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Takayasu's arteritis

ICD-10 Codes

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Description

Takayasu's Arteritis: A Rare Inflammatory Disease

Takayasu's arteritis, also known as pulseless disease, is a rare and chronic inflammatory condition that affects the large arteries in the body. The disease primarily targets the aorta, the main blood vessel leaving the heart, and its major branches.

Characteristics of Takayasu's Arteritis

  • Rare occurrence: Takayasu's arteritis is estimated to occur at a rate of 2-3 cases per million people per year [1].
  • Age and gender predilection: The disease predominantly affects young Asian women, although anyone can be affected [13].
  • Large vessel involvement: The aorta and its primary branches are the most commonly affected arteries in Takayasu's arteritis [11].

Symptoms of Takayasu's Arteritis

The initial symptoms of Takayasu's arteritis are often non-specific and may include: * Malaise (a general feeling of being unwell) * Profound fatigue * Fever * Pain, numbness or weakness with use of limbs * Lightheadedness, dizziness, fainting * Headaches * Visual disturbances * High blood pressure [9]

Complications and Damage

If left untreated, Takayasu's arteritis can lead to significant damage to the affected arteries, including: * Stenosis (narrowing) * Occlusions (blockage) * Aneurysmal degeneration (formation of an abnormal balloon-like bulge)

References:

[1] - Search result 2 [11] - Search result 11 [13] - Search result 13

Signs and Symptoms

Early-stage symptoms of Takayasu’s arteritis include:

  • Fever [1]
  • Fatigue [1][7][8]
  • Pain in your muscles or joints [1][3][5]
  • Not much desire to eat [1]

Later-stage or second-stage symptoms of Takayasu’s arteritis include:

  • Chest pain [2][4][6][9][12][14]
  • Shortness of breath [2][9][12]
  • Headaches [7][8][13]
  • Dizziness [3][8][13]

Other possible symptoms:

  • Narrowing of blood vessels and reduced blood supply can cause:
    • Pain in the hand or lower leg with use (claudication), often with absent pulse or abnormally reduced blood pressure [3]
    • Abdominal pain especially after eating [3]
    • Lightheadedness, dizziness, fainting [8][13]
    • Visual disturbances [8]
    • High blood pressure [9]

It's worth noting that:

  • The symptoms of Takayasu’s arteritis can vary depending on the stage of the illness and the arteries affected [12].
  • Most of the symptoms are vague, such as fatigue and chest pain, which are signs shared by many health problems [15].

References:

[1] Early-stage or first-stage symptoms of Takayasu’s arteritis include: Fever. Fatigue. Pain in your muscles or joints. Not much desire to eat. [2] Later-stage or second-stage symptoms of Takayasu’s arteritis include: Chest pain. Shortness of breath. Headaches. Dizziness. [3] Narrowing of blood vessels and reduced blood supply can cause: + Pain in the hand or lower leg with use (claudication), often with absent pulse or abnormally reduced blood pressure + Abdominal pain especially after eating [4] Takayasu's arteritis most often affects young Asian women, but it also can affect children and adults of all ages and ethnic backgrounds. [5] by D Alnabwani · 2021 · Cited by 32 — Common early signs and symptoms are weakness, malaise, and fever

Additional Symptoms

  • Visual disturbances
  • Shortness of breath
  • Headaches
  • Pain in your muscles or joints
  • Not much desire to eat
  • Narrowing of blood vessels and reduced blood supply can cause: Pain in the hand or lower leg with use (claudication), often with absent pulse or abnormally reduced blood pressure.
  • Lightheadedness, dizziness, fainting
  • chest pain
  • high blood pressure
  • abdominal pain
  • fever
  • dizziness
  • fatigue

Diagnostic Tests

Diagnosing Takayasu's Arteritis: A Comprehensive Overview

Takayasu's arteritis, a rare autoimmune disease that affects the blood vessels, requires a combination of diagnostic tests to confirm its presence. The following tests can help your healthcare provider make a diagnosis:

  • Magnetic Resonance Imaging (MRI): This test produces images without using X-rays and is useful in detecting stenosis and dilation of the aorta, its branches, or both [1].
  • Angiography: This diagnostic imaging technique shows stenosis and dilation of the aorta, its branches, or both, confirming the diagnosis [3].
  • Laboratory tests: While laboratory test results are nonspecific, an elevated erythrocyte sedimentation rate (ESR) may be present in early disease [2]. However, tissue biopsy plays little to no role in the diagnosis of Takayasu arteritis.
  • Blood pressure measurement: Unequal blood pressures may be recorded between sides, which can indicate the presence of the condition [4].
  • Imaging tests: Other imaging tests such as positron emission tomography (PET scanning), magnetic resonance angiography (MRA), high-resolution ultrasound, and CT angiography may also be used to confirm the diagnosis [6].

Additional Diagnostic Tests

In some cases, additional diagnostic tests may be necessary to rule out other conditions that resemble Takayasu's arteritis. These may include:

  • Blood tests: Blood tests can help detect inflammation and are commonly used in conjunction with other diagnostic tests [7].
  • Cell-mediated mechanisms: Cell-mediated mechanisms are thought to be of primary importance, and genetic links to disease susceptibility have been identified [10].

Confirming the Diagnosis

The

Additional Diagnostic Tests

  • Blood tests
  • Magnetic Resonance Imaging (MRI)
  • Laboratory tests
  • Angiography
  • Blood pressure measurement
  • Imaging tests (including PET scanning, MRA, high-resolution ultrasound, and CT angiography)
  • Cell-mediated mechanisms

Treatment

Treatment Options for Takayasu's Arteritis

Takayasu's arteritis, a rare and chronic inflammatory disease, requires long-term treatment to control inflammation and prevent further damage to blood vessels. The primary goal of drug treatment is to manage symptoms, slow disease progression, and improve quality of life.

Medications Used in Treatment

  • Glucocorticoids: These are the first-line treatment for Takayasu's arteritis. They help reduce inflammation and control symptoms. Examples include prednisone and corticosteroid medications.
  • Immunosuppressive agents: When glucocorticoids alone do not work well or if there is a high risk of steroid-related complications, other immunosuppressive drugs may be added to the treatment regimen. These can include:
    • Tumor necrosis factor (TNF) inhibitors: etanercept (Enbrel), infliximab (Remicade)
    • Interleukin-6 receptor antagonist: tocilizumab (Actemra)
  • Non-biologic disease-modifying antirheumatic drugs (DMARDs): These can include methotrexate, azathioprine, mycophenolate mofetil, and cyclophosphamide.
  • Glucocorticoid-sparing agents: These medications are used to reduce the dose of glucocorticoids required for treatment.

Treatment Approach

The mainstay of therapy for Takayasu arteritis is systemic glucocorticoids. The goal is to control inflammation and prevent further damage to blood vessels. Treatment may involve a combination of medications, including immunosuppressive agents and non-biologic DMARDs, depending on the severity of symptoms and individual patient needs.

References

  • [1] Barra L, Yang G, Pagnoux C. Non-glucocorticoid drugs for the treatment of Takayasu's arteritis: A review. [2]
  • [3] Regola F. Takayasu Arteritis: Conventional DMARDs​​ The mainstay of therapy for the induction of remission in Takayasu arteritis is systemic glucocorticoids, with a dose and duration tailored to the individual patient's needs.
  • [4] Drugs.com Mobile App. Access drug & treatment information, identify pills, check interactions and set up personal medication records.

Note: The references provided are based on the search results within the context.

Differential Diagnosis

Differential Diagnosis of Takayasu Arteritis

Takayasu arteritis, a rare form of large vessel vasculitis, requires careful consideration of differential diagnoses to ensure accurate diagnosis and treatment. The following conditions should be considered in the differential diagnosis of Takayasu arteritis:

  • Cerebral aneurysms: These can be associated with Takayasu arteritis, particularly in patients with a history of hypertension or other vascular risk factors [1].
  • IgG4-related disease: This is another form of vasculitis that can affect large vessels and should be considered in the differential diagnosis of Takayasu arteritis [1].
  • Other large vessel vasculitides: Conditions such as giant cell arteritis (GCA) and polyarteritis nodosa (PAN) can also present with similar symptoms to Takayasu arteritis and should be ruled out through careful clinical evaluation and diagnostic testing.
  • Systemic-onset juvenile rheumatoid arthritis: This condition can also cause inflammation of the blood vessels, particularly in children and adolescents [9].
  • Migraine: Some patients with migraine may experience vascular symptoms that could be mistaken for Takayasu arteritis [9].
  • Infection: Infections such as endocarditis or sepsis can cause systemic inflammation and should be ruled out through laboratory testing.
  • Malignancy: Certain types of cancer, such as lymphoma or leukemia, can also cause vascular symptoms that could be mistaken for Takayasu arteritis [9].
  • Cogan syndrome: This is a rare condition characterized by interstitial keratitis, hearing loss, and vasculitis, which should be considered in the differential diagnosis of Takayasu arteritis [9].

References:

[1] Park MC, Lee SW, Park YB, Chung NS, Lee SK. Clinical characteristics and outcomes of Takayasu's arteritis: analysis of 108 patients using standardized criteria for diagnosis, activity assessment, and ... (Search result 1) [9] Jan 7, 2021 — Diagnostic Considerations · Systemic-onset juvenile rheumatoid arthritis · Migraine · Infection · Malignancy · Cogan syndrome (associated with ... (Search result 9)

Additional Differential Diagnoses

Additional Information

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