Objective: To develop evidence-based guidelines for the management of giant cell arteritis (GCA) as a complement to guidelines in other areas of rheumatology, issued by the Swedish Society of Rheumatology.

Methods: A working group selected key areas for recommendations, reviewed the available evidence, and wrote draft guidelines. These were discussed and revised according to standard procedures within the Swedish Society of Rheumatology, including a one-day meeting open to all members. For key recommendations, the quality of evidence was assessed according to GRADE. The final guidelines were approved by the Society board in March 2018.

Results: The guidelines include recommendations on diagnostic procedures, pharmacological treatment, follow-up, and adjuvant treatment. Ultrasonography is complementary to temporal artery biopsy (TAB) in the diagnostic work-up. Other imaging techniques (magnetic resonance imaging and positron emission tomography/computed tomography) are important in evaluating large-vessel involvement. Glucocorticoids (oral, or intravenous in cases with ischaemic complications) remain the first line treatment for GCA. Addition of tocilizumab is recommended for patients with relapsing disease who meet five criteria, representing active disease that has been objectively verified by TAB or imaging. Tocilizumab may also be considered in patients with newly diagnosed GCA who are at major risk of severe glucocorticoid side effects. Based on current evidence, tocilizumab treatment for >1 year cannot be recommended.

Conclusion: These guidelines are based on current evidence and consensus within Swedish rheumatology. Following major developments in diagnostics and treatment of GCA, such guidelines are important for clinical practice, and should be updated on a regular basis.