Vascular Pathology Flashcards
How are arteries and veins connected?
By capillary beds, which have a low pressure
What is the problem with arteriovenous malformation?
Arteries and veins are connected by a tangle of worm-like vasculature
What does the arteriovenous malformation problem cause?
Causes a large shunt of blood form the arteries to the veins which leads to a high-output cardiac failure
What is a Berry aneurysm?
Focal abnormal dilation of an artery due to an underlying defect in the media
Where are berry aneurysms typically found?
Circle of Willis near major branch points
What are the risk factors for berry aneurysms?
- HTN
- Smoking
What other diseases are berry aneurysms seen in?
- Polycystic Kidney Disease
- Marfan Syndrome
- Ehlers Danlos Syndrome
How does a berry aneurysm rupture and what does it cause?
- Ruptures due to increased intracranial pressure like during sex or passing stool
- Most common cause of subarachnoid hemmorhage
What are some clinical features of a berry aneurysm?
- Worst headache of their life
- Neck pain
- Vomiting
- Double vision
- Seizures
- Loss of consciousness
What is a Mycotic aneurysm?
An aneurysm caused by an infected artery wall (from circulating organisms)
What is fibromuscular dysplasia?
A focal, irregular thickening in medium and large arteries
What arteries are affected by fibromuscular dysplasia?
- Carotid
- Renal
- Splanchnic
- Vertebral
What causes fibromuscular dysplasia? Who is most likely to develop it?
- Due to a developmental issue
- First degree relatives have higher incidence and is more frequent in women
What does fibromuscular dysplasia look like on an angiography?
Beads on a string
What is primary hypertension?
Often idiopathic and is 90-95% of cases
What are the unmodifiable risks of primary hypertension?
- Increased age
- Genetic factors
What are the modifiable risks of primary hypertension?
- Stress
- Obesity
- Physical inactivity
- Increased salt consumption
What are some kidney issues that cause secondary hypertension?
- Renovascular disease
- Renal artery stenosis
- Polycystic kidney disease
What are some endocrine issues that cause secondary hypertension?
- Primary aldosteronism
- Cushing syndrome
- Pheochromocytoma
What are some cardiovascular issues that cause secondary hypertension?
- Coarctation of the aorta
What does renal artery stenosis cause? What will be the clinical sign?
- Hypertension, decreased GFR, and chronic kidney disease
- Will hear an abdominal bruit
What does coarctation of the aorta cause? What will be the clinical sign?
- It is a narrowing of the aorta
- Causes HTN in the UE’s and hypotension in the LE’s
What are the effects of hypertension on the heart or aorta?
- Cardiac hypertrophy and CHF
- Ischemic heart disease
- Aortic dissection
What are the effects of hypertension on the kidneys?
- Renal dysfunction and failure
What are the effects of hypertension on the brain and eyes?
- Multi-infarct dementia and cerebrovascular hemorrhage/stroke
- Increased intracranial pressure/papilledema
- Retinopathy
What is hyaline arteriolosclerosis?
- Generalized and severe in chronic HTN
- There is an increase in smooth muscle matrix synthesis and plasma protein leakage across damaged endothelium
What does hyaline arteriolosclerosis lead to?
Could lead to a hyaline nephrosclerosis causing an impairment of the renal blood supply and cause ischemic glomerulosclerosis
What is defined as a hypertensive crisis?
- Systolic >180-200 OR
- Diastolic >120
What is a hypertensive emergency?
When there is a hypertensive crisis with end organ damage
What is hyperplastic arteriolosclerosis? When does it occur?
- Smooth muscle forms concentric lamellations showing “onion skinning” (could be accompanied with fibrinoid deposits and vessel wall necrosis)
- Occurs in severe HTN
What is atherosclerosis?
Hardening of the arteries
What are the three types of atherosclerosis?
- Arteriolosclerosis
- Atherosclerosis
- Monckeberg medial sclerosis
What is arteriolosclerosis? What does it affect? What does it cause?
- Hyaline and hyperplastic arteriolosclerosis
- Affects small arteries or arterioles
- Causes downstream ischemic injury
What is atherosclerosis? What does it cause?
- Atheromatous plaque formation
- Causes stenosis/occlusion occurs; plaque rupture; aneurysm
What is Monckeberg medial sclerosis? Who does it affect?
- Calcification of muscular arteries; specifically the internal elastic membrane
- Is an age related degenerative process that does not cause narrowing of the lumen
What are the non modifiable risk factors of atherosclerosis?
- Genetic: multifactorial inheritance
- Increased age: 40-60 years old
- Gender: women on birth control or other hormones have protective effect
What are the modifiable risk factors of atherosclerosis?
- Hyperlipidemia
- HTN
- Smoking
- Diabetes
- Metabolic syndrome
- Inflammation
- Hyperhomocysteinemia
- Lipoprotein A
- Lack of exercise
- Competitive/stressful lifestyle
- Obesity
What is the pathogenesis of atherosclerosis?
- Chronic endothelial injury occurs (like increased permeability or leukocyte adhesion)
- Macrophages are activated and smooth muscle is recruited
- Macrophages and smooth muscle cells engulf lipid causing fatty streaks
- Smooth muscle proliferates, collagen and other extracellular matrix is deposited causing a fibrofatty atheroma
What are the growth factors that cause the smooth muscle proliferation in atherosclerosis?
- PDGF
- Fibroblast growth factor
- TGF-a
What are patients most likely to suffer from as they get older with atherosclerosis?
- Aneurysm and rupture of the vessel due to mural thrombosis, embolization, or wall weakening
- Occlusion by thrombus due to plaque rupture, plaque erosion, plaque hemorrhage, mural thrombosis, or embolization
- Critical stenosis due to progressive plaque growth
What is an aneurysm?
- Excessive localized abnormal dilation of a blood vessel or ventricular wall
What is a true aneurysm?
- Intact, but thinned, muscular wall at the site of dilation
What is a false aneurysm?
- Defect through the wall of the vessel communicating with an extravascular hematoma that freely communicates with the intravascular space
What is an arterial dissection?
- Occurs when blood enters a defect in an arterial wall and tunnels between its layers
What is the mutation in Marfan Syndrome?
- FBN1 fibrillin gene
What does the mutation in Marfan Syndrome cause?
- Causes an increased in TGF-B which results in elastic tissue weakening
What is the mutation in Ehlers Danlos syndrome?
- Collagen
What is the clinical presentation of Ehlers Danlos syndrome?
- Tall
- Hyperelastic and fragile skin
- Joint hypermobility
- Lens subluxation
- Abnormal wound healing
- Widened scars, bruising
- Mitral valve prolapse
- Kyphoscoliosis in some areas
- Rupture of colon, cornea, and large arteries
What is tertiary syphilis?
Sexually transmitted disease, spirochete T. pallidum
When does tertiary syphilis occur?
More than 5 years after primary infection
What does tertiary syphilis do?
- Cause obliterative endarteritis of vasa vasorum which causes thoracic aneurysm
- Aortic valve regurgitation can occur
Who are most likely to be seen with an abdominal aortic aneurysm?
- Older white males who smoke
Where does the AAA occur?
- Below the renal arteries and above the split
What does an asymptomatic AAA present with?
- Pulsatile abdominal mass
- Incidental finding or during workup of PVD
What does a symptomatic AAA present with?
- Nonruptured will have pain in the back and abdomen
- Ruptured will have severe acute pain, a pulsatile abdominal mass, and hypotension
What is the primary risk for a thoracic aortic aneurysm?
- HTN
What are some other causes of thoracic aortic aneurysm?
- Syphilic aortitis
- Connective tissue disease (marfan)
- Vasculitis
What are some clinical symptoms of a thoracic aortic aneurysm?
- Breathing difficulties (respiratory tree involved)
- Dysphagia (esophagus involved)
- Cough (recurrent laryngeal nerve involved)
What happens in an aortic dissection?
- Intimal tear with blood filled channel separating the media
What can an aortic dissection progress to?
- A rupture with excruciating pain in the anterior chest
What is the triad of an aortic dissection?
- Thoracic pain
- Pulse abnormalities
- mediastinal widening on CXR
What are some causes of an aortic dissection?
- HTN
- CT disorders (Marfan, Ehlers Danlos)
What is the most common type of aortic dissection?
- Debakey I
What kind of vessels does Giant cell arteritis affect?
Large to small arteries in the head (temporal, vertebral, ophthalmic)
What are the symptoms for giant cell arterities?
- Headache and flu like symptoms
Who is most likely seen with giant cell arteritis?
- Older men (>50)
What can occur if a diagnosis is not made in a reasonable time?
- Vision loss can occur with ophthalmic artery involvement
What Abs show up in giant cell arteritis?
- Anti-smooth muscle
- Anti-endothelial
What is Takayasu Arteritis?
- A granulomatous autoimmune vasculitis of large to medium arteries
What happens in takayasu arteritis?
- There is a thickening of the aorta or major branch vessels
Who does takayasu arteritis affect?
- Middle age adults (<50)
What are the clinical symptoms of takayasu arteritis?
- Weakening of the UE pulses
- Ocular disturbances
- +/- fatigue
- HTN
- Fever
- Weight loss
What size vessels does polyarteritis nodosa affect?
- Small to medium vessels
Who is most likely to have PAN?
Young adults; 1/3 have HepB
What do the vessels look like in PAN?
- Shows necrotizing, segmental inflammation with infiltration of eosinophils, lymphocytes, neutrophils, and macrophages
Which specific vessels does PAN affect?
- Renal
- Heart
- Liver
- GI tract
What is the clinical presentation of PAN?
- Rapidly accelerating HTN
- Abdominal pain and bloody stools
- Myalgias
- Peripheral neuritis
What can be used to cure PAN?
- Immunosuppresion
Who does Kawasaki Disease affect?
Illness in infancy or early
What size vessels does kawasaki disease affect?
Large to medium vessels
What is the trigger for kawasaki disease?
- Viral infection
- Activated T cells and monocytes/macrophages
What is the clinical presentation for kawasaki disease?
- Febrile
- Strawberry tongue
- Conjunctivitis
- erythema of palms and soles
- Cervical lymphadenopathy
- Genital rash
What vessels does Churg-Strauss affect?
- Small vessels
How does Churg-Strauss affect the body?
- Necrotizing vasculitis associated with asthma, hypereosinophilia, and lung infiltrates
What does Churg-Strauss resemble? How is it different?
- Inflammation resembles PAN but has addition of eosinophils and granulomas
What organ systems are involved in Churg-Strauss?
- Skin (palpable purpura)
- GI tract bleeding
- Renal disease
How does Churg-Strauss affect the heart?
- Cardiomyopathy/myocarditis and infarction; heart is involved in 60% of patients and may cause death in half with syndrome
What size vessels does Wegener’s Granulomatosis affect?
- Small to medium vessels
Who does Wegener’s granulomatosis affect generally?
- 40 yo males
What is the immune reaction process of wegener’s granulomatosis?
- T-cell mediated hypersensitivity due to inhaled environmental or microbial material
Which ANCA is wegener’s granulomatosis positive for?
PR3-ANCA
Which ANCA is Churg-Strauss positive for?
MPO-ANCA
What size of vessels does Bechet’s disease affect?
- Small to medium
What are the clinical signs of Bechet’s disease?
- Oral aphthous ulcers
- Genital ulcers
- Uveitis
Which HLA is associated with Bechets?
HLA-B51
What is required for diagnosis with Bechet’s disease?
- Neutrophilic vasculitis
What is Bechet’s disease treated with?
- Steroids and TNF antagonists
What size of vessels does Buerger Disease (thromboangiitis obliterans) affect?
- Acute and chronic vasculitis of small and medium vessels
Which arteries specifically does Buerger disease affect?
- Tibial and radial arteries (hands and feet)
Who is Buerger disease most prevalent in?
- Heavy smokers <35 years old
Where is buerger disease most seen?
- HLA haplotypes in Israel, India, and Japan
What is the progression of the buerger disease?
- Thrombosis
- Vascular insufficiency
- Tissue death
What is raynaud’s phenomenon?
- Vasospasm of small arteries
What causes raynaud’s phenomenon?
- Cold or emotion
Why is raynaud’s phenomenon called “red, white, and blue”?
There is proximal vasodilation, central vasoconstriction, and distal cyanosis
What is primary raynaud’s phenomenon?
- Symmetric involvement of digits
- Occurs in young women
- Episodic with sporadic remission
What is secondary raynaud’s phenomenon?
- Asymmetric involvement of digits
- Is a component of another disease like SLE, scleroderma, or thromboangiitis obliterans
- Worsens with time
What is a myocardial vessel vasospasm?
Excessive vasoconstriction of myocardial arteries or arterioles which may cause ischemia or infarct
What are some causes of myocardial vascular contraction?
- High levels of vasoactive mediators (cocaine, increased caffeine intake, epi or NE)
- Elevated thyroid hormones
- Autoantibodies and T cells in scleroderma
What is takotsubo cardiomyopathy?
- AKA broken heart syndrome
- Emotional distress causes a surge of catecholamines
What causes varicose veins?
Sustained intraluminal pressure causing valvular incompetence
How does stasis dermatitis occur?
Caused by stasis, congestion, thrombus, edema, pain, and ischemia
What is the most common cause of portal hypertension?
Cirrhosis
What does portal hypertension lead to?
- Esophageal varices
- Splenomegaly
- Hemorrhoids
- Distended superficial abdominal veins
What are some causes of superior vena cava syndrome?
- Lung carcinomas
- Lymphomas
- Aneurysms
- AV fistula
- Fibrosis
What do obstructions in superior vena cava syndrome cause?
- Marked dilations of the veins in the head, neck, chest wall, and arms with cyanosis
- Facial swelling and neurologic manifestations
What can happen with involvement of the pulmonary vessels in SVC syndrome?
- Respiratory distress
What causes inferior vena cava syndrome?
- Hepatocellular carcinoma, renal cell carcinoma or thrombosis (DVT)
What do obstructions in inferior vena cava syndrome cause?
- Marked lower extremity edema
- Distention of superficial lower gastric veins
What is lymphangitis?
Inflammation of the lymphatic channels
What infection can cause lymphangitis?
- Acute inflammation by group A B-hemolytic strep
What are clinical signs of lymphangitis?
- Painful, red streaks
- Enlargement of draining lymph nodes
What is primary lymphedema?
- A congenital defect; familial milroy disease
What is secondary lymphedema?
- Blockage of normal lymphatics
What can cause secondary lymphedema?
- Malignant tumor, surgical procedures, and post-irradiation fibrosis
- Skin will look like an orange peel
What is nevus simplex?
- A simple birthmark seen on the forehead, eye, nose, and upper lip
- Most regress on their own
How common is a nevus simplex?
- 40-70% of infants have one
What is a port wine stain?
- A progressive ectasia of vascular plexus
- Persists into adulthood
What is Sturge-Weber syndrome?
- A trigeminal nerve facial port wine nevi
What are some other clinical symptoms of Sturge-Weber syndrome?
- Mental retardation
- Seizures
- Hemiplegia
- Skull radio-opacities
- Glaucoma
What is vascular telangiectasia?
- Permanent dilation of small vessels and capillaries forming a discrete lesion
Where do vascular telangiectasias most likely occur?
- Seen most on face, neck, and upper chest
What induces vascular telangiectasias?
- Increased circulating estrogen (pregnancy, liver disease)
What causes hereditary hemorrhagic telangiectasia?
- AD mutation of TGF-B signaling
What does hereditary hemorrhagic telangiectasia look like clinically?
- Abnormal blood vessel formation in skin, mucous membranes, and organs
What are hemangiomas?
- Common benign vascular tumor found on head or neck
- Most common benign tumor of childhood
What is a capillary hemangioma?
- Most common
- Involves the skin, mucous membranes, and viscera
What does a capillary hemangioma look like?
- Red spot; thin walled cap, tightly packed together
What is a strawberry hemangioma?
- Specific type of capillary hemangioma
- Present at birth but regresses
What is a cavernous hemangioma?
- Irregular dilated vascular channels making a lesion with an indistinct border
- More likely to involve deep tissue and bleed
Describe a pyogenic granuloma?
- Rapidly growing
- Found in oral mucosa or on finger
- Granuloma gravidarum is PG of pregnancy and involves the gingiva of pregnant women
What is a glomus tumor?
- A mesenchymal tumor of modified smooth muscle cells arising from glomus body
Where does a glomus tumor arise?
- Can occur anywhere but most likely to occur in fingers
- Will present as blue and painful (esp in temp change)
What is a lymphangioma?
- A benign, lymphatic neoplasm
What is a simple lymphangioma?
- Involves head and neck with axillary predilection
What is a cavernous lymphangioma?
- Involves the neck or axilla in children
- Often seen in turner syndrome or other developmental syndromes
What is bacillary angiomatosis?
- A reactive vascular proliferation to gram neg bartonella bacilli
Who is a bacillary angiomatosis most likely seen in?
- Occurs in immunocompromised (HIV, transplant)
What is a bacillary angiomatosis treated with?
Antibiotics
What causes kaposi sarcoma?
- HHV8
How many types of kaposi sarcoma are there? What are they?
- Classic (sporadic)
- Endemic
- Iatrogenic
- AIDS associated
What is the classic type of kaposi sarcoma?
- Affects mediterranean, Eastern European, or middle eastern men
- Affects distal LE
What is the endemic type of kaposi sarcoma?
- Affects african children (<10) and has lymph node involvement
What is the iatrogenic type of kaposi sarcoma?
- Related to T cell immunosuppression in transplant patients
What is the AIDS type of kaposi sarcoma?
- Most common type in US and most common type of AIDS related illness
- Disseminates widely and has an aggressive course
What is an angiosarcoma?
- A malignant vascular tumor that is locally invasive
What are some specific risk factors of angiosarcomas?
- Liver– arsenic, thorotrast, PVC
- Lymphedema with axillary lymph node dissection –> lymphangiosarcoma
- Radiation for carcinoma
What does a balloon angioplasty do?
- Compress but can rupture occluding plaque
What occurs in coronary metallic stents?
- Could cause endothelial injury (causing immediate thrombus)
- Antithrombotic meds are given prior to procedure
What is given to reduce the restenosis?
- Drug-eluting stents
What are the types of vascular grafts?
- Synthetic
- Autologous
What are vascular grafts used for?
- Used to release vessel occlusion or bypass
When are synthetic grafts used?
- Used only for large vessels like AAA
