THU0286 MANAGEMENT OF TAKAYASU ARTERITIS: A SYSTEMATIC LITERATURE REVIEW INFORMING THE 2018 UPDATE OF THE EULAR RECOMMENDATIONS FOR THE MANAGEMENT OF LARGE VESSEL VASCULITIS

Abstract

Background European League Against Rheumatism (EULAR) recommendations on large vessel vasculitis (LVV) management were published in 2009.1 However, given recent advances, especially in therapeutics, an update was needed. Objectives To collect available evidence on management of LVV to inform the 2018 update of the EULAR management recommendations. Methods Two independent systematic literature reviews (SLRs) were performed, one focused on diagnosis and monitoring and the other on drugs and surgical treatments. Using a predefined PICO strategy, Medline, Embase and Cochrane databases were accessed, and eligible papers reviewed. This abstract reports the main results for Takayasu arteritis (TAK). Results on giant cell arteritis (GCA) are presented elsewhere. Results A total of 283 articles were selected from the SLRs. Relevant heterogeneity precluded a meta-analysis. The clinical pattern of TAK disease may differ according to age and gender. Males appear to have more complications than females. The presence of major complications (e.g. aneurysm formation), a progressive disease course and a weaker inflammatory response appear to relate to a more unfavorable prognosis, with survival rates as low as 43% at 15 years after diagnosis. Thus, follow up is essential, but there is no good quality evidence regarding the best timing and methods for disease monitoring. High-quality evidence to guide the treatment of TAK was not found. Glucocorticoids (GC) are widely accepted as first line treatment. However, many patients require doses above 10 mg prednisolone per day for disease control. Conventional immunosuppressive drugs and TNF-inhibitors appeared to be beneficial in case series and open-label studies, but high-quality evidence for their use is lacking. Tocilizumab showed modest signs of efficacy in a randomized controlled clinical trial, although the primary endpoint was not achieved. Revascularization procedures (aneurysm/stenosis) may be required and should be performed during stages of inactive disease. Conclusion Evidence to guide monitoring and treatment of TAK patients is predominantly derived from observational studies with low level of evidence. Therefore, higher-quality studies are needed in the future.