21 Year old female presented with aoic arch aneurysm she underwent resection and the specimen was sent to histopathological examination. It showed all three layers were involved and giant cells were present. What is the probable diagnosis –
Correct Answer: Nonspecific aooaeritis
Description: Nonspecific aooaeritis Takayasu aeritis It is granulomatous vasculitis affecting medium and larger aeries. The disease has a strong predeliction for the aoic arch and its branches. Takayasus aeritis classically involves the "aoic arch". Histological changes Transmural involvement (all three layers are involved). - Media * Mononuclear infiltrates to granulomatous inflammation with giant cells - Adventitia --> Mononuclear infiltrates - Vasa vasorum --> Perivascular cuffing As the disease progresses collagenous scarring with admixed chronic infiltrates occur in all three layers of the vessel wall. Narrowing of the lumen occurs with or without thrombosis pathological changes in various organs reflect the compromise of blood. flow through the involved vessels. Aoic lesions of Takayasu aeritis share many attributes with Giant cell aeritis including clinical features and histology, The distinction is typically made on the basis of the age of the patient. > 50 years Giant cell Aeritis < 50 yrs Takayasu disease Clinical features of Takayasu's Aeritis :? Nonspecific features of Vasculitis - Fatigue. weight loss and fever Vascular symptoms (Due to luminal narrowing of the aoic arch). - Marked weakening of pulses in upper extremities It is also knowns as "pulseless disease" Q. - Reduced blood pressure in upper extremitiese Ocular disturbances - Visual defects - Retinal hemorrhages - Total blindness Takayasu disease can also cause Myocardial infarction. - Due to narrowing of the coronary aeries. Hypeension in 50% cases - Narrowing of renal aeries lead to hypeension in half the cases. Takayasu' Frequency of Aeriographic abnormalities and potential clinical manifestations of aerial involvement in Aery Potential clinical manifestation Subclan * Arm claudication, Raynaud's phenomenon Common carotid * Visual changes, syncope transient, ischaemic attacks stroke Abdominal Aoa * Abdominal pain, nausea vomiting Renal * Hypeension, renal failure, aoic insufficiency, congestive hea failure Veebral * Visual changes, dizziness Coeliac axis * Abdominal pain, nausea vomiting Iliac * Leg claudication Pulmonary * Atypical chest pain dyspnea Coronary * Chest pain myocardial infarction
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Surgery
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