FRI0341 Clinico-pathological discrepancies and causes of death in takayasu arteritis: a retrospective analysis of 60 fatal cases
Authors: O Zimba, M Bagriy
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Takayasu arteritis (TA) may present with a wide spectrum of symptoms common to other diseases leading to delayed or missed diagnosis. <h3>Objectives</h3> To investigate the rate of undiagnosed clinically cases of TA, and provide a detailed analysis of the wrong diagnoses and the underlying causes of death. <h3>Methods</h3> A retrospective clinicopathological analysis of sixty autopsy cases (52 males and 8 females aged 18 to 45 years) of TA over period of 11 years have been performed. The median age at disease onset was 25, 7 years. <h3>Results</h3> In 33 cases (55%), TA was not diagnosed during the clinical stages but only in autopsy. The most common incorrect clinical diagnosis was atherosclerosis of the aorta and its branches (celiac trunk, renal, mesenteric and iliac arteries) that has misdiagnosed in 14 cases (42.4%) of TA. TA was misdiagnosed as myocardial infarction/ischaemic heart disease in 5 (15.1%), perforated peptic ulcer in 3 (9.1%), polyarteritis nodosa in 3 (9.1%), and infective endocarditis in 2 cases (6.1%). There were other discrepancies in 6 cases (18.2%); in these cases, cerebral haemorrhage, rheumatic heart disease, pulmonary embolism, pheochromocytoma, chronic glomerulonephritis and lung cancer were the wrong clinical diagnoses. The leading position in the mortality structure due to TA belongs to septic shock that observed in 19 cases (31.7%) due to peritonitis/acute abdomen caused by mesenteric artery occlusion with subsequent intestinal necrosis in 12 (63.1%), lower limb gangrene in 6 (31.6%), and prosthetic aortic graft infection in 1 (5.3%). The second-leading cause of death was acute heart failure due to myocardial infarction, and renovascular arterial hypertension (25%). The third common cause of death was haemorrhagic shock in 14 cases (23.3%). The acute bleeding was caused by ruptured aortic aneurysm in abdominal part in 6 cases (42.8%) and ascending aorta with cardiac tamponade in 4 (28.6%). In 2 cases (14.3%), the source of hemorrhage was ulcers of gastrointestinal tract. The surgery has complicated by lethal bleeding in 2 cases (14.3%). In other patients, causes of death were cerebral haemorrhage in 6 (10%), renal failure in 3 (5%), respiratory failure in 1 (1.7%), and revascularization syndrome in 1 (1.7%). In one case, TA coexisted with scleroma of larynx, and asphyxia was direct cause of death. <h3>Conclusions</h3> Our date show a high number of cases of TA (55%) that were identified at autopsy but were not diagnosed clinically. It can be assumed that aortic atherosclerosis, myocardial infarction/ischaemic heart disease were the most common wrong diagnoses in TA. The leading causes of death in TA are septic shock, acute heart failure, and haemorrhagic shock. In addition, it should be noted that in our series of autopsy cases of TA, males (86, 7%) were most frequently affected compared with results other investigations. Interestingly, that the correct diagnosis of TA was established before death in all females9 cases. It seems to be a trend to miss the clinical diagnosis of TA in male patients. <h3>Disclosure of Interest</h3> None declared