Comparing treatment options for large vessel vasculitis

ABSTRACT

Introduction

Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are the major forms of large vessel vasculitis (LVV). Glucocorticoids represent the cornerstone of LVV treatment, however, 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. Therefore, conventional and biologic immunosuppressive drugs have been proposed to obtain a glucocorticoid-sparing effect.

Areas covered

We searched PubMed® using the keywords ‘giant cell arteritis/drug therapy’ and ‘Takayasu Arteritis/drug therapy’ OR ‘Takayasu Arteritis/surgery’ This review focuses on the management of LVV, based on the current evidence while highlighting the differences in terms of therapeutic management of TAK and GCA.

Expert opinion

Conventional disease modifying anti-rheumatic drugs, such as methotrexate or azathioprine, are recommended in association to glucocorticoids for selected GCA and all TAK patients. Two randomized placebo-controlled trials recently demonstrated the efficacy of tocilizumab in reducing relapses and cumulative prednisone dosage in GCA patients with newly diagnosed or relapsing disease. Observational evidence and two small randomized controlled trials support the use of TNF-alpha inhibitors and tocilizumab as glucocorticoid-sparing agents in relapsing TAK, albeit high-quality evidence regarding the management of TAK is still lacking.

KEYWORDS:

• Biologics
• giant cell arteritis
• glucocorticoids
• large vessels vasculitis
• takayasu arteritis
• treatment

Article highlights

• Both GCA and TAK are rare conditions, affecting the aorta and its major branches. High-dose GCs are effective in inducing remission in both conditions. However, relapses and recurrences frequently occur, when glucocorticoids are tapered or stopped, determining a prolonged exposure to steroid treatment and a subsequent increased risk of GCs-related side effects.

• Conventional disease modifying anti-rheumatic drugs, such as methotrexate or azathioprine, are recommended in association to glucocorticoids for selected GCA and all TAK patients. However, high-quality evidence regarding the efficacy and safety of csDMARDs in both diseases are still lacking.

• GIACTA trial demonstrated the efficacy of TCZ in newly diagnosed and relapsing GCA patients in terms of lower relapsing rate and cumulative GCs dose, leading to the approval of TCZ for the treatment of GCA. However, open questions on the use of TCZ in GCA remain and need to be clarified with further studies.

• TNF-α blockers are ineffective in newly diagnosed GCA patients.

• Observational studies strongly support the use of antiTNF-α agents and tocilizumab in TAK patients with relapsing disease despite treatment with GCs and cDMARDs. Further RCTs are needed to define the correct management for patients with TAK.

• To date, evidence on the efficacy of abatacept, ustekinumab, secukinumab, rituximab and JAK inhibitors in both GCA and TAK are too preliminary to advocate their routine use in clinical practice.

• Clinical trials assessing the efficacy and safety of bDMARDs and tsDMARDs in GCA and TAK patients are currently ongoing, paving the way for a better definition of the indications for biological treatment in patients with LVV in the next future.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.