In the first case, Takayasu’s arteritis involved the aortic arch causing amaurosis fugax and symptoms from aortic regurgitation. The active inflammation of her Takayasu’s arteritis was treated medically before proceeding with surgical treatment of her severe aortic regurgitation.
In the second case, Takayasu’s arteritis primarily involved the abdominal aorta and renal vasculature. Her disease stabilized on combination therapy with azathioprine and infliximab, but recurred with tapering of therapy. Active involvement of the left renal artery at presentation caused hypertension and acute renal failure. Her renal function improved with immunosuppressive treatment and her hypertension was controlled with medical therapy and without the need for endovascular intervention.
Conclusion
These cases demonstrate some of the various manifestations of TAK as it affects different portions and branches of the aorta. Prompt diagnosis requires a knowledge of the potential clinical manifestations of large-vessel vasculitis. Optimal treatment of this complicated disease remains an area of active investigation. It requires close patient monitoring, trials of various immunosuppressive regimens and, at times, surgical or endovascular intervention.
Ashwini Komarla, MD, is a fellow in the Division of Rheumatology at the University of Pennsylvania in Philadelphia.
Michael George, MD, is also a fellow in the Division of Rheumatology at the University of Pennsylvania in Philadelphia.
Antoine Sreih, MD, is assistant professor at Penn Vasculitis Center, Division of Rheumatology at the University of Pennsylvania in Philadelphia.
Chris Derk, MD, MS, is associate professor and program director in the Division of Rheumatology at the University of Pennsylvania in Philadelphia.
Take Home Points
- TAK is a rare cause of large-vessel vasculitis, primarily affecting the aorta and its major branches.
- TAK most commonly affects women in their 20s or 30s.
- Patients may present with nonspecific inflammatory complaints and develop such features as absent peripheral pulses, bruits, blood pressure discrepancies between limbs, and limb claudication as specific arterial lesions develop.
- Diagnosis is made by angiography. Inflammation may be localized to a portion of the thoracic or abdominal aorta and its branches or may involve the entire aorta.
- Initial treatment is typically glucocorticoids at a dose of 1 mg/kg/day. Relapse is common with tapering of therapy.
- Other immunosuppressive agents that have been studied include methotrexate, anti-TNF therapy, and more recently tocilizumab and rituximab. The ongoing AGATA trial of abatacept in TAK is the first randomized controlled trial in TAK.
- A higher rate of restenosis or occlusion after grafting, angioplasty, or stenting has been observed in patients with TAK. Outcomes are improved if surgical procedures are performed after active inflammation is treated medically.
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