Abstract: Aortitis and periaortitis include a heterogeneous group of conditions, which may be idiopathic or secondary to infectious diseases or non-infectious inflammatory disorders. Therefore, treatment differ considerably depending on the underlying cause. Our aim was to assess the administered treatment and therapeutic response in a wide series of patients with aortitis and periaortitis from a single reference University center. Retrospective and observational study of patients with aortitis and/or periaortitis from a referral center in Northern Spain. The main outcomes include: clinical remission (absence of clinical symptoms), complete remission (absence of clinical symptoms and analytical normalization), and imaging remission (absence of clinical symptoms and normalization of imaging alterations). Safety data during evolution were also evaluated. We included 134 patients (87 female/47 male) (mean age: 55.1 ± 9.1 years) with 132 aortitis and two periaortitis. The main diagnostic method was the positron emission tomography (PET), showing a non-specific increase in 18F-FDG uptake of the aorta with/without its major branches. The patients were treated with: glucocorticoids (n = 128; 95.5%), conventional immunosuppressive drugs (n = 68; 50.7%), biological therapy (n = 45; 33.5%), antibiotics (n = 3; 2.2%), and surgery (n = 6; 4.5%). After a mean follow-up of 39.2 ± 26.7 months, 72 patients (53.7%) experienced clinical remission, 64 (47.8%) complete remission and only 15 (11.2%) imaging remission. Aortitis and periaortitis are conditions that require an early diagnosis and treatment. Glucocorticoids are the first line of treatment in patients with non-infectious aortitis or periaortitis. 18F-FDG PET/CT scan is a useful imaging technique to evaluate the disease activity and response to therapy.