AB0377 CLINICAL REVIEW OF 19 CASES WITH IDIOPATHIC AORTITIS – A SINGLE CENTRE STUDY
Authors: M. J. Villar, S. Sangle, D. D’cruz
Journal: Annals of the Rheumatic Diseases (2021)

Background: Aortitis is a group of disorders leading to inflammation in the aorta. When aortitis has no clinical evidence of systemic vasculitis, it is called idiopathic aortitis or clinically isolated aortitis (CIA). There is no consensus on the management of CIA. Objectives: Our purpose is to describe our cohort of patients with CIA and their response to treatment. Methods: This is a retrospective analysis of 19 patients with CIA. All records of patients with CIA were analyzed and demographic variables, comorbidities, symptoms, images, histology, treatment received, outcome and mortality were recorded. The description of quantitative variables was made using the median and the interquartile range (IQR). Results: Nineteen patients were analyzed. Diagnosis was made by imaging in 18 (94.7%), one patient was diagnosed by histology after aortic root surgery. Patient characteristics are detailed in Table 1. The median duration of follow- up was 38 months (IQR 43). Seven patients (36.8%) had constitutional symptoms including fever, weight loss, sweats and fatigue. 4 (21.05%) presented with back or abdominal pain for a mean duration of 3 months (SD 0.81) before the diagnosis. In 8 (42.1%) patients the diagnosis was made incidentally. All patients had negative treponema, hepatitis B, hepatitis C and Human Immunodeficiency Virus (HIV) serology. All patients had negative autoimmune serology included ANCA. Interestingly 4 (21.05%) had positive lupus anticoagulant without other manifestation of antiphospholipid syndrome. The type of aortic involvement was aortitis in 10 patients (52.6%), inflammatory aneurysm in 8 (42.1%) and dissection in 1 (5.2%). Seven (36.8%) patients had thoracic and abdominal aorta involvement, 6 (31.5%) only thoracic aorta and 6 (31.5%) only abdominal aorta. Aortic histology was obtained in 5 patients, 2 had necrotizing arteritis with giant cell pattern and 2 had lymphoplasmacytic pattern. Temporal artery biopsy was done in one patient and the result was negative for GCA. All patients received corticosteroids as a remission inducing agent, 12 (63.1%) received methotrexate and 2 (10.5%) mycophenolate. 2 patients died (10.5%). The median prednisone dose at the beginning was 20 mg (IQR 20) and at remission was 5 mg (IQR 20). 41.6% (5/12) of patients treated with steroids plus methotrexate were able to stop steroids without reactivation over a median follow-up time of 23.5 months (IQR 31). (Table 2.) Details of treatment, ESR and CRP pre and post treatment is shown in Figure 1. Seven patients (36.8%) had follow-up imaging, none of them showed active inflammation, new aneurysm, dissection or disease progression. Conclusion: Treatment of isolated aortitis with steroids and methotrexate was effective resulting in clinical and laboratory improvement and allowed cessation or decrease in steroids to 5 mg or less in 83% of patients. This therapeutic approach may be useful in patients with CIA. References: [1]Cinar, I., Wang, H., &amp; Stone, J. R. (2017). Clinically isolated aortitis: pitfalls, progress, and possibilities. Cardiovascular Pathology, 29, 23–32. Disclosure of Interests: None declared