COEXISTENT TAKAYASU ARTERITIS AND SARCOIDOSIS: A CASE REPORT

Abstract

SESSION TITLE: Tuesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Takayasu Arteritis (TAK) is a granulomatous large vessel vasculitis that predominantly affects the aorta, major aortic branches and pulmonary arteries resulting in pulselessness. Although initially described in Asian countries, the disease has a worldwide distribution with an annual incidence of only 2.6 cases per million in North America. Sarcoidosis is a systemic granulomatous disease of unknown cause that can affect every organ. We describe a rare case of TAK with coexistent sarcoidosis. CASE PRESENTATION: A 36-year-old Caucasian woman was referred for evaluation of mediastinal lymphadenopathy. At the age of 22, she underwent repair of ascending aortic aneurysm and was diagnosed with TAK on histopathology. She subsequently underwent multiple surgeries for repair of her aortic arch, descending thoracic aorta and vascular bypass procedures. Over the subsequent 14 years she had been treated with corticosteroids, cyclophosphamide, infliximab, adalimumab, and methotrexate. On presentation, she complained of night sweats for 6 months. She was receiving prednisone 10mg daily and methotrexate 25mg subcutaneously weekly. She denied any fever, weight loss, cough, sputum production or chest pain. Physical examination revealed normal vital signs and no abnormalities. Laboratory data were normal including lactate dehydrogenase, C-reactive protein and negative interferon gamma release assay. A chest CT demonstrated enlarged mediastinal lymphadenopathy. She underwent EBUS with TBNA of mediastinal lymph nodes. The histopathology showed non-necrotizing granulomas consistent with sarcoidosis. DISCUSSION: Including the present case, there are 24 confirmed cases of concomitant TAK associated with sarcoidosis reported in the literature. In the United States, the prevalence of TAK is 2.6 per million, and the prevalence of sarcoidosis is 60 per 100,000 adults. Therefore, if the development of TAK and sarcoidosis were independent events, the prevalence of individuals developing TAK and sarcoidosis concomitantly in their lifetime would be 1.56/100,000,000. Since 24 cases have been reported in the world literature since 1990, we suspect that concomitant development of these diseases in one patient did not occur by chance alone and relates to some unifying mechanism. One postulation for this observed association may be that in a fraction of these cases, the granulomatous inflammation may have been the result of TAK, and not have represented true sarcoidosis. As sarcoidosis is a disease of unknown etiology and connective tissue diseases may cause granulomatous inflammation, it is possible that the granulomatous inflammation may be induced by TAK rather than the development of sarcoidosis. CONCLUSIONS: Given the prevalence of these diseases, concomitant development of these two diseases is unlikely to be by chance alone and probably reflects a unifying mechanism. Clinicians should be aware of this association. Reference #1: 1. Hall S, Barr W, Lie JT, Stanson AW, Kazmier FJ, Hunder CG. Takayasu arteritis: a study of 32 North American patients. Medicine (Baltimore). 1985 Mar;64(2):89–99. Reference #2: 2. Judson MA. The Clinical Features of Sarcoidosis: A Comprehensive Review. Clin Rev Allergy Immunol. 2015 Aug;49(1):63-78. Reference #3: 4. Chapelon-Abric C, Saadoun D, Marie I, Comarmond C, Desbois AC, Domont F, et al. Sarcoidosis with Takayasu arteritis: a model of overlapping granulomatosiss. A report of seven cases and literature review. Int J Rheum Dis. 2018 Mar;21(3):740-745. DISCLOSURES: No relevant relationships by Biplab Kumar Saha, source=Web Response No relevant relationships by Aditi Saha, source=Web Response