329. DISEASE SPECTRUM AND OUTCOMES IN PATIENTS WITH INFLAMMATORY AORTITIS: A SINGLE-CENTRE EXPERIENCE

Abstract

Background: Inflammatory aortitis is a rare condition that can occur in the context of primary systemic vasculitis, systemic autoimmune disease or in isolation. We describe the clinical spectrum of the disease and outcomes in patients with inflammatory aortitis treated in a large UK tertiary centre. Methods: We retrospectively reviewed all case records of patients with a diagnosis of Aortitis who attended our Vasculitis clinic at Louise Coote Lupus Unit at Guys and St Thomas' Hospital from 2005 till 2016. Data for patient demographics, clinical characteristics, serological status, histology and imaging findings, including FDG-PET scans were collected from the patient records. Results: 18 patients (45% women) were identified; mean age was 59 (Range 21-82 years). Most were Caucasians (83%) and the rest were Afro-Caribbean. Median follow up was 16 months (1 month-10 years). The clinical subgroups were idiopathic aortitis (8), Giant cell arteritis (4), Takayasu's arteritis (3), Granulomatosis with Polyangiitis (1), isolated thoracic aortitis (1) and retroperitoneal fibrosis (1). There was a diagnostic delay of 0-24 months (median 2m) from initial onset of symptoms to diagnosis, two patients were diagnosed incidentally on imaging. At initial presentation, the median CRP was 50 (5-255) and the median ESR was 56 (range 5-106). ANCA positivity was observed in 3 patients, negative in 14 and ANCA was not measured in 1 patient. Mean creatinine at presentation was 76 umol/L. Histology reports were available for 5 patients (28%) which showed IgG4 positive plasma cells in 1 case (aortic tissue), granulomatous changes on subglottic tissue in 1 patient with known GPA, 1 positive and 2 negative temporal artery biopsies. FDG- PET scans in 16 patients (89%) showed pan-aortitis in 5 patients, thoracic and subclavien artery uptake in 4, ascending aorta and arch involvement in 3 patients, infrarenal aortitis in 1 and 2 had negative FDG- PET scans. Mean initial treatment dose Prednisolone dose was 30mg (7.5-80); 9 patients received DMARD therapy (6 on Methotrexate, 3 on Azathioprine), while 2 had cyclophosphamide, 1 had rituximab and 1 had FEC-T chemotherapy for associated Lymphoma. Significant disease related complications included 2 patients needing thoracic stents and aortic root repair, 1 each with abdominal and thoracic artery dissection and one developed cardiac amyloidosis. 3 deaths were reported: 1 due to treatment noncompliance and 2 unrelated deaths. Conclusion: Inflammatory Aortitis has a varied clinical spectrum with underlying disease subgroups which can lead to diagnostic delay. Early diagnosis and treatment is needed to prevent potentially life threatening complications.