BackgroundTakayasu arteritis (TA) may present with a wide spectrum of symptoms common to other diseases leading to delayed or missed diagnosis.ObjectivesTo investigate the rate of undiagnosed clinically cases of TA, and provide a detailed analysis of the wrong diagnoses and the underlying causes of death.MethodsA 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.ResultsIn 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.ConclusionsOur 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 females' cases. It seems to be a trend to miss the clinical diagnosis of TA in male patients.Disclosure of Intere...

BackgroundCardiac involvement is a significant cause of the death and disability in systemic vasculitis. The comprehensive analysis of histopathologic findings in polyarteritis nodosa (PN) can help better understand mechanisms of excessive cardiovascular risk in patients with systemic vasculitis.ObjectivesTo investigate the variation of cardiac pathological findings in autopsy cases of PN.MethodsA retrospective analysis of cardiac pathological changes was performed in 37 autopsy cases of PN over period of 15 years. There were 28 males and 9 females, ranging from 23 to 85 years. The median age at disease onset was 35, 7 years; the median duration of disease was 2.1 years with a range of 2 month to 7 years.ResultsThe destructive-productive vasculitis of the coronary arteries have been found in 30 cases (81.1%) of PN. The vessels most affected were myocardial, and epicardial medium and small-sized arteries. Histologically, the remodelling of coronary artery tree in PN had a wide range of acute and chronic changes that likely were depended on the duration of the disease. There were three types of pathological findings: (1) arteritis with predominantly destructive changes; (2) arteritis with predominantly proliferative changes; (3) destructive-productive arteritis. The early phase of PN has been characterized by abnormalities of vessels wall from mucoid swelling to fibrinoid necrosis. The most common was panarteritis with involvement of all layers of the arterial wall, and inflammatory responses with intramural, and perivascular infiltrates, mainly composed of lymphocytes, and neutrophils. However, endo-, meso-, and periarteritis also were observed in some cases. The most remarkable lesions in coronary arteries were the nodules visible to the naked eye along coronary arteries. They were present in 9 cases (30%), and can be described as well-demarcated nodular thickening of the artery walls with size from 0.1 to 0.4 cm. The pathological basis of nodules are areas of focal inflammation, destruction and aneurysmal dilatations of artery wall. In cases of chronic course of PN, the intimal hyperplasia due to proliferation of endothelial cells have been observed. Interestingly, that this productive endarteritis with luminal narrowing of coronary arteries was common in patients who were exposed to different types of occupational xenobiotic (silica dust, pesticides, insecticides, solvents, heavy metals). In 5 (16.7%) cases, luminal occlusion due to intimal proliferation, fibrosis and thrombus formation led to the myocardial infarction, and death. Pathological manifestation of cardiac involvement in PN included the left ventricular hypertrophy due to renovascular arterial hypertension in 26 cases (70.3%). In addition, interstitial myocarditis was observed in 4 cases (10.8%).ConclusionsOur date suggest that cardiac involvement is common in polyarteritis nodosa (81.1%), and coronary vasculitis affecting medium and small-sized arteries with wide range of acute and chronic changes can be the life-threatening condition.Disclosure of ...

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BackgroundArterial occlusive disease is a significant cause of the disability due to lower limbs amputations. In addition to atherosclerosis, systemic vasculitides can present with progressive critical limb ischemia and could be misdiagnosed as atherosclerotic occlusive disease.ObjectivesTo investigate the pathological findings in vessels of the lower limbs amputated due to Takayasu arteritis (TAK), Buerger’s disease (BD) in comparison to atherosclerotic occlusive disease (AOD) and diabetic angiopathy (DA). Additionally, to develop an algorithm for clinico-histopathological differential diagnosis.MethodsThe specimens of vessels segments were obtained from the nine anatomical levels of amputated lower extremities in 132 patients, of which 30 were with TAK, 42 with BD, 30 with AOD and 30 with DA.ResultsIn cases of BD, the exudative-productive endo-mesoarteritis with intimal hyperplasia from stenosis to complete obliteration have been found in the lower leg and foot arteries. No morphological abnormalities were observed in proximal arteries above knee. The pathologic hallmark of BD was panphlebitis with intimal hyperplasia, most often in anterior and posterior tibial veins, vena dorsalis pedis and superficial vein. The most remarkable finding in cases of TAK was mesoarteritis and luminal narrowing due to the reactive intimal hyperplasia in proximal arterial segments. In approximately half of the cases, TAK was not diagnosed clinically and identified only based on the pathological examination. The most common incorrect clinical diagnosis was atherosclerotic occlusive disease. In seven cases of TAK, concomitant premature atherosclerosis of varying severity was observed. Interestingly, in our series of cases of TAK, males were most frequently affected. The correct clinical diagnosis of TAK was made in all females’ cases. It seems to be a trend to miss the clinical diagnosis of TAK in men. In cases of AOD, pathological examination revealed stenotic atherosclerotic plaques occurred most prominently in the tunica intima of femoral and popliteal arteries. The most common morphologic features in cases of DA were media calcinosis and fibrous lipid plaques both distal and proximal arterial segments. The algorithm for clinico-histopathological differential diagnosis of TAK, BD, AOD and DA consists of the following: a) the best diagnostic performance can be obtained with four-five specimens of the magistral vessels from the proximal and distal parts of the amputated limb and the superficial vein at the level of the upper third of the shin; b) using routine and special staining techniques (H and E, Masson’s trichrome stain for collagen fibres, Hart’s elastin stain); c) analysis of the demographic and clinical data; d) interpretation of pathologic changes in vessels of the lower limbs; e) making a pathologic diagnosis.ConclusionsThe current findings could contribute to the improving the differential diagnosis of Takayasu arteritis, Buerger’s disease, atherosclerotic occlusive disease and diabetic angiopathy.Disclosure of InterestN...