What is the initial treatment for large vessel vasculitis?

From the Guidelines

The initial treatment for large vessel vasculitis is high-dose glucocorticoid therapy, typically prednisone starting at 40-60 mg/day, which should be initiated immediately to induce remission and prevent complications. This recommendation is based on the most recent and highest quality study, which provides a level of evidence of 4 and a strength of recommendation of C 1. The study suggests that high-dose glucocorticoid therapy should be initiated promptly upon diagnosis to prevent complications such as vision loss in giant cell arteritis or vascular damage in Takayasu arteritis.

Key Considerations

• The high dose of glucocorticoids is typically maintained for 2-4 weeks until symptoms improve and inflammatory markers normalize, followed by a slow taper over many months 1.
• For patients with severe disease or those at high risk of glucocorticoid side effects, adjunctive therapy with methotrexate or tocilizumab may be added from the start 1.
• Regular monitoring of symptoms, inflammatory markers (ESR, CRP), and potential steroid side effects is essential during treatment 1.
• Aspirin may be recommended for patients with giant cell arteritis to reduce the risk of ischemic complications, although this is not universally agreed upon 1.

Adjunctive Therapy

• Methotrexate (15-25 mg weekly) or tocilizumab (162 mg subcutaneously weekly or 8 mg/kg IV monthly) may be used as adjunctive therapy in selected patients with giant cell arteritis or Takayasu arteritis 1.
• The use of adjunctive therapy should be individualized based on the patient's disease severity, risk of glucocorticoid side effects, and response to initial treatment 1.

Monitoring and Follow-up

• Regular follow-up and monitoring of disease activity in patients with large vessel vasculitis is recommended, primarily based on symptoms, clinical findings, and ESR/CRP levels 1.
• Imaging studies, such as ultrasound or MRI, may be used to monitor disease activity and response to treatment in selected patients 1.

From the FDA Drug Label

The primary efficacy endpoint was the proportion of patients achieving sustained remission from Week 12 through Week 52 Sustained remission was defined by a patient attaining a sustained (1) absence of GCA signs and symptoms from Week 12 through Week 52, (2) normalization of erythrocyte sedimentation rate (ESR) (to < 30 mm/hr without an elevation to ≥ 30 mm/hr attributable to GCA) from Week 12 through Week 52, (3) normalization of C-reactive protein (CRP) (to < 1 mg/dL, with an absence of successive elevations to ≥ 1mg/dL) from Week 12 through Week 52, and (4) successful adherence to the prednisone taper defined by not more than 100 mg of excess prednisone from Week 12 through Week 52.

The initial treatment for large vessel vasculitis, specifically Giant Cell Arteritis (GCA), typically involves a combination of:

• Tocilizumab (TCZ): administered subcutaneously or intravenously
• Prednisone taper: to reduce inflammation and prevent relapse The goal of treatment is to achieve sustained remission, defined by the absence of GCA signs and symptoms, normalization of ESR and CRP, and successful prednisone tapering 2.

From the Research

Initial Treatment for Large Vessel Vasculitis

• The initial treatment for large vessel vasculitis typically involves high doses of glucocorticoids, which are effective in inducing remission in both giant cell arteritis and Takayasu arteritis 3.
• Glucocorticoids represent the cornerstone of large vessel vasculitis treatment, but relapses and recurrences frequently occur when they are tapered or stopped, determining a prolonged exposure to glucocorticoids and a subsequent increased risk of glucocorticoid-related side effects 4.
• Conventional disease modifying anti-rheumatic drugs, such as methotrexate or azathioprine, are recommended in association to glucocorticoids for selected giant cell arteritis and all Takayasu arteritis patients 4.

Role of Biological Agents

• Biological agents, such as tocilizumab, have been proposed to obtain a glucocorticoid-sparing effect and have shown efficacy in reducing relapses and cumulative prednisone dosage in giant cell arteritis patients with newly diagnosed or relapsing disease 4.
• Observational evidence and small randomized controlled trials support the use of TNF-alpha inhibitors and tocilizumab as glucocorticoid-sparing agents in relapsing Takayasu arteritis, albeit high-quality evidence regarding the management of Takayasu arteritis is still lacking 4.
• Anti-tumor necrosis factor-α drugs seem effective in patients with refractory Takayasu arteritis, but have failed to do so in giant cell arteritis 5.

Treatment Guidelines

• The American College of Rheumatology/Vasculitis Foundation has published guidelines on the management of several forms of primary systemic vasculitides, including giant cell arteritis and Takayasu arteritis 6.
• The guidelines highlight the key recommendations, aspects where they diverge from other published guidelines, controversies, and areas of uncertainty 6.