Pathology- Disease Of Blood Vessels

Front 1

Vasculitis

Back 1

Inflammation of the blood vessels

2Types:
Immune Mediated
Infectious

Front 2

Antineutrophil Cytoplasmic Antibodies

Back 2

Ab's directed against neutrophil cytoplasmic Ag's

2Types:
MPO-ANCA- constituent of lysosomal granules (p-ANCA)
PR3-ANCA- constituent of neutrophilic granules (c-ANCA)

Pathogenesis: MPO and PR3 expressed on surface of activated neutrophils, Ab's cross react with MPO & PR3, subsequent exposure results in high titer, ANCAs then activate neutrophils and causes endothelial injury

Front 3

Giant Cell Arteritis

Back 3

Epi: older individuals >50
Eti: Mononuclear infiltrate (Giant Cells)
RF:
Path: Cell mediated immune response against Ag in the internal elastic lamina
Sx: Fever, Fatigue, Facial Pain, Headache, Temporal artery tenderness, Many of the head arteries can be involved (sometimes the Aortic Arch), Palpable nodules
Px:
Tx:
Note: MOST COMMON VASCULITIS

Front 4

Takayasu Arteritis

Back 4

Epi: occurs in women <40
Eti: Mononuclear infiltrate (Giant Cells)
RF:
Path: Most likely a cell-mediated immune response against Ag in the vessel wall
Sx: Decreased pulses and BP in upper extremities, Coldness or numbness in fingers, Increase in BP in lower extremities, Visual disturbances, Blindness, Dizziness, Focal weakness, Hemiparesis pulmonary hypertension, Typically involves Aorta and its branches and the pulmonary arteries
Px:
Tx:
Note:

Front 5

Polyarteritis Nodosa

Back 5

Epi: most common in young adults, episodic
Eti:
RF:
Path: Unknown, but causes segmental transmural inflammation with fibroid necrosis, thrombosis, aneurysms, fibrosis and firm nodules
Sx: Fever, Malaise, Hypertension, Uremia, Abdomenal pain, Myalgias, Peripheral neuritis, Skin lesions
Px:
Tx:
Note:

Front 6

Atherosclerosis

Back 6

Epi:
Eti: Increased cholesterol floating in the blood
RF: Hypercholesterolemia, FMHx
Path: Raised nodular lesion in the arteries that has fibrous cap (extracellular matrix) and necrotic core (cell debris, cholesterol crystals, and foam cells). Usually involved in Abdominal Aorta, Coronary Arteries, Carotid Arteries and Circle of Willis
Sx: Tends to be ASYMPTOMATIC
Px: Tendency to ulcerate causing superimposed thrombus or could cause aneurysm if involved in the media (weakness of vessel wall)
Tx:
Note: MOST IMPORTANT DISEASE OF THE ARTERIES

Front 7

Familial Hypercholesterolemia

Back 7

Epi:
Eti:
RF:
Path: Mutation of the LDL receptor gene required for uptake
Sx: Increased LDL particles in the plasma, Accelerated atherosclerosis
Px: Heterozygotes (2-3x increase in athero), Homozygotes (5-6x increase in athero)
Tx:
Note: MOST COMMON INHERITED DISORDER OF LIPOPROTEIN METABOLISM

Front 8

Response to Injury Hypothesis

Back 8

Chronic Endothelial Injury (Hyperlipidemia, Hypertension, Smoking, Viruses, Toxins, Immune reactions)

Endothelial Dysfunction (Increased permeability, Increased expression of leukocyte adhesion molecules, Monocyte adhesion & emigration)

Oxidized LDL, Macrophage activation, Smooth muscle emigration from media to intima

Macrophages and smooth muscle cells engulf lipid

Smooth muscle proliferation, Collagen & other ECM deposition, Extracellular lipid

Front 9

Hypertension

Back 9

Epi: Blacks, Older
Eti: Genetic Factors (Single Gene Mutations), Environmental Factors (Stress, Obesity, Physical inactivity, Heavy salt consumption)
RF:
Path: Mutation or Environmental factor that causes increased peripheral resistance and/ or increased blood volume
Sx: Systolic Pressure >139 mmHg, Diastolic Pressure >89 mmHg
Px:
Tx: Anti-hypertensives
Note:

Front 10

Atherosclerosis

Back 10

Epi:
Eti: Increased cholesterol floating in the blood
RF: Hypercholesterolemia, FMHx
Path: Raised nodular lesion in the arteries that has fibrous cap (extracellular matrix) and necrotic core (cell debris, cholesterol crystals, and foam cells). Usually involved in Abdominal Aorta, Coronary Arteries, Carotid Arteries and Circle of Willis
Sx: Tends to be ASYMPTOMATIC
Px: Tendency to ulcerate causing superimposed thrombus or could cause aneurysm if involved in the media (weakness of vessel wall)
Tx:
Note: MOST IMPORTANT DISEASE OF THE ARTERIES

Front 11

Familial Hypercholesterolemia

Back 11

Epi:
Eti:
RF:
Path: Mutation of the LDL receptor gene required for uptake
Sx: Increased LDL particles in the plasma, Accelerated atherosclerosis
Px: Heterozygotes (2-3x increase in athero), Homozygotes (5-6x increase in athero)
Tx:
Note: MOST COMMON INHERITED DISORDER OF LIPOPROTEIN METABOLISM

Front 12

Response to Injury Hypothesis

Back 12

Chronic Endothelial Injury (Hyperlipidemia, Hypertension, Smoking, Viruses, Toxins, Immune reactions)

Endothelial Dysfunction (Increased permeability, Increased expression of leukocyte adhesion molecules, Monocyte adhesion & emigration)

Oxidized LDL, Macrophage activation, Smooth muscle emigration from media to intima

Macrophages and smooth muscle cells engulf lipid

Smooth muscle proliferation, Collagen & other ECM deposition, Extracellular lipid

Front 13

Hypertension

Back 13

Epi: Blacks, Older
Eti: Genetic Factors (Single Gene Mutations), Environmental Factors (Stress, Obesity, Physical inactivity, Heavy salt consumption)
RF:
Path: Mutation or Environmental factor that causes increased peripheral resistance and/ or increased blood volume
Sx: Systolic Pressure >139 mmHg, Diastolic Pressure >89 mmHg
Px:
Tx: Anti-hypertensives
Note:

Front 14

Kawasaki Disease

Back 14

Epi: Children
Eti:
RF:
Path: Immunoregulatory defect leads to autoantibodies attacking endothelial and vascular smooth muscle
Sx: Fever, Erythema, Lymphadenopathy, Edema, Rash, Coronary arteritis that can lead to aneurysm, thrombosis or stenosis
Px: Usually self-limiting
Tx:
Note: Leading cause of acquired heart disease in children

Front 15

Microscopic Polyangiitis
(Leukocytoclastic Vaculitis)

Back 15

Epi:
Eti:
RF:
Path: Patients have MPO-ANCA causing a hypersensitivity reaction to drugs, micro-organisms, vaccines, or tumor agents
Sx: Palpable purpura, Hemoptysis, Hematuria and proteinuria, Abdominal pain and bleeding, Myalgias, Affects the arterioles, capillaries, and venules
Px:
Tx:
Note:

Front 16

Churg-Strauss Syndrome

Back 16

Epi: Rare
Eti:
RF: Associated with Asthma, Allergic rhinitis, and Eosinophilia
Path: Hypersensitivity reaction to some Ag usually exhibiting fibrinoid necrosis, eosinophilic infiltrates, and granulomas
Sx: Heart, Skin, GI, Kidneys
Px: Cardiac involvement accounts for almost half of all deaths
Tx:
Note:

Front 17

Wegener Granulomatosis

Back 17

Epi:
Eti:
RF:
Path: PR3-ANCA causing cell-mediated hypersensitivity reaction to inhaled Ag
Sx: Necrotizing granulomas in the respiratory tract, Focal necrotizing of granulomatous vasculitis affecting small arteries and micro vasculature, Necrotizing or crescentic GN, Bilateral nodular & cavitary lung infiltrates, Sinusitis, Ulcers in nasopharynx, Renal disease, Skin rashes, Myalgia
Px:
Tx:
Note:

Front 18

Thromboangiitis Obliterans
(Buerger Disease)

Back 18

Epi: Heavy cigarette smokers, <35 yrs, Common in Israel, Japan, India
Eti:
RF:
Path: Most likely a hypersensitivity reaction to a component of cigarette smoke, Involving medium and small arteries
Sx: Cold sensitivity & pain in extremities, instep claudication, ulceration of toes and fingers, Gangrene
Px: If stop smoking it is good
Tx: Smoking cessation
Note: