Long-term outcome of 251 patients with Takayasu arteritis on combination immunosuppressant therapy: Single centre experience from a large tertiary care teaching hospital in Southern India

Abstract

Our strategy of upfront combination immunosuppressant therapy stabilised disease activity in 92.8% of patients, while 7.2% had true refractory disease. Relapse free survival was 66% at 5 years and 52% at 10 years. Damage progression was arrested in 68% and only 2 fatalities were observed. Initial steroid dose of 0.5mg/kg/day had similar efficacy as 1mg/kg/day dose.

Recent advances in the management of Takayasu arteritis

“…TA is a difficult disease to manage, with considerable controversy in differentiating activity from damage. GC forms the mainstay of medical therapy, with recent literature suggesting that a lower initial dose of 0.5 mg/kg/day of prednisolone may be adequate to control disease activity . Conventional DMARDs such as methotrexate, azathioprine, MMF, leflunomide and cyclophosphamide are commonly used as steroid‐sparing agents, although the evidence for their use is based on small case series, many of which are retrospective; while favorable clinical responses are reported in the majority, influence on angiographic progression is not widely studied.…”

“…Isolated reports exist on the use of rituximab and ustekinumab in refractory disease with mixed results. Vascular interventions in the form of endoluminal angioplasty (with or without stenting) or rarely, open vascular bypass surgery are useful in presence of critical vascular ischemia, although they should be performed only during inactive disease state along with continuation of peri‐operative immunosuppression for the best outcomes . The authors recommend the management of TA to be guided by ITAS‐2010 and ITAS‐A (CRP) and angiography; upfront immunosuppression with initial 0.5 mg/kg/day of prednisolone/equivalent dose of deflazacort alongwith mycophenolate 2 to 3 gram/day is the combination used successfully by the author.…”

“…TNF blockers may best be reserved for patients refractory to this line of management. Authors adopted this line of management keeping the background of a resource limited set up in mind and in view of their experience on favourable outcome of treatment …”

“…Traditionally, a dose of 1 mg/kg prednisolone daily (or equivalent) had been used for the initial 3–6 months with tapering once inflammation has been controlled . Recent literature suggests that an initial daily GC dose of 0.5 mg/kg/day prednisolone equivalent alongwith upfront 2nd line agents like mycophenolate facilitates tapering of steroids and may be associated with similar rates of early response without increasing the risk of relapse in the longer term . Patients receiving long‐term GC should be given due consideration for the prevention of osteoporosis as well as detection and management of other forms of toxicity associated with GC use such as diabetes mellitus and cataracts …”

“…A recently published Chinese cohort of 30 patients with TA treated with MMF 1.5–2.0 g/day (alone or in combination with methotrexate or azathioprine) for a median of 17 months reported favorable clinical responses in 24 patients (two continued to have active disease, four withdrew due to side effects), with retardation of vascular progression assessed by Doppler ultrasonography . In a long term follow up data published recently, authors mentions about edge in efficacy and safety of MMF, as compared to Methotrexate and Azathioprine in their series of 251 patient with TA . Therefore, MMF appears to be a safe and effective DMARD for stabilizing and controlling disease activity in TA.…”