Large Vessel Vasculitis

Large Vessel Vasculitis is a topic covered in the Washington Manual of Medical Therapeutics.

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Takayasu Arteritis

General Principles

Epidemiology

Also known as the “pulseless disease.” It is most common in individuals of Asian descent and affects women more than men; the peak onset is in the third decade.

Diagnosis

Clinical Presentation

  • Clinical presentation is divided into three phases, but disease presentation is variable. Only half of the patients have constitutional symptoms and monophasic disease can also occur.
  • Prepulseless phase is manifested by constitutional symptoms like fever, weight loss, generalized malaise, myalgias, and arthralgias.
  • Second phase is characterized by vessel pain and tenderness, like carotidynia, due to vessel inflammation.
  • Burned-out or fibrotic phase is characterized by ischemic symptoms due to arterial stenoses. Some of the symptoms include limb claudication secondary to subclavian stenosis, chest pain secondary to aortitis, and less commonly coronary arteries involvement, hypertension secondary to aortic or renal arteries involvement, strokes or transient ischemic attack (TIA) due to common carotid stenosis or dizziness, and visual impairment due to vertebral artery involvement.

Diagnostic Testing

  • There are no specific tests for the diagnosis of Takayasu arteritis. Some of the laboratory abnormalities are the reflection of inflammation like anemia of chronic disease, thrombocytosis, elevated ESR, and CRP.
  • Imaging studies include:
  • MRI and CT angiography for the identification of wall edema, vessel thickness, and areas of stenosis. However, there is no good correlation between the wall changes and disease activity.
  • Intra-arterial angiography is the gold standard to detect the involved vessels. It is important to note that it only provides information about the vessel lumen but no details about the vessel walls.
  • PET scan technology is useful to detect areas of active arteritis. This can help differentiate between the inflammatory versus burned-out phases.

Treatment

Medications

  • Patients in the inflammatory phases (phase one and two) are started on pharmacologic therapy to prevent further development of stenosis or aneurysms. Patients are initially treated with oral glucocorticoids (prednisone 1 mg/kg) and then started on a steroid-sparing agent. Options include methotrexate, azathioprine, leflunomide, mycophenolate mofetil, cyclophosphamide, anti-TNF agents, and tocilizumab. Selection of steroid-sparing agent will depend on disease severity and medication tolerance. Hypertension should also be aggressively treated to prevent ischemia.
  • Surgical management is also available for amenable stenosis. These include angioplasty, endovascular stenting, and bypass surgery.

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Takayasu Arteritis

General Principles

Epidemiology

Also known as the “pulseless disease.” It is most common in individuals of Asian descent and affects women more than men; the peak onset is in the third decade.

Diagnosis

Clinical Presentation

  • Clinical presentation is divided into three phases, but disease presentation is variable. Only half of the patients have constitutional symptoms and monophasic disease can also occur.
  • Prepulseless phase is manifested by constitutional symptoms like fever, weight loss, generalized malaise, myalgias, and arthralgias.
  • Second phase is characterized by vessel pain and tenderness, like carotidynia, due to vessel inflammation.
  • Burned-out or fibrotic phase is characterized by ischemic symptoms due to arterial stenoses. Some of the symptoms include limb claudication secondary to subclavian stenosis, chest pain secondary to aortitis, and less commonly coronary arteries involvement, hypertension secondary to aortic or renal arteries involvement, strokes or transient ischemic attack (TIA) due to common carotid stenosis or dizziness, and visual impairment due to vertebral artery involvement.

Diagnostic Testing

  • There are no specific tests for the diagnosis of Takayasu arteritis. Some of the laboratory abnormalities are the reflection of inflammation like anemia of chronic disease, thrombocytosis, elevated ESR, and CRP.
  • Imaging studies include:
  • MRI and CT angiography for the identification of wall edema, vessel thickness, and areas of stenosis. However, there is no good correlation between the wall changes and disease activity.
  • Intra-arterial angiography is the gold standard to detect the involved vessels. It is important to note that it only provides information about the vessel lumen but no details about the vessel walls.
  • PET scan technology is useful to detect areas of active arteritis. This can help differentiate between the inflammatory versus burned-out phases.

Treatment

Medications

  • Patients in the inflammatory phases (phase one and two) are started on pharmacologic therapy to prevent further development of stenosis or aneurysms. Patients are initially treated with oral glucocorticoids (prednisone 1 mg/kg) and then started on a steroid-sparing agent. Options include methotrexate, azathioprine, leflunomide, mycophenolate mofetil, cyclophosphamide, anti-TNF agents, and tocilizumab. Selection of steroid-sparing agent will depend on disease severity and medication tolerance. Hypertension should also be aggressively treated to prevent ischemia.
  • Surgical management is also available for amenable stenosis. These include angioplasty, endovascular stenting, and bypass surgery.

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