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Takayasu Arteritis: Causes, Symptoms & Treatment

Takayasu arteritis is a rare chronic inflammatory disease of the large blood vessels, primarily the aorta. It mainly affects young women and can lead to severe circulatory disorders.

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Things worth knowing about "Takayasu arteritis"

Takayasu arteritis is a rare chronic inflammatory disease of the large blood vessels, primarily the aorta. It mainly affects young women and can lead to severe circulatory disorders.

What is Takayasu Arteritis?

Takayasu arteritis (also known as Takayasu's arteritis or pulseless disease) is a rare, chronic inflammatory condition affecting the large blood vessels. It belongs to the group of large vessel vasculitides and primarily involves the aorta and its main branches, including the carotid arteries, renal arteries, and arteries supplying the arms and legs. The disease causes thickening and narrowing of vessel walls, which can severely impair blood circulation. It predominantly affects young women under the age of 40 and is most common in Asia, although it occurs worldwide.

Causes and Risk Factors

The exact cause of Takayasu arteritis has not yet been fully established. It is considered an autoimmune disease, in which the immune system mistakenly attacks and inflames the walls of large blood vessels. The following factors are discussed:

Genetic predisposition: Certain HLA genotypes (especially HLA-B52) are associated with an increased risk.
Infectious triggers: Infections, particularly with Mycobacterium tuberculosis, are considered possible triggering factors.
Immunological dysregulation: Abnormal activation of T-lymphocytes and macrophages plays a central role in vessel wall inflammation.
Sex and age: Women of childbearing age (15–40 years) are significantly more often affected.

Symptoms

The disease progresses in two phases. The early phase is dominated by general signs of inflammation, while the late phase is characterized by vascular symptoms.

Early Phase (Systemic Inflammatory Phase)

Fever and general malaise
Weight loss and night sweats
Fatigue and exhaustion
Muscle and joint pain

Late Phase (Vascular Occlusive Phase)

Absent or weakened pulse in one or both arms (hence the name pulseless disease)
Blood pressure differences between the two arms (>10 mmHg)
Dizziness, visual disturbances, or fainting due to carotid artery involvement
High blood pressure caused by renal artery involvement
Pain or weakness in the arms during physical activity (arm claudication)
Chest pain when the coronary arteries are affected

Diagnosis

Diagnosing Takayasu arteritis can be challenging, as early symptoms are non-specific. The following examinations are used:

Laboratory tests: Elevated inflammatory markers such as ESR (erythrocyte sedimentation rate) and CRP (C-reactive protein), often accompanied by anemia.
Imaging techniques: MRI angiography, CT angiography, or PET-CT to visualize vascular changes and inflammatory activity.
Doppler ultrasound: Non-invasive assessment of blood flow and vessel wall thickening.
Conventional angiography: Gold standard for precise visualization of stenoses and occlusions.
ACR/EULAR classification criteria: Standardized criteria to support the diagnosis.

Treatment

Treatment of Takayasu arteritis aims to control inflammation, prevent vascular damage, and avoid complications.

Drug Therapy

Corticosteroids (e.g., prednisolone): First-line therapy for remission induction; started at high doses and gradually tapered.
Immunosuppressants (e.g., methotrexate, azathioprine, mycophenolate mofetil): Used in addition to reduce steroid requirements and prevent relapses.
Biologics (e.g., tocilizumab, TNF inhibitors): Increasingly used in refractory cases or frequent relapses.
Antiplatelet agents (e.g., aspirin): To prevent vascular occlusion.

Interventional and Surgical Therapy

Percutaneous transluminal angioplasty (PTA) with or without stenting: For significant vessel narrowing.
Vascular surgery (bypass procedures): For severe stenoses or aneurysms that cannot be managed otherwise.

Prognosis and Disease Course

Takayasu arteritis frequently follows a relapsing course and may lead to permanent vascular damage despite treatment. However, with early and consistent therapy, the prognosis has improved significantly. Regular follow-up examinations are essential to detect relapses early and adjust treatment accordingly.

References

Hellmich B. et al. - EULAR recommendations for the management of large vessel vasculitis. Annals of the Rheumatic Diseases, 2020.
Kerr GS. - Takayasu's Arteritis. Rheumatic Disease Clinics of North America, 1995.
Dejaco C. et al. - Imaging in diagnosis and follow-up of large vessel vasculitis. Best Practice & Research Clinical Rheumatology, 2018.

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