Flashcards in Vascular: Pathoma, BRS, First Aid Deck (164):
What is vasculitis?
Inflammation of blood vessel wall
BV wall has what 3 components?
1. endothelial intima
2. smooth muscle media
3. CT adventitia
Etiology of vasculitis is usually what?
Unknown or immune mediated.
Clinical features of vasculitis? 2
1. Nonspecific symptoms of inflammation
2. Symptoms of organ ischemia
Nonspecific symptoms of inflammation include? (4)
3. Weight loss
Symptoms of organ ischemia are due to what?
Luminal narrowing or thrombosis of inflamed vessels
Large-vessel vasculitis involves what?
Aorta and its branches
Medium vessel vasculitis involves what?
Muscular arteries that supply organs
Small vessel vasculitis involves what? (3)
Temporal Giant Cell arteritis is what?
Granulomatous vasculitis that classically involves branches of the carotid artery
Temporal giant cell arteritis is the most common vasculitis in what patients?
Older adults (especially females)
How does temporal giant cell arteritis present? 6
1. Unilateral Headache: Due to temporal artery
2. Visual distrubance: Ophthalmic artery (can go blind)
3. Jaw claudication
4. Polymyalgia rheumatica
5. Elevated ESR
6. Nodules along course of artery involved
Biopsy of temporal giant cell arteritis reveals what? 4
Inflamed vessel wall (fibrosis)
Lesions are segmental
Treatment of temporal giant cell arteritis is?
Takayasu arteritis is what?
Granulomatous vasculitis that involves aortic arch at branch points
What demographic has takayasu arteritis the most?
Young asian females
How does takayasu arteritis present?
1. Visual symptoms
2. Neurologic smptoms
3. Weak or absent pulse in upper extremity
4. ESR elevated
Treatment of takayasu arteritis?
What syndrome does takayasu arteritis produce?
Aortic arch syndrome
What are the two medium vessel vasculitises?
Polyarteritis nodosa is what?
Necrotizing vasculitis involving muscular arteries that supply organs.
What organ is spared in polyarteritis nodosa?
How does polyarteritis nodosa classically present?
Hypertension: Renal artery involvement
Abdominal pain with melena: Mesenteric artery
IHD: Coronary arteries
What serum antigen is associated with Polyarteritis nodosa?
Hepatitis B surface antigen
Early lesion of polyarteritis nodosa consists of what?
Transmural inflammation with fibrinoid necrosis
How do early lesions in polyarteritis nodosa heal?
What does this produce?
String of pearls appearance
Treatment for polyarteritis nodosa? 2
What happens if not treated?
Fibrinoid necrosis is clasically seen in what two scenarios?
1. Malignant HTN
Early lesions have what effect in polyarteritis nodosa?
What can result?
Weaken vessel wall
What mediates polyarteritis nodosa?
What organ causes most deaths in polyarteritis nodosa?
Kidneys: Vasculitis in arterioles and glomeruli and renal artery lesions
Kawasaki disease typically affects what demographic?
Asian children under 4 years old.
How does kawasaki disease present? 5
3. Red rash of palms and soles
4. Enlarged cervical lymph nodes
5. Strawberry tongue
What artery is dangerous for kawasaki disease?
Can lead to:
1. Thrombosis with MI
2. Aneurysm with rupture
Treatment for kawasaki disease is what?
2. IV immunoglobulin
Buerger disease is what?
Necrotizing vasculitis involving digits
How does Buerger disease present?
Autoamputation of fingers and toes
What is Buerger's disease heavily associated with?
Which leads into what is the best treatment for Buerger disease?
Cessation of smoking
What demographic typically presents with Buerger's disease?
Young Jewish men
What are the four small vessel vasculitis?
Wegener Granulomatosis is what?
Fibrinoid-Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys
Classic presentation of Wegener granulomatosis?
1. Middle-aged male
2. Sinusitis/nasopharyngeal ulceration
3. Hemoptysis with bilateral nodular lung infiltrates
4. Hematuria: Glomerulonephritis
Treatment of Wegener granulomatosis 2
What happens despite treatment
Wegener granulomatosis correlates with what serum level?
Circulating-Antineutrophil cytoplasmic antibodies (C-ANCA)
Biopsy of Wegener granulomatosis reveals what?
Large necrotizing granulomas (with giant cells) with adjacent necrotizing vasculitis
The TRIAD of Wegener granulomatosis?
1. Focal necrotizing vasculitis
2. Necrotizing granulomas in lung and URT
3. Necrotizing glomerulonephritis
Microscopic polyangiitis is what?
Necrotizing vasculitis of multiple organs especially lung and kidney?
How do microscopic polyangiitis symptoms differ from wegener's? 2
No nasopharyngeal symptoms
Treatment of microscopic polyangiitis? 2
Corticosteroids and cyclophosphamide
What serum level correlates with microscopic polyangiitis AND Churg-Strauss syndrome?
Churg-strauss syndrome is what?
Necrotizing granulomatous inflammation with eosinophils involving multiple organs especially lungs and heart
How does Churg-strauss syndrome present? 4
2. Peripheral eosinophilia
3. Palpable purpura
What is elevated in Churg-Strauss syndrome? 2
Henoch-Schoenlein Purpura is what?
Vasculitis due to IgA immune complex deposition
Henoch-Schoenlein Purpura is the most common vasculitis in what demographic?
How does Henoch-Schoenlein Purpura present? 5
1. Palpable purpura on buttocks and legs
2. GI pain and bleeding
3. Hematuria (IgA nephropathy)
4. Follows URT infection (Causes excess IgA)
How to treat Henoch-Schonlein Purpura?
Usually goes away on own but steroids if needed
What is Raynaud's disease?
Recurrent vasospasm of small arteries and arterioles
Symptom of Raynaud's disease?
What precedes it
Pallor/Cyanosis especially fingers and toes
Raynaud disease is most common in who?
Young, healthy women
What is Raynaud phenomenon?
Similar to Raynaud disease but is secondary to an underlying disorder
What is the underlying disorder in Raynaud's phenomenon? 2
SLE = Lupus
CREST syndrome = Systemic sclerosis
Hypertension is what?
Increased blood pressure
Does hypertension involve pulmonary or systemic circulation?
Systemic hypertension is defined how?
greater than 140/90
What is normal blood pressure?
less than 120/80
Primary hypertension is of what etiology?
What percentage of hypertensions?
Risk factors for primary hypertension? 6
Race (african americans higher, asians lower)
Lack of physical activity
What can primary hypertension lead to? 4
1. Retinal changes
2. Left ventircular hypertrophy --> Cardiac failure
3. Benign nephrosclerosis
Secondary hypertension is due to what?
Secondary to a known cause (5% of HTN cases)
What is most common cause of secondary HTN?
2 causes of renal hypertension?
1. Renal artery stenosis
2. Disorders of renal parenchyma
Renal artery stenosis has what effect?
Decrease blood to glomerulus --> JGA secretes renin -->--> Ang II -->
1. Vasoconstriction --> Increase peripheral resistance
2. Renal release of aldosterone --> Increase Na+ resorption --> Increase plasma volume
The effects of ATII leads to what?
HTN with increased plasma renin and unilateral atrophy (due to low blood flow)
Important cause of renal artery stenosis in elderly males?
Important cause of renal artery stenosis in young females?
Fibromuscular dysplasia: Developmental defect of BV wall leading to thickening of large and medium arteries
Endocrine disorders leading to hypertension? 6
1. Primary aldosterone/Conn Syndrome
3. Cushing syndrome
Other causes of HTN? 4
1. Coarctation of aorta (Upper body HTN)
2. Toxemia of pregnancy
3. CNS disorders: (brain tumors)
4. Amphetamines and steroids
What is benign HTN?
Mild or moderate elevation in blood pressure
Clinical effects of benign HTN?
Silent, damage over looooong period
What is most common HTN, benign or malignant?
Malignant HTN is defined how?
Blood pressure of >200/120
What demographic has malignant HTN the most?
Young black males
How does malignant HTN present? 4
1. Acute renal failure
4. Retinal hemorrhages
What does malignant HTN most likely result in? 3
What important renal change does malignant HTN produce?
Malignant nephrosclerosis in which glomeruli rupture forming a flea-bitten kidney
What is arteriosclerosis?
Thickened blood vessel walls in arteries making them hard
3 patterns of arteriosclerosis
3. Monckeberg medical calcific sclerosis
Atherosclerosis is what?
Intimal plaque that obstructs blood flow
What does the plaque in atherosclerosis consist of? (3)
1. Necrotic lipid core of choelsterol
2. Fibromuscular cap
What arteries are involved in atherosclerosis?
Large and medium size: abdominal, coronary, popliteal, carotid
What are the modifiable risk factors for atherosclerosis? 4
What are the nonmodifiable risk factors for atherosclerosis? 3
1. Age (increase with age)
2. Gender (Males and postmenopausal females)
What is the pathogenesis of atherosclerosis?
Damage to endothelium --> Lipids leak into intima --> Lipids oxidized --> Consumed by macrophages --> Form foam cells --> Inflammation/healing leads to deposition of ECM and proliferation of smooth muscle.
How does atherosclerosis begin and appear as?
When do these arise?
Fatty streaks: Yellow lesions of intima with lipid-laden macrophages
Early in life
Complications of atherosclerosis are how important?
Greater than 50% of all disease in Western countries
4 main complications of atherosclersosis?
1. Stenosis of medium sized vessels --> Impaired blood flow and ischemia
2. Plaque rupture with thrombosis --> MI/Stroke
3. Plaque rupture with embolization
4. Weakening of vessel wall results in aneurysm
Impaired blood flow and ischemia in turn can lead to what? (3)
1. Peripheral vascular disease
3. Ischemic bowel disease
What are fibrous plaques also known as?
What is difference in LDL and HDL in atherosclerosis?
LDL: Makes it worse
HDL: Makes it better
What is arteriolosclerosis?
Narrowing of small arterioles
Two divisions of arteriolosclerosis?
Hyaline arteriolosclerosis is caused by what?
Proteins leaking into vessel wall --> Vascular thickening
How does hyaline arteriolosclerosis appear on microscopy?
Hyaline arteriolosclerosis is the result of what?
1. Long standing benign HTN
Why does diabetes cause hyaline arteriolosclersosis?
Non-enzymatic glycosylation of BM --> Leaky BV wall
Hyaline arteriolosclerosis results in what? 2
1. Organ ischemia
2. Glomerular scarring --> Chronic renal failure
Hyperplastic arteriolosclerosis is what?
Thickening of vessel wall by hyperplasia of smooth muscle (concentric, onion-like)
Hyperplastic arteriolosclerosis is the consequence of what?
What does hyperplastic arteriolosclerosis result in?
1. End organ ischemia
2. Fibrinoid necrosis of vessel wall
3. Acute renail failure --> Flea-bitten appearance
What is Monckeberg medial calcific sclerosis?
Calcification of the media of muscular arteries; nonobstructure
Is Monckeberg medial calcific sclerosis clinically significant?
What arteries are most often affected in Monckeberg?
Radial and ulnar
What is aortic dissection?
Intimal tear with dissection of blood through mdia of the aortic wall
Where does aortic dissection occur?
Proximal 10 cm of the aorta (high stress)
Place where weakness of media is
Most common cause of aortic dissection?
What 3 things will also cause this?
Bicuspid aortic valve, Marfan and Ehlers-Danlos weaken CT in media
Hypertension causes aortic dissection how?
Hyaline arteriolosclerosis of vasa vasorum --> Decrease flow --> Atrophy of media
How does aortic dissection present?
Sharp tearing chest pain radiating to the back
Complications of aortic dissection?
Which is most common cause of death
1. Pericardial tamponade (most common)
2. Rupture with fatal hemorrhage
3. Obstruction of branching arteries --> Organ ischemia
Thoracic aneurysm is what?
Balloon-like dilation of thoracic aorta
What is thoracic aneurysm due to?
Weakness in aortic wall
How does thoracic aneurysm typically present? 3
1. Tertiary syphilis
What happens in thoracic aneurysm?
Endarteritis of vasa vasorum --> Luminal narrowing --> Decreased flow --> Atrophy of vessel wall
Hwo does aorta appear in thoracic aneurysm?
Major complication of thoracic aneurysm?
Dilation of aortic valve root --> Aortic valve insufficiency
Other complications of thoracic aneurysm?
1. Compression of trachea or esophagus
What is abdominal aortic aneurysm?
Where is it usually?
Balloon-like dilation of abdominal aorta?
Below renal arteries but above aortic bifurcation
What is abdominal aortic aneurysm due to mainly? 4
How does abdominal aortic aneurysm present?
Pulsatile abdominal mass that grows with time
What are the complications of abdominal aortic aneurysm? (3)
1. Rupture: Greater than 5cm
2. Compression of local structures (ureter)
What are Berry aneurysms?
Small saccular lesions seen in small arteries of brain, especially circle of Willis
Where do berry aneurysms grow?
Weakness at bifurcations of cerebral arteries?
Berry aneurysms are the most common cause of what?
Venous thrombosis/phlebothrombosis most commonly arises where?
Deep veins of lower extremities
What is Venous thrombosis/phlebothrombosis if it becomes inflamed?
Predisposing factors of Venous thrombosis/phlebothrombosis include?
1. Venous circulatory stasis
2. Obstructed venous return
Main result of Venous thrombosis/phlebothrombosis?
Embolism --> Pulmonary infarct
What are varicose veins?
Abnormally dilated and tortuous veins in superficial veins of lower extremeties.
Predisposing factors to varicose veins? (2)
1. Increased venous pressure (Pregnant, obese)
2. People that stand a lot.
What are five benign vascular tumors?
1. Spider telangiectasia
2. Hereditary hemorrhagic telangiectasia (OWR)
4. Glomangioma/Glomus tumor
5. Cystic hygroma
What is spider telangiectasia?
What is it associated with?
Dilated small vessel surrounding radiating fine channels
Hyperestrinism: Chronic liver or pregnant
What is heredtiary hemorrhagic telangiectasia?
Autosomal dominant condition with localized dilation and convolution of venules and capillaries of skin and mucous membranes.
When do hemangiomas present?
At birth, but regress through childhood.
Hemangioma is the most common tumor of what demographic?
Hemangioma has what characteristic symptom?
Port-wine stain birthmarks
Strawberry/Capillary hemangioma is what?
Who is it seen in?
Closely packed capillary-like channels
Infants (1/200 births)
Cavernous hemangioma consist of what? Cherry
Large cavernous vascular spaces in skin, mucosal surfaces, and organs
What disease usually causes cavernous hemangioma?
von Hippel-Lindau disease
What is a cherry hemangioma?
Benign capillary hemangioma of elderly
What is Sturge-weber disease?
Congenital vascular disorder that affects capillaries
How does sturge-weber disease manifest? 4
1. Port wine stain
2. Leptomeningeal angiomatosis
What is glomangioma/glomus tumor?
Small purple painful subungual nodule in a finger or toe
What is a cystic hygroma?
What disease is it associated with?
Cavernous lymphangioma that occurs in neck or axilla.
What is bacillary aniogmatosis?
Benign capillary skin papules in AIDS patients
What are the 4 malignant vascular tumors?
4. Kaposi sarcoma
What is hemangioendothelioma?
Intermediate between benign and malignant
What is hemangiopericytoma?
Tumor from pericytes and can be benign or malignant
What is a angiosarcoma?
Malignant proliferation of endothelial cells
What is angiosarcoma associated with? 2
2. Radioactive Thorotrast
What is liver angiosarcoma associated with?
What is Kaposi sarcoma?
Low-grade malignant proliferation of endothelial cells.
What disease is kaposi sarcoma associated with?
How does kaposi syndrome present?
Nodules on skin