P181 Clinical assessment of radiologically defined clinically isolated aortitis in a single centre study

Abstract

Abstract Background/Aims Aortitis may occur in the absence of systemic vasculitis and is termed clinically isolated aortitis (CIA). Our purpose was to describe a cohort of patients with CIA and compare their characteristics with aortitis patients associated with rheumatic diseases. Methods We conducted a retrospective analysis of 32 patients with non-infectious aortitis evaluated in our centre. Online records were analysed. We included demographic variables, symptoms, laboratory data, imaging, treatment, outcomes, and mortality. The description of quantitative variables was made using percentages and medians with interquartile range (IQR). Results Out of 32 patients, 10 were classified as CIA and 22 had other inflammatory diseases (approx. 1:2 ratio) - fourteen had giant cell arteritis, 4 had Takayasu's arteritis, 2 with granulomatosis with polyangiitis, 1 each with IgG4 related disease and ankylosing spondylitis, classified according to the ACR/EULAR criteria for each disease. All patients underwent PET-CT imaging confirming isolated aortitis; none had an artery biopsy. Table 1 shows the main clinical characteristics. Among the CIA patients, the median duration of follow-up was 65 months (IQR 42-72). Three patients were asymptomatic and seven had constitutional symptoms (fever, weight loss, sweats, fatigue). Only one patient reported abdominal, back pain. The diagnosis was incidental in two cases. All patients had negative infection screen and serological markers for other rheumatologic conditions. Six (60%) patients had involvement only of the abdominal aorta, 2 had only thoracic aorta, and 2 had abdominal and thoracic aortitis. Six patients (60%) were treated with glucocorticoids (GCs) and methotrexate, 2 with GCs alone, 1 with GCs and mycophenolate mofetil, and 1 patient had resistant disease requiring tocilizumab. Four patients had follow-up PET-CT imaging: 2 showed reduction in FDG avidity, 1 had a stable tracer uptake, and 1 had no evidence of persistent aortitis. All the CIA patients demonstrated clinical and biochemical (ESR/CRP) improvement and at follow-up. Conclusion Our CIA patients had a male predominance, between the sixth and seventh decades, consistent with the previous literature. Aneurysms are more common in CIA patients than in those with GCA or TAK. There is no fixed protocol for CIA but conventional DMARDs and glucocorticoids are helpful. Disclosure S. Jain: None. M. Muñoz-Urbano: None. A. Khormi: None. S. Sangle: Other; Principle Investigator for HAVEN Trial. D. D'Cruz: Consultancies; GlaxoSmithKline, CSL Vifor. Grants/research support; Medical Research Council, National Institute for Health and Care Research. Other; Principal Investigator Clinical trials with Pfizer. Eli Lilly, AbbVie, Syneos, Idorsia.