From the Guidelines
Takayasu arteritis is a rare, chronic inflammatory disease that primarily affects the aorta and its major branches, causing narrowing, thickening, and potential blockage of these large blood vessels, typically affecting young women, particularly those of Asian descent, and often beginning between ages 15 and 30 1.
Key Characteristics
- The disease progresses in two phases: an early "active" inflammatory phase with symptoms like fever, fatigue, weight loss, and night sweats, followed by a chronic phase characterized by vascular complications such as decreased pulses, blood pressure differences between arms, claudication, and potential serious complications including stroke, heart failure, and aortic regurgitation.
- Diagnosis involves clinical evaluation, laboratory tests showing inflammation (elevated ESR/CRP), and imaging studies like angiography, CT, MRI, or ultrasound to visualize vessel abnormalities.
Treatment Approach
- Treatment aims to control inflammation and prevent vascular damage, primarily using corticosteroids like prednisone (starting at 0.5-1 mg/kg/day) as first-line therapy, with a strong recommendation for high-dose daily oral GC + non-GC immunosuppressive agent: MTX, AZA, +/-TNFi for active TAK not on immunosuppressive therapy 1.
- For steroid-resistant cases or to reduce steroid dependence, immunosuppressants such as methotrexate (15-25 mg weekly), azathioprine (2 mg/kg/day), or mycophenolate mofetil are added.
- Biological agents like tocilizumab or infliximab may be used in refractory cases.
- Surgical interventions like bypass grafting or angioplasty may be necessary for severe vascular stenosis.
Monitoring and Management
- Regular monitoring with clinical assessment, inflammatory markers, and vascular imaging is essential to track disease activity and treatment response.
- The treatment target is sustained remission (absence of clinical signs and symptoms of active Tak associated with normal acute phase reactants) plus ability to taper GCs to the specified target without relapse 1.
From the Research
Definition and Characteristics of Takayasu Arteritis
- Takayasu arteritis (TAK) is a chronic, granulomatous, large-vessel systemic vasculitis, mostly affecting the aorta and its large branches 2, 3, 4, 5, 6.
- It is characterized by autoimmune-mediated inflammation, vascular remodeling, and endothelial dysfunction 4.
- The disease progresses through three stages: active, chronic, and healing phase, each presenting distinct clinical features 4.
Diagnosis of Takayasu Arteritis
- Diagnosis can be challenging due to non-specific clinical manifestations and the lack of specific diagnostic tests 2, 4, 6.
- Various imaging modalities, such as angiography, ultrasound, and Doppler techniques, play a crucial role in the diagnosis of TAK by visualizing arterial involvement and assessing disease extent 4, 6.
- The 2022 ACR/EULAR classification criteria for Takayasu arteritis can be used for diagnosis 3.
Management and Treatment of Takayasu Arteritis
- Management involves a multidisciplinary approach, with disease-modifying anti-rheumatic drugs (DMARDs) as the cornerstone of medical therapy 4.
- Synthetic and biologic DMARDs are used to induce remission, control inflammation, and prevent complications 4.
- Corticosteroids are the treatment of choice, and if remission does not occur, methotrexate or other immunosuppressive agents can be added 2, 6.
- In cases of refractory disease activity, leflunomide, biologic agents such as TNF inhibitors, and tocilizumab may be tried 2, 3.
- Endovascular interventions, including balloon angioplasty, stent and stent graft replacement, or surgery, may be necessary in selected cases with persistent lesions that cannot be reversed with medical treatment 2, 5, 6.