Giant cell arteritis: what is new in the preclinical and early clinical development pipeline?

Introduction: Giant Cell Arteritis (GCA) is the most common systemic vasculitis worldwide. For decades, glucocorticoids have represented the mainstay of treatment, at the expense of toxic systemic effects owing to prolonged courses of high-dose treatment regimens. The search for effective drugs permitting lower glucocorticoid treatment regimens in GCA has been afrustrating one. The recent successful therapeutic application of tocilizumab, an interleukin-6 receptor inhibitor, has transformed the treatment of GCA and catalyzed research exploring other promising therapeutic targets.Areas covered: This review explores emerging drugs in preclinical and clinical development for the management of GCA, in addition to synthesizing data on the current standard of care therapeutic agents. Drug therapies were identified by search of MEDLINE and PubMed in addition to trials from registries (clinicaltrials.gov, clinicaltrialsregister.eu, pubmed.gov) from theyear 2010.Expert opinion: Tocilizumab has revolutionized the treatment of GCA. However, much remains to be learned about its optimal usage in GCA and asubstantial minority of pa tients do not achieve sustained glucocorticoid-free remission. Numerous exciting new agents are under investigation to fill this treatment gap in GCA, with the GM-CSF inhibitor mavrilimumab, and IL-12/23 blockade with ustekinumab providing promise through targeting the GCA pathogenic pathway in its proximal portion.