AORTIC ROOT REPLACEMENT FOR TAKAYASU-ARTERITIS ASSOCIATED WITH ULCERATIVE-COLITIS AND ANKYLOSING-SPONDYLITIS - REPORT OF A CASE

A 26-year-old man was admitted for treatment of congestive heart failu re resulting from aortic regurgitation. The patient had been on medica l treatment for ulcerative colitis (UC) since he was 14 years old and for ankylosing spondylitis (AS) since he was 20 years old. On admissio n, gradients of blood pressure among the extremities were observed. Ec hocardiography revealed marked dilation of the left ventricle (LV), hy pokinetic wall motion of the LV, slightly prolapsed aortic cusps with annular dilatation, and severe aortic regurgitation. Computed tomograp hic scans demonstrated an aneurysmal dilation of the ascending aorta a nd thickening of the descending and abdominal aortic wall. Digital sub traction angiography demonstrated an aneurysmal dilation of the ascend ing aorta; however, there was no clear evidence of steno-occlusive les ions in the brachiocephalic vessels. Blood studies showed positive inf lammatory signs and negative rheumatoid factor. HLA typing showed A2, 24(9), B27, 67, Cw1, 7, and DR1, 2. Based on these data, the diagnosis of Takayasu arteritis associated with UC and AS was made. Aortic root replacement was performed. Steroid therapy was restarted immediately after surgery. Histologic studies of the aortic wall showed findings c ompatible with Takayasu arteritis. The combination of these rare disea ses suggests that they have a common pathophysiologic background.