroethylene (PTFE) grafts are typically used. In contrast, autogenous saphenous vein grafts are employed for extremity or isolated renal or mesenteric revascularization. However, caution should be exercised when using the saphenous vein due to its known tendency for aneurysmal degeneration.
In the surgical procedure, it is crucial to ensure that both the proximal and distal anastomotic sites are free of any inflammatory involvement. Grafting onto segments of arteries affected by inflammation can lead to early graft failure. A study conducted at the University of Southern California, which involved 40 patients with TA, revealed that renovascular procedures were the most common. Other procedures included various aortic reconstructions, aorta-carotid bypasses for cerebrovascular insufficiency, and bypasses to distal subclavian, axillary, or brachial arteries to address upper extremity ischemia.
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