What is the workup for chest pain due to large vessel vasculitis (Giant Cell Arteritis or Takayasu Arteritis)?

From the Guidelines

The workup for chest pain due to large vessel vasculitis should begin with a thorough clinical evaluation, laboratory tests, and imaging studies, prioritizing the identification of giant cell arteritis (GCA) and Takayasu arteritis (TAK) as the primary causes of large vessel vasculitis, as recommended by the 2021 American College of Rheumatology/Vasculitis Foundation guideline 1. The initial laboratory tests should include inflammatory markers such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), which are typically elevated in vasculitis, as well as complete blood count, comprehensive metabolic panel, cardiac enzymes (troponin, CK-MB) to rule out myocardial infarction, and autoimmune markers like antinuclear antibodies, rheumatoid factor, and anti-neutrophil cytoplasmic antibodies. Imaging studies are crucial and should start with a chest X-ray, followed by CT angiography of the chest which can demonstrate vessel wall thickening, stenosis, or aneurysms, as suggested by the ACR Appropriateness Criteria for noncerebral vasculitis 1. For definitive diagnosis, consider FDG-PET scan which can show increased metabolic activity in inflamed vessel walls, and MR angiography provides detailed vascular imaging without radiation exposure, as recommended by the 2018 update of the EULAR recommendations for the management of large vessel vasculitis 1. In some cases, vascular ultrasound may be helpful for accessible vessels, and cardiac evaluation with ECG and echocardiogram should be performed to assess for complications like aortic regurgitation or myocardial involvement. If diagnosis remains uncertain, a vascular biopsy may be necessary, though this is often challenging for large vessels. Treatment typically involves high-dose corticosteroids (prednisone 1 mg/kg/day) initially, with addition of steroid-sparing agents like methotrexate (15-25 mg weekly) or tocilizumab (162 mg subcutaneously weekly) for maintenance therapy, as recommended by the 2021 American College of Rheumatology/Vasculitis Foundation guideline 1. Key points to consider in the workup and treatment of large vessel vasculitis include:

• Early diagnosis and treatment are essential to prevent serious complications such as aortic aneurysm, dissection, or occlusion
• The use of noninvasive imaging techniques, such as CT angiography and MR angiography, to assess vessel wall inflammation and damage
• The importance of monitoring inflammatory markers and adjusting treatment accordingly
• The potential need for surgical intervention in cases of severe vascular damage or complications.

From the Research

Workup for Chest Pain due to Large Vessel Vasculitis

• The workup for chest pain due to large vessel vasculitis typically involves a combination of clinical evaluation, laboratory tests, and imaging studies to confirm the diagnosis and assess the extent of disease.
• According to 2, glucocorticoid therapy is the treatment of choice for large vessel vasculitis, including giant cell arteritis and Takayasu arteritis.
• The use of conventional immunosuppressants such as methotrexate may be considered as adjunctive therapy to glucocorticoid therapy in cases of glucocorticoid-resistance or complications 2, 3.

Diagnostic Approach

• A thorough clinical evaluation, including a detailed medical history and physical examination, is essential to diagnose large vessel vasculitis.
• Laboratory tests, such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), may be used to assess inflammation and monitor disease activity.
• Imaging studies, such as angiography or positron emission tomography (PET), may be used to visualize the affected blood vessels and assess the extent of disease.

Treatment Options

• Glucocorticoids are the mainstay of treatment for large vessel vasculitis, but may be associated with significant toxicity, particularly with long-term use 3, 4.
• Conventional immunosuppressants, such as methotrexate, may be used to reduce the dose of glucocorticoids and minimize toxicity 2, 3.
• Biologic agents, such as tocilizumab, may be effective in treating relapsing giant cell arteritis and Takayasu arteritis 5, 6.
• Other treatment options, such as rituximab and leflunomide, may be considered in refractory cases or in patients with specific disease phenotypes 6.