Large-and small-vessel vasculitis are complex potentially life-threatening systemic autoimmune diseases that have recently been subjected to considerable immunologic and clinical research. Following the other reviews of this series, here we aim to summarise some of the most significant studies that have been recently published on the pathogenesis, clinical features and novel treatments of systemic vasculitis.
ObjectivesTo develop treat-to-target (T2T) recommendations in giant cell arteritis (GCA) and polymyalgia rheumatica (PMR).MethodsA systematic literature review was conducted to retrieve data on treatment targets and outcomes in GCA/PMR as well as to identify the evidence for the effectiveness of a T2T-based management approach in these diseases. Based on evidence and expert opinion, the task force (29 participants from 10 countries consisting of physicians, a healthcare professional and a patient) developed recommendations, with consensus obtained through voting. The final level of agreement was provided anonymously.ResultsFive overarching principles and six-specific recommendations were formulated. Management of GCA and PMR should be based on shared decisions between patient and physician recognising the need for urgent treatment of GCA to avoid ischaemic complications, and it should aim at maximising health-related quality of life in both diseases. The treatment targets are achievement and maintenance of remission, as well as prevention of tissue ischaemia and vascular damage. Comorbidities need to be considered when assessing disease activity and selecting treatment.ConclusionThese are the first T2T recommendations for GCA and PMR. Treatment targets, as well as strategies to assess, achieve and maintain these targets have been defined. The research agenda highlights the gaps in evidence and the need for future research.
BackgroundSince the development of the EULAR recommendations for the use of imaging in large vessel vasculitis (LVV) in 2017, new data has emerged in the field of imaging techniques and their application in the diagnosis and follow-up of patients with giant cell arteritis (GCA) and Takayasu arteritis (TAK).ObjectivesTo summarize the evidence on different imaging techniques for diagnosis, monitoring, and outcome prediction in LVV in order to inform a EULAR task force updating the recommendations for imaging in LVV.MethodsSystematic literature review (SLR) on studies published between 2017-2022 on ultrasound (US), magnetic resonance imaging (MRI), computed tomography (CT), positron emission tomography (PET)-CT/MRI and fluorescein angiography in patients with LVV (PROSPERO registration CRD42022360545). Eligible study designs included randomized controlled trials and observational studies but excluded case-controlled studies. Two reviewers independently performed data extraction, synthesis, and risk of bias assessment. For studies on diagnosis, meta-analyses were performed using data from both the original and updated SLR whenever possible. Pooled sensitivities and specificities were obtained by fitting random effects models for all studies and for studies with low risk of bias separately. Meta-analyses were performed in R version 4.2.1. using the “lme4” package. The description of observations without inferences and the heterogeneity of reported data precluded any meta-analysis for outcome prediction or monitoring.ResultsA total of 4696 references were identified. Thirty-eight studies on GCA (n=32), TAK (n=2), and GCA and TAK (n=4) were included through the update, adding up to eighty-one studies from both SLRs. Pooled sensitivities and specificities for US, MRI and PET-CT using a clinical diagnosis of GCA as the reference standard are depicted in Table 1. No studies on the diagnostic value of imaging techniques were found for TAK. The US evaluation of patients with suspected GCA, including the assessment of both cranial and extracranial vessels, showed a higher pooled sensitivity (95%CI) (89% [73%-96%] vs 70% [59%-79%]) and similar specificity (95%CI) (91% [83%-95%] vs 91% [84%-94%]) compared to only including cranial vessels. Studies on outcome prediction (n=5) and monitoring (n=10) reported change of signs of vasculitis along with disease activity and proposed composite scores comprising several vessel territories for US, MRI and PET-CT.ConclusionUS, MRI and PET-CT revealed a good performance for the diagnosis of GCA. Assessing both cranial and extracranial vessels with US leads to a higher pooled sensitivity with a similar pooled specificity compared to an assessment limited to cranial vessels.Table 1.Pooled sensitivities and specificities of diagnostic studies on GCA with clinical diagnosis as reference standardIndex testNumber of studiesPooled sensitivity (95%CI)Pooled specificity (95%CI)All studiesUS230.76 (0.66,0.83)0.91 (0.86,0.94)MRI80.82 (0.76,0.86)0.92 (0.84,0.97)PET-CT50.80 (0.70,0.87)0.91 (0.67,0.98)Low risk of bias studiesUS80.88 (0.83,0.92)0.96 (0.86,0.99)MRI30.81 (0.71,0.89)0.98 (0.89,1.00)PET-CT40.76 (0.67,0.83)0.95 (0.71,0.99)GCA, giant cell arteritis; MRI, magnetic resonance imaging; PET-CT, positron emission tomography – computed tomography; US, ultrasoundREFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsPhilipp Bosch Speakers bureau: Janssen, Grant/research support from: Pfizer, Milena Bond: None declared, Christian Dejaco Consultant of: AbbVie, Eli Lilly, Galapagos, Janssen, Novartis, Pfizer, Roche, Sanofi, Grant/research support from: AbbVie, Cristina Ponte Consultant of: AbbVie, Vifor, Pfizer, AstraZeneca, Grant/research support from: AbbVie, Vifor, Pfizer, AstraZeneca, Sarah Mackie Speakers bureau: Roche/Chugai, Vifor and Pfizer, Consultant of: Roche/Chugai, Sanofi, AbbVie, AstraZeneca, Louise Falzon: None declared, Wolfgang A. Schmidt Speakers bureau: Chugai, Novartis,Roche, and Sanofi, Consultant of: Chugai, GSK, Novartis, Roche,and Sanofi, Sofia Ramiro Consultant of: AbbVie, Eli Lilly, Galapagos, MSD, Novartis, Pfizer, Sanofi, UCB, Grant/research support from: AbbVie, Eli Lilly, Galapagos, MSD, Novartis, Pfizer, Sanofi, UCB.
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