takayasu s arteritis is a granulomatous vasculitis of unknown etiology that affects mainly the aorta and its branches . as a result of intimal fibroproliferation , segmental stenosis , occlusion , dilatation , and aneurysmal formation of the involved vessels
it is an uncommon disease , with an approximate incidence of 23 cases per year per million individuals and usually affects young female of asian ascendance during their second and third decades of life .
we describe a case of a previously healthy caucasian female whose takayasu s arteritis presented as an association of aortic and main left coronary aneurysms with severe aortic insufficiency .
a 26-year - old caucasian female was admitted to our hospital with a 3-week history of fatigue , malaise , exertion dyspnea , ortophnea and paroxysmal nocturnal dyspnea . there was a marked difference on the blood pressure measurement between the arms ( 140/90 mmhg on the right arm and 90/60 mmhg on the left ) .
the brachial pulse could not be felt on the left arm and a systolic murmur was heard on the left infraclavicular area . on cardiac auscultation , a diastolic murmur ( + + + + /vi )
was heard on the aortic area , and there were some crackles in the basal regions of both lungs .
her erythrocyte sedimentation rate ( esr ) was 64 mm on the first hour and the result of the serum c - reactive protein ( crp ) was 24 mg / dl ( normal : 06 mg / dl ) . based on the 1990 american college of rheumatology criteria , a diagnosis of takayasu s arteritis was made .
a high - resolution thorax computed tomography ( ct ) showed a 4-cm aortic aneurysm spanning the ascending and the proximal descending portions , as well as the aortic arch .
cineangiocoronariography confirmed the findings of the ct and also revealed a severe aortic insufficiency and a large main left coronary aneurysm ( figure 1 ) .
a three - day course of intravenous high - dose methylprednisolone ( 1000 mg ) was administered , as well as medications for the management of the heart failure ( diuretics , digoxin , angiotensin - converting enzyme inhibitor ) , which resulted in a remarkable improvement in general symptoms .
methotrexate was started at 15 mg / week as a steroid - sparing medication . 

takayasu s arteritis is primarily a chronic inflammatory vasculitis characterized by stenosis of large and medium sized arteries .
the coronary arteries are involved in about 10% of cases , but aneurysm formation , especially affecting the main left coronary , is a very rare finding .
destruction of the elastic fibers in the media of the vessel is the leading pathogenic mechanism of aneurysms formation . in some situations of massive aortic regurgitation
coronary aneurysms predispose to thrombus formation and acute myocardial infarction , even in patients receiving aspirin and/or warfarin . on the other hand , revascularization under inflammatory circumstances
carries a higher risk of complications , such as stenosis , suture line dehiscence and pseudoaneurysm formation . a case of successful surgical resection of a giant right coronary artery has been reported , even though the best surgical timing in takayasu s arteritis still remains controversial , as long as even when asymptomatic and with no serological evidence of current inflammation ( normal esr ) , up to 44% of these patients show some degree of histologic active disease . to our knowledge , this is the first report of an association of aortic and coronary aneurysms with severe aortic insufficiency in a takayas s arteritis patient .
the complexity of this case , allied to the absence of previously described medical interventions specific to this situation , certainly turns it into a therapeutic challenge .
proper follow - up is warranted , to get nearer to an accurate decision about the best moment to choose for a surgical approach , considering its costs and benefits when dealing with vasculitic vessels and its inner complications .
 takayasu s arteritis is a granulomatous vasculitis of unknown etiology that affects mainly the aorta and its branches . as a result of intimal fibroproliferation , segmental stenosis , occlusion , dilatation , and aneurysmal formation of the involved vessels 
 may develop . 
 it is an uncommon disease and usually affects young asian female patients during the second and third decades of life . 
 coronary arteries are exceptionally affected and coronary aneurysm formation is a very rare finding . 
 we describe a case of a previously healthy 26-year - old caucasian female whose takayasu s arteritis presented as a previously undescribed association of aortic and main left coronary aneurysms with severe aortic insufficiency . 