Treatment – Takayasu Arteritis
Types of Treatments
- Glucocorticoids (such as prednisone) are usually used to suppress the systemic symptoms and arrest the progression of TA.
- Methotrexate (MTX)
- Azathioprine (AZA)
- Mycophenolatemofetil (MMF)
- Cyclophosphamide (CYC)
- Monoclonal anti-TNF agents
Immunosupressants are often used in combination with steroids.
If the disease has persisted and caused vascular damage leading to cerebral, coronary, renal, visceral, and limb ischemia of an organ, surgery may be necessary.
- Percutaneous transluminal angioplasty (PTA) with stenting along with therapy to alleviate the affected organ ischemia. This treatment is avoided in the active stage of the disease activity.
- Bypass surgery
- Balloon dilatation
- Unilateral nephrectomy
- TNF agents
Patients that do not respond to steroids or immunosupressants can be treated with biological therapies. Biological therapies have been used in adult patient populations, and in some cases, have shown a remission rate of 70-90% (Clifford & Hoffman, 2014). It should be noted that these methods of treatment are new and have not been extensively used in children to this date.
Monitoring disease progression / Outcome measures:
The systemic and vascular features of the disease should both be measured / monitored to understand the progression of the disease.
Outcome measures used include:
- Disease-extent index (DEI.Tak)
- Survey that is administered to monitor TA activity after diagnosis. It accounts for only clinical findings, (not imaging or biomarkers). It consists of 59 items assessing 11 domains including: systemic, cutaneous, mucous membranes, eyes, ENT (ear, nose, and throat), chest, abdomen, renal, nervous system, genitourinary, and cardiovascular domains.
- Indian Takayasu Clinical Activity Score (ITAS2010)
- Derived from DEI.Tak, ITAS2010 is used in monitoring disease activity. This test considers clinical manifestations as well as acute phase reactants such as ESR and CRP. The test contains 44 items, 33 of which focus on the cardiovascular system. Seven key items can be scored out of 2 while the rest can be scored out of 1. Higher scores are correlated with disease activity.
- Takayasu Arteritis Damage Score (TADS)
- Captures extent of damage caused by TA. There are 42 items for seven systems.
Physicians can also use our online Disease Activity Calculator to enter active items, determine the activity score, and print this form to add to the patient’s medical chart.
There is currently no one superior agent used to treat childhood TA. However, individual centres have provided treatment protocols with a high success rate.
In one study, 6 patients between the ages of 12 to 17 years were treated using the following:
|1. If the disease was on only one side of the diaphragm with no pulmonary disease||Oral steroids and oral MTX (12.5mg/m2/week)|
|2. If the disease was more widespread||Oral steroids and oral CYC (1.5-1.7 mg/kg/day) for a total of 12 weeks (max total dose of 150mg/kg) followed by oral MTX as above (12.5mg/m2/week) for 12 to 18 months|
|3. Life threatening disease||Pulse methylprednisolone given for 3 consecutive days|
|4. In patients whose APRs were normal||Oral prednisone started at 1 mg/kg/day for 4 weeks tapered to a maintenance dosage of 5 to 10 mg/day by 12 weeks.|
In the last visit of this study, 5 patients were all found to be symptom-free. During the course of this study, one patient died and one patient had a relapse stenosis of the graft after one year (though found symptom-free later). All patients were still on low-dose alternate-day corticosteroids as of June 1, 2006.
Figure 3: Flowchart outlining the treatment protocol of Takayasu arteritis
Hunter GG. Treatment of Takayasu arteritis. UpToDate: http://www.uptodate.com/contents/treatment-of-takayasu-arteritis?source=see_link. Accessed July 7, 2016.
Forsey J, Dhandayuthapani G, Hamilton MCK, Martin RP, Tizard EJ, Ramanan AV. Takayasu arteritis: key clinical factors for early diagnosis. Arch Dis Child, 2011. 96(5): p. 176-82.
Ozen S, Duzova A, Bakkaloglu A, Bilginer Y, Cil BE, Demircin M, Davin JC, Bakkalog M. Takayasu Arteritis in Children: Preliminary Experience with Cyclophosphamide Induction and Corticosteroids Followed by Methotrexate. J Pediatr, 2007. 150(1): p. 72-6.
Source: Ashish JM, Ruchika G, Sathish K, Debashish D. Childhood-onset Takayasu arteritis: an update. Int J Rheum Dis, 2016. 19(2): p. 116-26.
Source: Ozen S, Duzova A, Bakkaloglu A, Bilginer Y, Cil BE, Demircin M, Davin JC, Bakkalog M. Takayasu Arteritis in Children: Preliminary Experience with Cyclophosphamide Induction and Corticosteroids Followed by Methotrexate. J Pediatr, 2007. 150(1): p. 72-6.