Materiały źródłowe

• #1 Takayasu’s arteritis – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/takayasus-arteritis/diagnosis-treatment/drc-20351340

  Your doctor will ask you about your signs and symptoms, conduct a physical exam, and take your medical history. He or she may also have you undergo some of the following tests and procedures to help rule out other conditions that resemble Takayasu’s arteritis and to confirm the diagnosis. Some of these tests may also be used to check on your progress during treatment. […] Blood tests. These tests can be used to look for signs of inflammation. Your doctor may also check for anemia. […] X-rays of your blood vessels (angiography). During an angiogram, a long, flexible tube (catheter) is inserted into a large artery or vein. A special contrast dye is then injected into the catheter, and X-rays are taken as the dye fills your arteries or veins. The resulting images allow your doctor to see if blood is flowing normally or if it’s being slowed or interrupted due to narrowing (stenosis) of a blood vessel. A person with Takayasu’s arteritis generally has several areas of stenosis.

• #2 Takayasu’s arteritis – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/takayasus-arteritis/symptoms-causes/syc-20351335

  Make an appointment with your doctor if you have other signs or symptoms that worry you. Early detection of Takayasu’s arteritis is key to getting effective treatment. […] If you’ve already been diagnosed with Takayasu’s arteritis, keep in mind that your symptoms may come and go even with effective treatment. Pay attention to symptoms similar to those that occurred originally or to any new ones, and be sure to tell your doctor promptly about changes.

• #3 Takayasu Arteritis Workup: Approach Considerations, Diagnostic Criteria, Assessing Disease Activity

  https://emedicine.medscape.com/article/332378-workup

  The Ishikawa criteria (1986) have been useful in defining Takayasu arteritis. One criterion is age younger than 40 years at diagnosis or at onset of characteristic signs and symptoms of 1-month duration. […] The American College of Rheumatology and the European Alliance of Associations for Rheumatology (ACR/EULAR) published updated classification criteria for Takayasu arteritis in 2022. […] Assessing disease activity in patients with Takayasu arteritis is frequently challenging, since clinical, biologic, and radiologic information do not always correlate. […] Angiography, the criterion standard for the diagnosis and evaluation of Takayasu arteritis, is used to evaluate only the appearance of the lumen and cannot be used to differentiate between active and inactive lesions. […] CT helical scanning angiography is a sensitive and specific diagnostic tool.

• #4 Takayasu’s Arteritis | UCSF Department of Surgery

  https://surgery.ucsf.edu/condition/takayasus-arteritis

  Takayasus arteritis is most commonly diagnosed in young Asian women near or under the age of 30. […] When a person under 40 years of age suffers from unexplained high blood pressure, this may be an early clue for a diagnosis of Takayasus arteritis. The following criteria are used to distinguish Takayasus arteritis from other forms of vasculitis: […] Symptom onset at age 40 or younger. […] Muscle weakness and pain during arm and leg movement. […] A weak pulse in the brachial artery, the major artery in the upper arm. […] A bruit, or a whooshing sound, which can be heard through a stethoscope, in the subclavian artery or the aorta. […] A differential of at least 10 mmHg in systolic blood pressure in the right versus the left arm.

• #5 Takayasu Arteritis Clinical Presentation: History, Physical Examination

  https://emedicine.medscape.com/article/332378-clinical

  The presentation of Takayasu arteritis is heterogeneous. Approximately 10% of patients with Takayasu arteritis are asymptomatic, and the diagnosis is suggested only by abnormal vascular findings on physical exam. […] In the American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for Takayasu arteritis, absolute requirements are age 60 years at diagnosis and evidence of vasculitis in the aorta or branch arteries, confirmed by vascular imaging. Additional criteria are assigned points; a cumulative score of 5 points has sensitivity of 93.8% and specificity of 99.2%. […] A thorough physical examination is essential, with particular attention paid to peripheral pulses, blood pressure in all 4 extremities, and ophthalmologic examination. The most discriminatory finding is a systolic blood pressure difference ( 10 mm Hg) between arms.

• #6 Takayasu Arteritis or Pulseless Disease – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/takayasu-arteritis

  This is made on the basis of formal criteria such as those published by the American College of Rheumatology. For diagnosis the patient should fulfil three or more of the criteria listed below: […] In practice clinical examination most commonly detects decreased or absent pulses in the arms and less frequently in the legs. Using a stethoscope, bruits, (a loud whooshing noise) may be detected, over the neck, chest or kidneys indicating narrowed arteries. High blood pressure is commonly found. In recent years specialist scans have been introduced to aid diagnosis and identify inflammation, narrowing and dilatation of arteries and the extent of the disease. These include positron emission tomography (PET scanning), magnetic resonance angiography (MRA), high-resolution ultrasound and CT angiography.

• #7 Takayasu Arteritis Clinical Presentation: History, Physical Examination

  https://emedicine.medscape.com/article/332378-clinical

  Hypertension due to renal artery involvement (and sometimes leading to hypertensive encephalopathy) is found in approximately 50% of patients. […] Absent or diminished pulses are the clinical hallmark of Takayasu arteritis, but pulses are normal in many patients. […] Ophthalmologic examination may show retinal ischemia, retinal hemorrhages, cotton-wool exudates, venous dilatation and beading, microaneurysms of peripheral retina, optic atrophy, vitreous hemorrhage, and classic, wreathlike peripapillary arteriovenous anastomoses (extremely rare). […] Clinical assessment in Takayasu’s arteritis: major challenges and controversies. […] Advances in the diagnosis of large vessel vasculitis.

• #8 Takayasu Arteritis Workup: Approach Considerations, Diagnostic Criteria, Assessing Disease Activity

  https://emedicine.medscape.com/article/332378-workup

  Laboratory test results in individuals with Takayasu arteritis tend to be nonspecific. The erythrocyte sedimentation rate may be high, generally greater than 50 mm/h, in early disease but normal later. The leukocyte count may be normal or slightly elevated. A moderate, normochromic anemia may be present in individuals with active disease. […] HLA typing is not a standard diagnostic procedure for North American patients. Presumably, a finding of HLA-B*52 in such patients reinforces the diagnosis, but it is not a definite diagnostic tool. […] Although conventional angiography has historically been the standard for diagnosis and evaluation of the extent of disease, computed tomography and magnetic resonance techniques have gained favor for initial evaluation, as they are less invasive than standard angiography and allow diagnosis of Takayasu arteritis earlier in the disease course.

• #9 Takayasu’s Arteritis | Doctor

  https://patient.info/doctor/takayasus-arteritis

  There are no diagnostic blood tests. FBC may show a normocytic anaemia in 50% of patients. ESR may be high (50 mm/hour or more) in the early stages but normal in the later stages. The leukocyte count may be normal or slightly raised. Circulating anti-endothelial antibodies may be present in high titres but these can be found in many connective tissue diseases. […] Angiography – this has been used for many years as the main diagnostic test. However, it evaluates the arterial lumen only and cannot distinguish between active and inactive lesions. It is, however, still used to characterise the condition into types dependent on arterial involvement.

• #9 Takayasu’s Arteritis | Doctor

  https://patient.info/doctor/takayasus-arteritis

  Takayasu’s arteritis is a rare, idiopathic systemic inflammatory disease affecting large arteries, including the aorta, its major branches and the pulmonary arteries. The mandatory criterion is angiography (conventional, CT or MRI) of the aorta or its main branches and pulmonary arteries showing aneurysm/dilatation, narrowing, occlusion or thickened arterial wall not due to fibromuscular dysplasia, or similar causes. The diagnostic criteria are: Signs and symptoms characteristic for more than one month. Claudication of limbs. Absence of pulses or differential pulse between arms 10 mm Hg. Palpitations. Syncope. Dyspnoea. Cervical pain. Fever. Amaurosis fugax (transient loss of vision in one or both eyes due to a lack of blood flow to the eye). Blurred vision. Pathological radiological findings in the middle region of the right or left subclavian artery.

• #10 Takayasu Arteritis – Vasculitis Foundation

  https://vasculitisfoundation.org/education/vasculitis-types/takayasu-arteritis/

  Abnormal exam findings may raise suspicion of TAK: a weak or absent pulse in the limbs (TAK is sometimes called the pulseless disease), low or high blood pressure, different blood pressure between arms and legs or sounds called bruits heard over the large arteries with a stethoscope. […] Two common blood tests that can help detect inflammation include the erythrocyte sedimentation rate (ESR) test, commonly called the sed rate and the C-reactive liver protein test (CRP). These tests, however, are not conclusive on their own and may be normal in up to 50 percent of patients with TAK. […] Non-invasive tests such as magnetic resonance angiogram (MRA) and computed tomography angiography (CTA) are usually enough to establish the diagnosis. An angiogram (an X-ray of a blood vessel that has been injected with a special contrast dye) is rarely needed. Imaging tests can show narrowing or blockage of the vessels or the formation of an aneurysman abnormal bulge in the blood vessel that can burst, with life-threatening consequences. Your doctor may order other imaging tests as indicated including the Doppler ultrasound and positron emission tomography (PET) scan.

• #11 Treatment of Chronic and Relapsing Takayasu’s Arteritis

  https://consultqd.clevelandclinic.org/treatment-of-chronic-and-relapsing-takayasus-arteritis

  Laboratory tests are not always helpful in establishing diagnosis or monitoring disease activity as the sedimentation rate (WSR) and C-reactive protein (CRP) may be normal at times of active disease, in up to 50% of patients. […] While establishing early diagnosis is difficult, determining and monitoring disease activity in patients with TAK also proves challenging. […] Risks assessment is very important in patients with TAK. […] The care of these patients relies on close monitoring of disease over time by combining evaluation of symptoms, vascular physical exam, laboratory tests and sequential imaging tests. […] Imaging tests at regular intervals allow for evaluation of location and extent of arterial lesions and the identification of lesions in new vascular territories and progression of previous arterial lesions. […] While TAK may be challenging to diagnose and treat, careful monitoring with the appropriate clinical expertise and tools and thoughtful, risk-sensitive treatment can improve outcomes for patients.

• #12 Pathophysiology, Diagnosis, and Management of Takayasu Arteritis: A Review of Current Advances

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10386905/

  Takayasu arteritis (TA) is a rare, chronic, inflammatory vasculitis that primarily affects large arteries, causing significant morbidity and mortality. […] Diagnosis of TA can be challenging due to non-specific clinical manifestations and the lack of specific diagnostic tests. Various imaging modalities, such as angiography, ultrasound, and Doppler techniques, play a crucial role in the diagnosis of TA by visualizing arterial involvement and assessing disease extent. […] An accurate and timely diagnosis of TA is essential for initiating appropriate management strategies. However, the diagnosis of TA can be challenging due to its non-specific clinical presentation and the lack of specific diagnostic tests. […] Angiography is considered the gold standard for visualizing arterial involvement and assessing the extent of disease.

• #13 Takayasu’s arteritis – Wikipedia

  https://en.wikipedia.org/wiki/Takayasu%27s_arteritis

  Diagnosis is based on the demonstration of vascular lesions in large and middle-sized vessels on angiography, CT scan, magnetic resonance angiography or FDG PET. Seeing abnormal diffuse arterial wall thickening, the 'macaroni sign’, with ultrasound is highly suggestive of the condition. […] FDG PET can help in diagnosis of active inflammation not just in patients with active Takayasu arteritis prior to treatment but also in addition in relapsing patients receiving immunosuppressive agents. […] Contrast angiography has been the gold standard. The earliest detectable lesion is a local narrowing or irregularity of the lumen. This may develop into stenosis and occlusion. The characteristic finding is the presence of „skip lesions,” where stenosis or aneurysms alternate with normal vessels. Angiography provides information on vessel anatomy and patency but does not provide information on the degree of inflammation in the wall.

• #14 Takayasu arteritis | MedLink Neurology

  https://www.medlink.com/articles/takayasu-arteritis

  Currently, noninvasive CTA and vascular MRI with its two components, contrast-enhanced MRI and MRA (conventional or 3-dimensional reconstruction), are considered to have good diagnostic accuracy. […] The 2018 EULAR recommendations include CTA, MRI/MRA, and PET-CT as noninvasive imaging modalities for the diagnosis of Takayasu arteritis in suspected individuals; CTA and MRI/MRA are recommended for monitoring patients who have already been diagnosed. […] Ultrasound is a noninvasive imaging technique used to visualize vessel wall thickening (usually circumferential) of the main arteries in Takayasu arteritis. […] No specific laboratory markers have been found to be helpful for the diagnosis of Takayasu arteritis. However, increased acute phase reactants, particularly ESR, may be helpful in assessing disease activity in Takayasu arteritis. […] Sequential imaging studies in Takayasu arteritis have revealed disease progression (determined by the presence of new vascular lesions) in about half of patients presenting with asymptomatic disease and normal ESR values.

• #15 UPDATE IN TAKAYASU’S ARTERITIS – Rheumatology Quarterly

  https://qrheumatol.com/articles/update-in-takayasus-arteritis/doi/qrheumatol.galenos.2023.36844

  The level of evidence for TAK management is low, and expert opinion is still the main determinant when managing patients with TAK during daily practice. […] Currently, conventional angiography is no longer considered as the gold standard imaging tool for the diagnosis of TAK. MRA is the gold standard modality for both the diagnosis and longitudinal follow-up of patients with TAK. […] In recent years, PET has become a widely used imaging tool for the diagnosis of TAK with high sensitivity. […] There is a clear need to develop a validated set of outcome measures for use in clinical trials of TAK.

• #16 Takayasu’s arteritis – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/takayasus-arteritis/diagnosis-treatment/drc-20351340

  Magnetic resonance angiography (MRA). This less invasive form of angiography produces detailed images of your blood vessels without the use of catheters or X-rays. magnetic resonance angiography (MRA) works by using radio waves in a strong magnetic field to produce data that a computer turns into detailed images of tissue slices. During this test, a contrast dye is injected into a vein or artery to help your doctor better see and examine the blood vessels. […] Computerized tomography (CT) angiography. This is another noninvasive form of angiography combining computerized analysis of X-ray images with the use of intravenous contrast dye to allow your doctor to check the structure of your aorta and its nearby branches and to monitor blood flow. […] Ultrasonography. Doppler ultrasound, a more sophisticated version of the common ultrasound, has the ability to produce very high-resolution images of the walls of certain arteries, such as those in the neck and shoulder. It may be able to detect subtle changes in these arteries before other imaging techniques can.

• #17 Takayasu Arteritis or Pulseless Disease – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/takayasu-arteritis

  There is no blood test that can be used to definitively confirm the diagnosis. The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) can help measure inflammation as a marker of activity of the illness and your response to treatment. […] Imaging studies are useful in the diagnosis and monitoring of TA. Their use may allow diagnosis before arterial damage is severe. However, none of them are 100 per cent accurate. We now use multimodality imaging, a combination of ultrasound, CT and MRI. […] Magnetic resonance imaging (MRI) is commonly used to aid diagnosis and assess the extent of TA. The scan does not involve radiation and the picture of the arteries can be enhanced by using a contrast medium. […] Doppler ultrasound (ultrasound scan): uses a probe which is passed over the carotid arteries in the neck. This allows assessment of blood flow and can detect and monitor the arterial wall and the degree of narrowing.

• #18 Pathophysiology, Diagnosis, and Management of Takayasu Arteritis: A Review of Current Advances

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10386905/

  Early recognition and initiation of treatment are crucial to preventing complications and improving patient outcomes. […] The diagnostic criteria for TA have evolved since the initial description by Ishikawa in 1988. […] The most widely used angiographic classification system for TA is the Numano system, proposed in 1996. […] Imaging plays a crucial role in the diagnosis and surveillance of TA. […] Non-invasive imaging techniques, such as 18F-fluorodeoxyglucose positron emission tomography (FDG-PET), Doppler ultrasound (DUS), computed tomography angiography (CTA), and magnetic resonance imaging (MRI), have gained importance in the diagnosis and surveillance of TA. […] Several biomarkers have been studied to aid in the diagnosis and surveillance of TA.

• #19 Takayasu Arteritis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459127/

  Takayasu arteritis (pulseless disease) is a systemic inflammatory condition characterized by damage to the large and medium arteries and their branches. […] Diagnosis is based on suspicion as well arteriographic findings. […] Clinical suspicion is needed to diagnose Takayasu arteritis, and thus a good, complete history and physical exam is a necessity. Imaging is vital, and CTA has recently become the standard for initial staging of disease distribution (previously it was conventional angiography) in Takayasu arteritis. […] CTA will allow for visualization of vessel wall thickening and luminal narrowing. […] Surgical revascularization is now rapidly evolving into a primary treatment choice. […] Surgical therapy is usually well tolerated due to the young age of most patients with Takayasu arteritis.

• #20 Chinese guideline for the diagnosis and treatment of Takayasu’s arteritis (2023)

  https://www.degruyterbrill.com/document/doi/10.1515/rir-2024-0002/html?srsltid=AfmBOooWQpq4sKFFyDtIfGvSGNh0bevZnOcMblIPgNkNPxZQX9JNYwWL

  With recent development in imaging techniques, conventional catheter-based arteriograms (DSA) have gradually been replaced by non-invasive computed tomography angiography (CTA). In addition, other non-invasive imaging modalities, including color Doppler ultrasound (CDUS), magnetic resonance angiography (MRA), positron emission tomography/computed tomography (PET/CT), can not only reveal the extent and degree of vascular lesions, but also can provide evidence for disease activity. Therefore, these non-invasive imaging modalities have replaced conventional angiography to a certain extent. […] […] The diagnostic value of the above-mentioned non-invasive imaging modalities is limited by various factors such as the range and anatomic location of vascular lesions, the operator dependency, accessibility and feasibility. In addition, there is currently no evidence to support the priority of imaging modalities. However, a systematic review in 2018 found that the diagnostic sensitivity of CDUS was 81% and the specificity was 100%. Therefore, noninvasive modalities should be the first line choice for the patients with suspected TAK. […] […] As CTA provides good sensitivity and specificity in identify large artery lesions and is widely available in China, it is the preferred imaging modalities for diagnosing TAK and assessing the extent of vascular lesions and disease severity.

• #21 Takayasu’s arteritis – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/takayasus-arteritis/diagnosis-treatment/drc-20351340

  Positron emission tomography (PET). This imaging test is often done in combination with computerized tomography or magnetic resonance imaging. positron emission tomography (PET) can measure the intensity of inflammation in blood vessels. Before the scan, a radioactive drug is injected into a vein or an artery to make it easier for your doctor to see areas of decreased blood flow.

• #22 Takayasu Arteritis or Pulseless Disease – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/takayasu-arteritis

  Positron emission tomography (PET scanning): requires the patient to be injected with radioactive fluorodeoxyglucose. PET scans are particularly good at detecting inflammation in the arterial wall and are often used in combination with CT scanning (CT-PET). […] Contrast-enhanced CT angiography is useful for the diagnosis of TA and can help identify the extent of damage to the arteries.

• #23 Current Diagnosis and Management of Takayasu Arteritis

  https://www.jstage.jst.go.jp/article/ihj/64/4/64_23-195/_article/-char/en

  Takayasu arteritis (TA or TAK) is a chronic large vessel vasculitis with predilection to affect the aorta and its branches. […] The new 2022 ACR/EULAR classification criteria for Takayasu arteritis incorporated imaging characteristics as an absolute requirement. […] ESR and CRP fails in accuracy as disease activity markers. Pentraxin 3 appears to be a relatively superior biomarker, which correlates with ITAS 2010 as per several studies. PET-CT is also increasingly being studied for assessing disease activity with variable results. […] The management of TAK involves use of steroids with upfront steroid sparing immunosuppressive agents. MMF is one such conventional DMARD/immunosuppressant with good efficacy and better safety profile, as reported in various cohort studies. Tocilizumab is proved to be a rapid remission inducing agent in refractory Takayasu arteritis in observational studies. TNF inhibitors in many uncontrolled studies showed good responses, and there is a need for good RCTs for confirmation. JAK inhibitors have also been used with success in a few reports.

• #24

  https://link.springer.com/article/10.1007/s10067-012-2149-3

  The value of [18F]FDG-PET in the diagnosis of large-vessel vasculitis and the assessment of activity and extent of disease. […] The role of 18F-fluorodeoxyglucose-positron emission tomography in the assessment of disease activity in patients with Takayasu arteritis. […] Is (18)F-fluorodeoxyglucose positron emission tomography scanning a reliable way to assess disease activity in Takayasu arteritis? […] Comparison of F18 FDG PET/CT findings with current clinical disease status in patients with Takayasus arteritis. […] The impact of 18F-FDG PET on the management of patients with suspected large vessel vasculitis. […] Takayasu arteritis: utility and limitations of magnetic resonance imaging in diagnosis and treatment. […] Additional value of positron emission tomography in diagnosis and follow-up of patients with large vessel vasculitides. […] Role of FDG PET-CT in Takayasu arteritis: sensitive detection of recurrences.

• #25 Takayasu’s Arteritis – Harvard Health

  https://www.health.harvard.edu/a_to_z/takayasus-arteritis-a-to-z

  Because Takayasu’s arteritis is so rare and because the early symptoms are not specific, a doctor may not suspect you have the disease until you develop symptoms of narrowed arteries. This could be months, or even years, after the disease started. […] The earliest clues that you may have Takayasu’s arteritis may be high blood pressure that can’t be explained by anything else, difficulty obtaining a blood pressure measurement on one side, a difference in blood pressure reading between the two arms, or symptoms of poor circulation all of these are unusual findings in a younger person. […] Your doctor may suspect that you have Takayasu’s arteritis if you have at least three of the six criteria developed by the American College of Rheumatology to distinguish it from other forms of vasculitis: symptoms beginning at age 40 or younger, muscle weakness and pain when you move your arms or legs, a weak pulse in the brachial artery (major artery in the upper arm), a sound called a bruit, which indicates turbulent blood flow, when your doctor listens to your aorta or subclavian artery with a stethoscope, signs of damage to the aorta or other arteries on a test called an angiogram, which is a series of x-rays of arteries, a difference of at least a 10 mm Hg (millimeters of mercury) in the systolic blood pressure (the first number in your blood pressure reading) in your right and left arms.

• #26 Chinese guideline for the diagnosis and treatment of Takayasu’s arteritis (2023)

  https://www.degruyterbrill.com/document/doi/10.1515/rir-2024-0002/html?srsltid=AfmBOooWQpq4sKFFyDtIfGvSGNh0bevZnOcMblIPgNkNPxZQX9JNYwWL

  In 2022, ACR/EULAR endorsed a new classification criteria for TAK, which included 2 absolute requirements and 10 additional clinical and image criteria. Patients who met the absolute requirements combined with a cumulative score of 5 points for additional clinical and image criteria could be classified as TAK. The sensitivity and specificity of this classification criteria were 93.8% and 99.2%, respectively. […] […] Most of the clinical symptoms of TAK are non-specific. Once relevant symptoms suggesting TAK occur, clinicians need to carefully differentiate whether the aortic damage is attributed to other causes through imaging examinations. Therefore, the panel recommends that for patients with suspected clinical presentations of TAK, multidisciplinary experts including rheumatologists, radiologists, and vascular physicians should collaborate to improve the accuracy of diagnosis. […]

• #27 Outcome of Takayasu Arteritis with Inactive Disease at Diagnosis: The Extent of Vascular Involvement As a Predictor of Activation | The Journal of Rheumatology

  https://www.jrheum.org/content/42/3/489

  Objective. Some patients with Takayasu arteritis (TA) have inactive disease at the time of diagnosis. The objective of our study was to investigate the clinical outcomes and factors that predict disease activation in patients with clinically inactive TA. […] At the time of diagnosis, patients were identified as having inactive disease according to the National Institutes of Health definition. […] A total of 59 patients with TA were classified as having inactive disease at the time of diagnosis. […] Substantial portions of patients with clinically inactive TA at the time of diagnosis experienced disease activation during followup. Type V disease may be an important predictive factor for disease activation in patients with clinically inactive TA. […] It is notable that there was a significant difference in the vascular involvement depending on the angiographic classification of TA (p = 0.008). Type V, which is the most extensive type, was more common in the activation group (12/13, 92.3%) than in the stable group (18/30, 39.1%).

• #28 Outcome of Takayasu Arteritis with Inactive Disease at Diagnosis: The Extent of Vascular Involvement As a Predictor of Activation | The Journal of Rheumatology

  https://www.jrheum.org/content/42/3/489

  Notably, type V (OR 10.969, 95% CI 1.144105.182, p = 0.038) was significantly associated with an increased risk of disease activation in patients with clinically inactive TA. […] The data presented herein show that a substantial portion of patients with clinically inactive TA at the time of diagnosis experienced disease activation during followup. Involvement of type V was significantly associated with an increased risk of TA activation. These findings suggest that a type V angiographic pattern may be an important predictor for disease activation.

• #29 Takayasu Arteritis – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/takayasu-arteritis

  Takayasu arteritis is an inflammatory disease affecting the aorta, its branches, and pulmonary arteries. […] Diagnosis is by aortic arteriography or magnetic resonance angiography. […] The diagnosis of Takayasu arteritis is suspected when symptoms suggest ischemia of organs supplied by the aorta or its branches or when peripheral pulses are decreased or absent in patients at low risk of atherosclerosis and other aortic disorders, especially in young females. […] Magnetic resonance angiography or CT angiography are be used to evaluate all branches of the aorta. […] Laboratory test results are nonspecific and not helpful in diagnosis. […] The following are indicators of disease activity in Takayasu arteritis: Symptoms and signs: New systemic symptoms (eg, fever, fatigue, weight loss, anorexia, night sweats), symptoms suggesting vasculitis involving new arterial territories (eg, claudication), new bruits, and/or new changes in BP measurements. […] However, Takayasu arteritis can progress silently even when clinical and laboratory studies suggest complete remission. Therefore, periodic imaging of the aorta and large arteries is mandatory. […] Disorders that mimic Takayasu arteritis must be excluded.

• #30 Takayasu Arteritis: Update on Monitoring of Disease Activity and Management

  https://www.ekjm.org/journal/view.php?number=25554

  Takayasu arteritis (TAK) is a chronic inflammatory disease characterized by granulomatous vasculitis of the aorta and its major branches. The rarity of the disease along with its heterogeneous clinical presentation typically lead to late diagnosis and delayed treatment. […] Furthermore, clinical and serological indices for monitoring disease activity are suboptimal, with no definitive evidence supporting therapeutic approaches in TAK. Nevertheless, there have been recent advances in disease assessment with new scoring systems (Indian Takayasu Arteritis Score), biomarkers including pentraxin 3 and soluble human leukocyte antigen-E, and imaging modalities such as 18F-fluorodeoxyglucose-positron emission tomography. […] A number of potential new therapeutic targets that may be useful for the treatment of TAK have been reported, and randomized controlled trials are needed to establish optimal therapeutic approaches.

• #31 Orphanet: Takayasu arteritis

  https://www.orpha.net/en/disease/detail/3287

  The diagnosis is difficult to establish and is frequently delayed (years or even decades). It is based on the clinical features and physical examination. Vascular manifestations could guide morphologic investigations. However, the main change is related to the use of noninvasive imaging techniques (ultrasound, magnetic resonance imaging, CT angiography and positon emission tomography). Such exams could allow early diagnosis by demonstrating arterial wall thickening or inflammation in the pre-stenotic phase. […] The differential diagnosis of TAK is very broad and may include other inflammatory diseases (atherosclerosis, giant cell arteritis, IgG4 related aortitis), infectious aortitis and fibromuscular dysplasia.

• #32 Takayasu’s arteritis – Wikipedia

  https://en.wikipedia.org/wiki/Takayasu%27s_arteritis

  The age at onset helps to differentiate Takayasu’s arteritis from other types of large vessel vasculitis. For example, Takaysu’s arteritis has an age of onset of <40 years, while giant cell arteritis has an age of onset >60 years. […] Takayasu arteritis is not associated with ANCA, rheumatoid factor, ANA, and anticardiolipin antibodies.

• #33 Intracranial and Extracranial Neurovascular Manifestations of Takayasu Arteritis | American Journal of Neuroradiology

  http://www.ajnr.org/content/38/4/766

  Intracranial vascular abnormalities in patients with Takayasu arteritis presenting with neurologic symptoms are not rare, with cerebral vasculitis seen in 7.8% of patients, and stroke secondary to large-vessel occlusion, in 3.9% of patients. […] A number of studies have also reported that intracranial involvement, including steno-occlusive disease and aneurysms, is rare in patients with TA. […] Our study, the largest to date with cerebral arteriographic imaging in patients with TA, to our knowledge, found intracranial arterial involvement of TA in 15.2% of patients, with 7.6% of patients having CNS vasculitis. […] Intracranial stenoses in TA could be secondary to inflammatory vasculitis or the result of prior embolization. […] Intracranial involvement of TA was never an isolated finding because all patients with intracranial vascular involvement of TA also had extracranial manifestations, including the aorta and/or major aortic branch vessels.

• #34 Intracranial Involvement in Takayasu’s Arteritis

  https://www.mdpi.com/2075-4418/11/11/1997

  Takayasu’s arteritis (TAK) is a large-vessel vasculitis that targets the aorta and its major branches. Although extracranial vascular involvement is uniformly present in this disease, the frequency of intracranial involvement in TAK has not been well studied. […] Intracranial vascular involvement in TAK is considered uncommon; however, it has not been well studied. Existing literature suggests that intracranial vascular disease may affect between 5% and 50% of TAK patients. […] In this study, we aimed to determine the prevalence of intracranial vascular lesions in TAK patients at a single Canadian university centre, describe the most frequently observed lesions, and review the existing literature regarding the frequency of intracranial vascular disease in TAK. […] Neuroimaging was performed in 15 of 20 patients (75%); however, images were unavailable to review in 2 patients, and 1 patient had non-vascular neuroimaging only, leaving 12 TAK patients with vascular neuroimaging studies available for review.

• #35 Intracranial and Extracranial Neurovascular Manifestations of Takayasu Arteritis | American Journal of Neuroradiology

  http://www.ajnr.org/content/38/4/766

  Takayasu arteritis is a rare, large-vessel vasculitis that presents with symptoms related to end-organ ischemia. […] While the extracranial neurovascular manifestations of Takayasu arteritis are well-established, little is known regarding the intracranial manifestations. […] In this study, we characterize the intracranial and cervical neurovascular radiologic findings in patients with Takayasu arteritis. […] Patients with Takayasu arteritis who presented to our institution between 2001 and 2016 with intracranial and/or cervical vascular imaging were included in this study. […] Images were evaluated for the presence of vascular abnormalities, including intracranial or extracranial stenosis, vessel-wall thickening, dissection, subclavian steal, aneurysms, infarcts, and hemorrhages. […] Among patients with intracranial vascular imaging, 3 (3.9%) had intracranial aneurysms, 3 (3.9%) had acute large-vessel occlusion, 6 (7.6%) had intracranial vasculitis, and 1 (1.3%) had reversible cerebrovascular constriction syndrome.

• #36 Intracranial Involvement in Takayasu’s Arteritis

  https://www.mdpi.com/2075-4418/11/11/1997

  At the time of vascular neuroimaging, 8 of 12 patients (66.7%) had neurologic symptoms, including stroke (4, 33.3%), vision changes (3, 25.0%), headache (2, 16.7%), or light-headedness (2, 16.7%). Intracranial vascular lesions were identified in four patients (33.3% of those with vascular neuroimaging available, 20% of all patients). […] The frequency of intracranial involvement in TAK may be more common than appreciated. Imaging of both the intra- and extra-cranial vessels should be considered in these young patients.

• #37 Pulmonary artery involvement in Takayasu’s arteritis: diagnosis before pulmonary hypertension | BMC Pulmonary Medicine | Full Text

  https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-019-0983-7

  This study was performed to analyze the clinical manifestations, imaging features, and prognosis of Takayasus arteritis (TA) with pulmonary arteritis (PA). […] The clinical manifestations of TA with PA are nonspecific. PH often complicates PA and is associated with a poor prognosis. Early clinical manifestations such as repeated fever, chest pain, hemoptysis, and recurrence of subpleural wedge-shaped shadows on chest CT should arouse suspicion of PA in patients with TA and prompt further investigations. This may allow PA to be diagnosed before the occurrence of PH. […] Accurate diagnosis of pulmonary artery involvement virtually always depends on imaging studies because the clinical manifestations and laboratory test results are usually nonspecific. […] The present study showed that PH occurred in 58.8% of patients with PA, consistent with previous studies (42.278.1%).

• #38 Pulmonary artery involvement in Takayasu’s arteritis: diagnosis before pulmonary hypertension | BMC Pulmonary Medicine | Full Text

  https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-019-0983-7

  Therefore, early diagnosis is particularly important. Notably, the rate of PH decreased during the most recent 5 years (20112015) compared with the previous 5 years (20062010) in our center (50.0% vs. 94.3%, respectively). […] The early manifestations of PA include recurrent fever, chest pain, hemoptysis, an elevated ESR, and recurrent subpleural wedge-shaped shadows, which should raise suspicion for PA and prompt further vascular imaging investigations. This may allow TA to be diagnosed before the occurrence of irreversible stenotic and fibrotic vascular lesions.

• #39 Takayasu arteritis: Symptoms, treatment, and outlook

  https://www.medicalnewstoday.com/articles/takayasu-arteritis

  Takayasu arteritis is a rare, chronic inflammatory disease that primarily affects the aorta and its main branches. […] Understanding the symptoms and risk factors is crucial for early diagnosis and effective management, as the disease can lead to serious complications, such as a stroke, if individuals do not seek treatment. […] Early intervention, diagnosis, and treatment are vital to managing Takayasu arteritis effectively and preventing severe complications. […] There is no single test for Takayasu arteritis. Diagnosis involves a combination of tests, including: […] A doctor will check for weak or absent pulses, differences in blood pressure between the arms, and sounds known as bruits. […] A doctor will assess for signs of inflammation, such as elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). […] This can include magnetic resonance angiography (MRA) or computed tomography angiography (CTA) scans to visualize the blood vessels and identify areas of narrowing or blockages. […] Early diagnosis and treatment are crucial to improving outcomes and managing the disease.

• #40 Diagnosis at Last: Takayasu’s Arteritis | Hackensack Meridian Health

  https://www.hackensackmeridianhealth.org/en/healthu/patient-perspectives/2019/07/diagnosis-at-last-takayasus-arteritis

  Takayasus arteritis is a rare but potentially fatal disease that involves inflammation in the walls of the largest arteries in the body, the aorta and its main branches. This inflammation leads to narrowing of the arteries, reducing blood flow to many parts of the body. Takayasus arteritis can lead to arm, leg and chest pain, high blood pressure, and eventually heart failure or stroke. Even with treatment, relapses are common and symptoms can come and go. […] Takayasus arteritis is more common in women than men, and while the disease most often starts in young adults, children and middle-aged people are not immune, according to the American College of Rheumatology. The disease results from an attack by the bodys own immune system, causing the inflammation.

• #41 Takayasu arteritis—advances in diagnosis and management | Nature Reviews Rheumatology

  https://www.nature.com/articles/nrrheum.2010.82

  Takayasu arteritis, a rare granulomatous vasculitis affecting young people, is associated with considerable morbidity and premature mortality. In most patients the diagnosis is delayed until after the development of substantial arterial injury. […] Studies of noninvasive imaging techniques suggest that these approaches might facilitate earlier diagnosis and have a role in monitoring disease progress; however, they remain limited in their ability to accurately quantify inflammatory disease activity in the arterial wall. […] A lack of controlled clinical trial data complicates the choice of therapy for Takayasu arteritis, and clinical indices for monitoring disease activity are currently suboptimal. […] 18FDG-CT-PET, MRA, CTA and US imaging might allow diagnosis of pre-stenotic disease and noninvasive monitoring of arterial injury; however, they remain limited in their ability to monitor disease activity. […] Controlled clinical trials are required to establish optimum therapeutic approaches, to demonstrate a beneficial effect on vascular outcome and to determine the utility of noninvasive imaging techniques. […] Non-invasive imaging in the diagnosis and management of Takayasu’s arteritis.

• #42 Azthena logo with the word Azthena

  https://www.news-medical.net/health/How-is-Takayasus-Arteritis-Diagnosed.aspx

  X-ray angiography or arteriography is the most common procedure for the diagnosis of Takayasu’s arteritis. This procedure produces x-ray images of the blood vessels. However, early stages of the disease which could potentially be reversible can sometimes not be detected with this method. […] Two non-invasive methods have been investigation to evaluate their ability in the early diagnosis of Takayasu’s arteritis, magnetic resonance imaging (MRI) and fluorodeoxyglucose positron emission tomography (FDG-PET). With FDG-PET, it is possible to detect high glucose metabolic areas, which are typically seen when inflammation in tissues are present. […] MRI produces high resolution images that show features of vascular inflammation. The thickening of vessel walls takes place very early in the inflammatory stage of the disease, which can be detected by MRI. This shows how important MRI is in the detection of early disease stages, when damage is more likely to be reversible.

• #43 Azthena logo with the word Azthena

  https://www.news-medical.net/health/How-is-Takayasus-Arteritis-Diagnosed.aspx

  In a study by Andrews et al. in 2004, it was suggested that the non-invasive methods MRI and FDG-PET can disclose important information about the disease such as the presence of vascular inflammation or the wall thickening compared to the common angiography. Additionally, FDG-PET can identify all inflamed vessel and could be more sensitive measuring continuing blood vessel inflammation compared to conventional methods. […] The study concluded that these non-invasive methods can help to diagnose the disease early on as well as assess response to treatment when compared to other assessment methods or invasive angiography. MRI and FDG-PET could be the future of Takayasus arteritis diagnosis and replace invasive angiography in many patients. […] MRA and FDG-PET have been demonstrated as accurate methods for the diagnosis of early stages of Takayasus arteritis as well the ongoing assessment after treatment. As they are non-invasive, risks remain low compared to invasive methods. However, further studies are necessary to evaluate the future use of these methods and eventually replace existing angiography.

• #44 Diagnosis at Last: Takayasu’s Arteritis | Hackensack Meridian Health

  https://www.hackensackmeridianhealth.org/en/healthu/patient-perspectives/2019/07/diagnosis-at-last-takayasus-arteritis

  In February 2018, Nathalie met with Ginger Janow, M.D., a pediatric rheumatologist at the Childrens Hospital. After reviewing her scans and meeting with Nathalie, Dr. Janow confirmed a rare but clear diagnosis: Takayasus arteritis. Takayasus is a potentially fatal disease that causes inflammation of the blood vessels. The inflammation can restrict blood flow and damage vital organs and tissues. […] Because patients may have very few or no symptoms, and the disease is so rare, its common for doctors to misdiagnose patients for years. […] Nathalie probably had it for at least six years before a proper diagnosis, Dr. Janow says. Going back, it becomes apparent that she had long-standing anemia, her blood pressure was very low, the numbness and tingling in her arms and legs were getting worse and she had fainting episodes. They were all treated as isolated things, but we figured out that it was all part of the same disease.

• #45 Delayed diagnosis of Takayasu’s arteritis: total abdominal aorta occlusion treated with axillo-bifemoral bypass. | The Journal of Rheumatology

  https://www.jrheum.org/content/31/2/393

  Takayasu’s arteritis is an inflammatory vasculitis that primarily affects the aorta and its major branches. Delayed diagnosis is common, and is largely attributed to the variable and nonspecific presentation of the initial symptoms. […] Involvement of the abdominal aorta causes severe claudication of the lower extremities, and bypass surgery is required in some cases. We describe a case in which diagnosis was delayed. Total occlusion of the abdominal aorta was successfully treated with axillobifemoral bypass.

• #46 Chinese guideline for the diagnosis and treatment of Takayasu’s arteritis (2023)

  https://www.degruyterbrill.com/document/doi/10.1515/rir-2024-0002/html?srsltid=AfmBOooWQpq4sKFFyDtIfGvSGNh0bevZnOcMblIPgNkNPxZQX9JNYwWL

  The current clinical diagnosis of TAK is mainly based on the TAK classification criteria issued by the ACR in 1990. This criteria set included 6 items: (1) Age at disease onset 40 years; (2) Claudication of extremities; (3) Decreased brachial artery pulse; (4) Blood pressure (BP) difference 10 mmHg (1 mmHg = 0.133 kPa); (5) Bruit over subclavian arteries or aorta; (6) Arteriogram abnormality. Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities, not due to arteriosclerosis, fibromuscular dysplasia, or similar causes; changes usually focal or segmental. Patients who met three or more of the above six items can be classified as TAK. The sensitivity of this set of criteria was 90.5% and the specificity was 97.8%. However, this classification criteria is not sensitive to the early diagnosis of TAK. […]

• #47 Early diagnosis and standardized treatment are critical to improve the prognosis of patients with Takayasu’s arteritis

  https://www.degruyter.com/document/doi/10.1515/rir-2024-0001/html?lang=en

  Early diagnosis and standardized treatment are critical to improve the prognosis of patients with Takayasus arteritis. […] The clinical manifestations of TAK are heterogenous and very non-specific at early phase, so many patients are mis-diagnosed. […] Therefore, early diagnosis should be emphasized. Improving early diagnosis rate and shorten the delayed diagnosis time is the first step to reach the final goal of improving the prognosis. […] Therefore, comprehensive evaluation of the disease should be emphasized. […] Imaging examinations are important tools for disease severity and activity evaluation. […] Therefore, multidisciplinary management of patients with TAK has been proven to be an effective approach to optimize treatment outcomes. […] The level of standardization of treatment should be improved.

• #48 Chinese guideline for the diagnosis and treatment of Takayasu’s arteritis (2023)

  https://www.degruyterbrill.com/document/doi/10.1515/rir-2024-0002/html?srsltid=AfmBOooWQpq4sKFFyDtIfGvSGNh0bevZnOcMblIPgNkNPxZQX9JNYwWL

  Takayasus arteritis (TAK) is a chronic granulomatous inflammatory disease that involves aorta and its primary branches. It is characterized by wall thickening, stenosis/obliteration or aneurysm formation of the involved arteries. In order to standardize the diagnosis and treatment of TAK in China, a clinical practice guideline with an evidence-based approach is developed under the leadership of National Clinical Medical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID). Eleven recommendations for 11 clinical questions that are important to the diagnosis and treatment of TAK are developed based on the latest evidence and expert opinions combined with real clinical practice in China. […] […] For patients with suspected TAK, we recommend adopting the 2022 ACR / EULAR TAK classification criteria for the diagnosis of TAK (1C). Patients with suspected clinical symptoms or signs of TAK should be referred to a team specialized in the diagnosis of TAK led by rheumatologists for diagnostic work-up. Vascular image presentations should take into consideration for the diagnosis and differential diagnosis of TAK (2D). Noninvasive imaging modalities should be considered as the first line choices for the early diagnosis of TAK (1B). […]

• #49 Early diagnosis and standardized treatment are critical to improve the prognosis of patients with Takayasu’s arteritis

  https://www.degruyter.com/document/doi/10.1515/rir-2024-0001/html?lang=en

  The National Clinical Research Center for Dermatologic and Immunologic Diseases (Peking Union Medical College Hospital) developed and released the first Chinese guideline for the diagnosis and treatment of Takayasus arteritis (2023) in an evidence based approach. […] This guideline covers 11 clinical scenarios that are important to the diagnosis and treatment as well as long-term management of TAK. […] We believe that the release of this guideline will greatly help in improving the current status of the diagnosis and treatment of TAK not only in China, but around the world.

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