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Vasculitis and other Vascular Diseases. Chris Kwan PBL 28. POP QUIZ!. QUESTION 1. List AND explain the main categories of things that cause vasculitides. QUESTION 2. Mrs Sandy Jarse comes into the clinic with her visibly disgruntled husband, Hugh, along
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Vasculitis and other Vascular Diseases Chris Kwan PBL 28
QUESTION 1 • List AND explain the main categories of things that cause vasculitides
QUESTION 2 • Mrs Sandy Jarse comes into the clinic with her visibly disgruntled husband, Hugh, along • Sandy brought Hugh along for his long overdue checkups • You take a blood pressure reading on Hugh and find his blood pressure is 200/140mmHg • Suppose you somehow got an arterial biopsy sample, what structural change would be expected?
QUESTION 3 • Miss Lolita Goth, aged 40, presents into the emergency ward with severe chest pain • The paramedics tell you the following history: • Excruciating 9/10 chest pain (30 minutes ago) • Unknown what brought it on • Gave patient opioids to control pain • Severe shortness of breath (30 minutes ago) • Currently on oxygen therapy • Sweating • What other things would you ask for in the history to clarify the presentation?
QUESTION 4 • You want to perform an examination on Lolita Goth • The results are as follows: • BP: 150/80 • Pulse: 68bpm, regularly regular • Heart sounds dual, nil murmurs • Epigastric tenderness • What are some potential DDx’s? Please explain why you chose them
QUESTION 5 • The CT scans and chest echocardiograms reveal that Lolita has an aortic dissection • The dissection was shown to be located in the ascending AND descending aorta • How would you classify this aortic dissection, as according to the Stanford Classification AND the Debakey Classification?
Vasculitides • Sx: Local ischaemic or constitutional (e.g. fever, myalgia, arthralgia, malaise)
Arteriosclerosis • Refers to any hardening of the arteries • Three categories: • Atherosclerosis: atheromatous plaque • Arteriolosclerosis: vessel wall thickening • Hyaline = ‘benign’, essential HTN, thicker intima • Hyperplastic = ‘malignant’ HTN, onion-skin intima • Monckeberg: calcium deposits without thickening of intima
Raynaud Phenomenon • Refers to fingers/toes getting pale-blue during the cold (from exaggerated vasoconstriction) • Usually benign • HOWEVER, 2° Raynaud Phenomenon implies arterial insufficiency • SLE • Scleroderma • Atherosclerosis • Buerger’s Disease
Pathogenesis of Aortic Dissections • Intimal tear connects media with aortic lumen • A blood channel is then formed between and along the laminar planes of media • This channel forces the planes apart
Clinical Aspects of Aortic Dissection • Young patients: tend to have Marfan’s syndrome or any other defect that weakens walls • Older patients: associated with HTN, which causes medial haematoma • May also be iatrogenic (e.g. arterial cannulations, bypass surgery, catheters) • Advanced atherosclerosis and syphilis cause extensive scarring that actually protects against dissection
Clinical Aspects of Aortic Dissection • Signs/Sx: • Severe chest pain (usually anterior chest that radiates to back, then moves downwards) • Radio-radial delay, radio-femoral delay • Might not get a BP reading • Can leak into pericardial, pleural or peritoneal cavities (often fatal) • Retrograde dissection (i.e. tears towards aorta) cardiac tamponade, MI, aortic sufficiency
Morphologies of Aneurysms • True: the aneurysm is part of the dilated wall • False: the aneurysm is a haematomaand is not part of the vessel wall
Pathogenesis of Aneurysms • Arterial media weakens via 2 causes: • Atherosclerosis • Cystic medial degeneration of arterial media • Predisposing factors: • Trauma • Congenital defects (e.g. Berry aneurysms) • Infection (e.g. mycotic aneurysm) • Syphillis (but severe forms actually protect against dissections)
Signs and Sx of Aneurysms • Abdominal aorta: Epigastric pulsation >3cm diameter • Ruptured cerebral artery: Stroke-like Sx • Non-ruptured cerebral artery: Fatigue, reduced balance, perception, speech problems