SUN-LB048 Takayasu Arteritis in a Young Woman with Type 1 Diabetes

Abstract

Takayasu arteritis (TAK) is a rare, chronic large-vessel vasculitis affecting primarily the aorta and its branches. It is uncommon in children and non-Asian ethnic groups. Co-incidence of TAK and type 1 diabetes (T1DM) is rare. We present a case of a 19-year-old Hispanic woman with longstanding T1DM and autoimmune thyroiditis who was diagnosed with TAK. She presented with T1DM and subclinical autoimmune thyroiditis at age 9. She was poorly adherent to diabetes care plan and admitted multiple times for DKA due to insulin omission. HbA1C average over 10 yrs was approximately 11%. At age 18 she presented to an outside hospital with severe abdominal pain and was diagnosed with appendicitis. Appendectomy was complicated by identification of an ischemic segment of small bowel, which was resected, and also for finding a thrombosis in the superior mesenteric artery (SMA). The SMA thrombosis was initially felt to be related to the prothrombotic state of poorly controlled DM1; she had a high atypical p-ANCA titer but erythrocyte sedimentation rate (ESR) had been normal. Her clinical course was complicated by severe cachexia resulting in multiple readmissions to address chronic abdominal complaints, malnutrition and diarrhea. She was also admitted for an episode of pyelonephritis during which she developed severe liver dysfunction of unclear cause. Diabetes remained poorly controlled. At age 19 she was admitted for DKA and weight loss. An abdominal bruit was identified on exam. There was no hypertension. Labs demonstrated a significant elevation of ESR of 101 mm/hr. A diagnostic evaluation was initiated. Imaging (CT and MR angiography) revealed chronic occlusion of the proximal SMA as well as vasculitis of the infrarenal aorta and inferior mesenteric artery. Conventional angiogram did not show vasculitis distal to the origin of the aortic vessels. Based on the constellation of findings including constitutional and gastrointestinal symptoms, the arterial bruit on exam, the high ESR, and findings on imaging, a diagnosis of large-vessel vasculitis (TAK) was made. She was treated with azathioprine and a taper of oral prednisone. Weight increased and ESR decreased initially down to 20 mm/hr, but ESR again increased (up to 40 mm/hr) with imaging evidence of ongoing vasculitis when steroids were weaned. This case adds to the sparse literature describing the association between TAK and T1DM. It highlights that TAK may occur in very young adults of non-Asian descent. It also showcases the difficulty in diagnosis of TAK, which may have a subacute and nonspecific presentation, in an individual with poorly controlled DM1 as well as SMA thrombosis, which has been described as an acute complication of DKA in case reports. The use of systemic glucocorticoids as a mainstay of TAK therapy may present additional challenges in an individual with DM1. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. s presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.