SAT0538 Aortic involvement in relapsing polychondritis: a systematic review
Authors: M. Erdogan, S.N. Esatoglu, G. Hatemi, V. Hamuryudan
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Aortic involvement (AI) is an important complication of relapsing polychondritis (RP). Current literature is based on case reports and small case series. <h3>Objectives</h3> To delineate the clinical characteristics and outcome of AI in RP through a systematic literature review (SLR). <h3>Methods</h3> The SLR covered all English articles retrieved with relevant keyword combinations listed in PubMed until October 2017. Initially the titles and abstracts were screened by two investigators and articles considered to be relevant (involvement of the aorta and the aortic valve) were identified. Data extraction was done by the same investigators. <h3>Results</h3> The SLR revealed 352 papers of which162 were discarded at the first step and a further 114 after full reading. After excluding 5 articles reporting on the same patient, we finally had 71 papers reporting 97 patients. The sex distribution was identifiable in 79 patients of whom 39 were men and 40 were women. The median age at the first symptom of RP was 31.5 years [IQR:24–43 years], the median age at RP diagnosis was 36 years [IQR: 28–43 years]; median age at the diagnosis of AI was 37 years [IQR:29–49 years]. Median duration from first RP diagnosis to AI diagnosis was less than 1 year [Range:0–21]. AI was the presenting symptom in 3 patients. Seventy patients (73%) had involvement of thoracic aorta and 16 (23%) had involvement of the abdominal aorta. Other involved arteries were: coronary (8 patients), subclavian (5 patients), renal (3 patients), iliac (3 patients) and others (8 patients). Two patients had neurologic and renal involvement, respectively. The most common symptoms were dyspnea (40%), followed by chest pain (13%), abdominal pain (13%) and fever (9%). The diagnosis of AI was made during surgery in 6 patients and with different radiologic methods ranging from chest x-ray to PET-CT. All patients excluding 1 received corticosteroids either alone or in combination with classical immunosuppressives (cyclophosphamide, azathioprine, methotrexate, mycophenolate-mofetil) or biologics (infliximab, tocilizumab, adalimumab). The majority of the patients (53/87 patients (61%) underwent surgery including aortic graft replacement, coronary by-pass, aortic valve or mitral valve replacement operations. 16 patients died after a median follow up of 24 [IQR:11–43] months. The reasons for deaths were mainly vascular (acute myocardial infarction, heart failure, cardiovascular operation, abdominal aorta dissection, aortic rupture and acute aortic valvular dysfunction). Information on follow-up was known in 30 patients for a median of 18.5 months [IQR 10–48 months]. <h3>Conclusions</h3> AI is less frequently recognised but prognostically important complication of RP. Thoracic aorta is the most frequently involved site. Surgery is needed in the majority of patients. Medical treatment is empirical and is based on glucocorticoids and immunosuppressives including biologic agents. Despite treatment, mortality is high (18% during a median of 24 months). <h3>Disclosure of Interest</h3> None declared