Investigational drugs in systemic vasculitis

ABSTRACT

Introduction: Treatment of systemic vasculitis is based on glucocorticoids (GC) in association with immunosuppressive therapy. There are still unmet needs, including earlier onset of response, more targeted therapies, reduction of relapse-risk and decrease of long-term GC and classic immunosuppressants toxicities.

Areas covered: In this review, we discuss investigational drugs in early phase clinical trials for induction of remission in vasculitis. We focus on ANCA-associated vasculitis, Behçet’s disease, giant cell arteritis, Takayasu arteritis, and cryoglobulinemic vasculitis. We performed a comprehensive review of articles published on pubmed and a review of clinical trials registered online (https://clinicaltrials.gov) for each vasculitis.

Expert opinion: Recent progress in the pathogenesis identifies new therapeutic targets. Some of these therapies are promising in GC sparing effects, in reducing relapse rate, and for their safety profile. These results need to be confirmed in large-scale phase III studies.

KEYWORDS:

• Vasculitis
• treatment
• biotherapies
• Behçet’s disease
• ANCA-associated vasculitis
• giant cell arteritis
• Takayasu

Article highlights

• GC is the cornerstone treatment of most vasculitides but there is concern about GC’s related morbidity and mortality

• Better understanding of vasculitides pathophysiology led to innovative-targeted therapies

• In AAV, complement targeted therapy is promising for inducing remission

• In GCA, anti-interleukins therapies (IL-6, IL12/23) are promising to reduce GC exposure and side effects

This box summarizes key points contained in the article.

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.

Additional information

Funding

This paper was not funded.