A systematic literature review to inform the 2025 EULAR recommendations for the management of polymyalgia rheumatica and primary large vessel vasculitis: management of disease including relapse and complications.

OBJECTIVES

EULAR recommendations for polymyalgia rheumatica (PMR) and large vessel vasculitis (LVV) were published in 2015 and 2020, respectively. New evidence necessitated an update. We conducted a systematic literature review (SLR) to inform the 2025 EULAR recommendations for PMR and LVV.

METHODS

An SLR was conducted on MEDLINE, EMBASE, and CENTRAL on adults with PMR, giant cell arteritis (GCA), or Takayasu arteritis (TAK), using Medical Subject Heading terms related to management strategies on 31 December 2024 (PROSPERO: CRD42024560403). Eligible studies were randomised controlled trials (RCTs) and observational studies published since April 2014 (PMR) or December 2017 (LVV). EULAR 2024 and ACR 2024 abstracts were hand searched. Risk of bias (RoB) was assessed using Cochrane RoB-2 and the Newcastle-Ottawa Scale.

RESULTS

Of 141 included articles (30 RCTs), low RoB GCA RCTs showed superiority of secukinumab (phase II), mavrilimumab (phase II), and upadacitinib (phase III) vs placebo. Phase II trials of sarilumab and sirukumab were prematurely terminated. GiACTA secondary analyses reported similar tocilizumab efficacy across cranial and PMR symptoms, with better SF-36 and fatigue improvements than placebo. TAK RCTs reported similar effectiveness of methotrexate or mycophenolate (some concerns about RoB) and superiority of adalimumab over tocilizumab (high RoB). Phase III PMR RCTs showed tocilizumab and sarilumab superiority (low RoB) over placebo; smaller phase II studies supported rituximab (low RoB) and abatacept (some concerns about RoB). One high RoB RCT showed similar efficacy of tofacitinib and GCs.

CONCLUSIONS

Multiple phase II and phase III RCTs inform the efficacy and safety of IL-6i, IL-17i, Janus kinase inhibitors, and B cell depletion in GCA and PMR; robust TAK trials remain lacking.