Objectives: To integrate evidence-based data with expert opinion to provide guidance for the diagnosis, follow-up and treatment of giant cell arteritis (GCA).
Methods: A systematic literature review (SLR) was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. To structure the key clinical questions, the task force employed the Population, Intervention, Comparison Outcome (PICO) format. The Oxford system was subsequently applied to grade the quality of the evidence and determine the strength of each recommendation.
Results: This guideline provides 16 recommendations. We recommend the use of methotrexate in addition to glucocorticoids as first-line treatment in all patients with GCA without ischaemic symptoms. We recommend leflunomide, azathioprine and mycophenolate mofetil as alternatives in these patients if methotrexate is not tolerated. We recommend tocilizumab in GCA patients with ischaemic symptoms or with refractoriness to at least one conventional immunosuppressive. We also recommend upadacitinib as an alternative to tocilizumab in patients with low cardiovascular risk. To our knowledge, our recommendations are the first recommending upadacitinib as an alternative treatment option for the treatment of GCA.
Conclusions: The large RCTs assessing and comparing new effective options are still required in GCA. Assessment of the value of conventional immunosuppressives, which are more cost-effective options compared to biologic agents, is another research area in GCA treatment especially for developing countries. Upadacitinib seems to be a promising option in GCA. However, more real-life experience is needed to assess the safety of upadacitinib in the elderly population with especially high cardiovascular risk.