Vascular Pathology Flashcards
Glomus
Benign vascular tumor arising from specialized smooth muscle cells of the glomus body
- often found on fingertips, under the nai
- painful
- usually biopsied to r/o melanoma
Complications of Atherosclerosis?
- Ischemia
- MI
- Stroke
- Aortic Aneurysms
- Peripheral vascular disease
Kawasaki Disease
- symptoms?
- epidemiology?
- Results in what disease if untreated?
- Conjunctivitis
- Rash
- Adenopathy (cervical)
- Strawberry tongue
- Hands and Feet changes
- Fever
- Usually affects Asian children
- Results in Coronary Aneurysm or MI if untreated
Disease characterized by the triad of:
- Recurrent Aphthous Ulcers
- Genital Ulcers
- Uveitis (pigmented eye)
Behget’s Disease
Bacillary Angiomatosis
Reactive vascular proliferation caused by gram neg bacteria:
1. Bartonella henselae - found in cats (causes Cat-scratch disease in immunocomprimised)
- Bartonella quintana - transmitted by human body lice
- bacteria induce HIF-1alpha
Hemangioma
Benign Vascular tumor arising from endothelium
- composed of blood-filled vessels
- very common
- malignant transformation is rare
- 4 subtypes
Hereditary hemorrhagic Telangiectasia (Osler-Weber-Rendu disease)
Benign Vascular Ectasia
- caused by mutation in TGF-beta signaling to endothelial cells
-nosebleed is most common symptom
Sturge-Webber Syndrome
Vascular ectasias
- Port wine colored skin patches in trigeminal nerve distribution
- may also have ipsilateral venous angiomas in the cortico leptomeningies
may lead to metal retardation, seizures
What are AGEs
Advanced glycation end products
- forms from the non-enzymatic rxn between reducing sugars (i.e. glucose) and proteinsm lipids or nucleic acids
- implicated in numerous diabetes and age-rlated diseases.
- first identified = glycated hemoglobin (HgbA1-C)
A Thoracic Aneurysm is most likely caused by?
Syphilis
Results in “tree-bark” appearance of aorta
- Granulomatous, classically involving the heart
- adults
- weak/absent pulse in upper extremities
Takayasu Arteritis
Causes of an Aortic dissection?
Inadequate or abnormal connective tissue synthesis (Proteoglycans, collagen, elastin)
- HTN - causes hyaline arteriosclerosis of the vaso vasorum which leads to atrophy/weaking of the media (Most common cause)
- mutations in TGF-Beta receptor or downstream signaling
- Collagen defect diseases (Marfans and Ehlers-Danlos syndrome)
Angiosarcoma
Vascular tumor arising from endothelium
- aggressive tumors
- 30% have 5 yr survival
- occurs in older adults
- younger PTs w/ smaller lesions have better prognosis
- Skin, soft tissue, breast, and liver are most common sites
- Histo: CD31+
An ascending aneurysm is most likely caused by:
HTN
ectasia (definition)
Local dilation of a structure
Fibromuscular dysplasia
Congenital vasculature anomaly characterized by:
- Alternating thickened and thinned arterial walls -> “string of beads” appearance
- media of arterial walls
- affects small to medium sized vessels
- commonly bilateral
- non-inflammatory, non-atherosclerotic
- occurs most commonly in younger women
- if occurs in renal artery, it can lead to renovascular HTN
Buerger Disease is caused by what?
- symptoms?
- Tx?
Smoking => inflammation and thrombosis of arteries supplying the hands and feet
Symptoms:
- claudation (cramping)
- Cold sensitivity
- gangrene w/ amputation of digits
Tx: stop smoking
Modifiable risks of atherosclerosis
- HTN
- Hypercholesterolemia
- high LDL
- Low LDL - smoking
- Diabetes
- Chronic inflammatory diseases
Name two Large-vessel Vasculitis diseases?
- Temporal (Giant cell) Arteritis
2. Takyasu Ateritis
Capillary Hemangioma (characteristics)
Most common form of hemangioma
- Affects Skin, subQ, mucus membranes, liver, spleen, and kidney
- histo: Thin walled w/ scant stroma
Characteristics of malignant vascular tumors:
- Usually do not form well organized vessels
- More cellular, more cytologic atypia
- may need confirmation with CD31, 34, and D2-40
Tx of Granulomatosis w/ Polyangitis (Wegner’s)
Cyclophosphamide and Corticosteriods
” 3 C’s of Wegners” - (drugs and C-ANCAs)
Name 3 medium vessel vasculitis diseases
- Polyarteritis Nodosa
- Kawasaki Disease
- Buerger Disease
Berry Aneurysms
Congenital vascular anomaly characterized by:
- Thin-walled arterial outpouching in cerebral vessels
- classically occurring at branched points (circle of Willis)
- Media is attenuated (thinned)
*can rupture and cause fatal intracerebral hemorrhage
Buzzers: “worst headache of my life”
T or F: Aneurysm involves only the media
FALSE!
Aneurysm involves ALL 3 layers
Constitutional/nonmodifiable risks of atherosclerosis
- Genetics
- Age
- Gender (pre-menopausal women protected)
What two diseases are associated with Arteriosclerosis?
- HTN
2. DM
- Granulomatous vasculitis
- occurs in older white females
- presents w/ headache, visual disturbances, and jaw claudication
- sometime Flu-like symptoms w/ joint and muscular pain
- elevated ESR
Temporal (Giant cell) Arteritis
- associated w/ polymyalgia rheumatica
- Tx: Steroids (STAT)
Microscopic finding associated w/ Wegner’s
Desrtuctive leukocytic Angitis of arteries an veins
- neutrophils, plasma cells, and eospinophils surrounding necrotic granuloma
Telangiectasia (definition)
Permanent dilation of preexisting small vessels (usually in skin or mucous membranes
- can be congenital or acquired
- not true neoplasm
Juvenile (strawberry) Hemangiomas (characteristics)
a form of Capillary hemangioma
- usually found on newborn skin
- grow rapidly for a few months then fade by 1-3 years
AGEs effects on vasculature:
- Induce cross-linking of collagen which can cause vascular stiffening and entrapment of LDL in the artery walls
- AGEs can oxidize LDL (major atherosclerosis developmental factor)
- AGEs bind to their receptors (RAGE) and cause oxidative stress as well as activate inflammation pathways causing further injury
Difference between Wegner’s and Microscopic polyarteritis?
- Palpable Purpura
- MPO-ANCA
- NO Granulomas!
An abdominal aneursym is most likely caused by:
Atherosclerosis
-Risk factors: men smokers > 50 yrs old
Presentation (triad) for AAA
- pulsatile abdominal mass
- hypotension
- flank pain
Arteriovenous (AV) Fistulas
Congenital anomally characterized by:
- an abnormal connection of artery to a vein WITHOUT a capillary bed
- occur most commonly as development defect
- also occur from ruptured arterial aneurysms into veins, pierce injury of an artery and vein and from inflammatory necrosis into adjecent vessels
- Chronic Sinusitis w/ saddle nose
- Hemoptysis (coughing blood)
- Hematuria (renal involvement)
- C-ANCAs (Proteinase-3)
Granulomatosis w/ Polyangitis (Wegner’s)
Triad:
- Granulomas
- Vasculitis
- Glomerulonephritis
Name 6 small vessel vasculitis disease
- Granulomatosis w. Polyangitis (Wegner’s)
- Microscopic Polyangitis
- Churg-Strauss Sundrome
- Behget Disease
- Henoch-Schonlein purpura
- Cryoglobulinemia
- Palpable purpura located on legs and ass
- Arthritis
- Abdominal pain (potentally some bloody stool)
- Renal disease (severe cases)
-Intussusception (from inflammed intestines)
Henoch-Schonlein Purpura
Kaposi’s Sarcoma
caused by KSHV (aka Human herpesvirus-8)
- disrupts normal cell division (prevents apoptosis by inhibiting p53)
- often found in late stage HIV patients
- also causes primary effusion lymphoma and castleman’s disease
- very similar to angiosarcoma
Vasculitis associated w/ Hepatitis B
Polyarteritis Nodosa
- Generalized vascular involvement
- NO lung involvement
Pyogenic Granulomas (characteristics)
type of capillary hemangiomas
- grow rapidly in areas of trauma
- oftern pedunculated
- often found on skin, gingiva, and oral mucosa
- *resembles granulation tissue
- painless and associated w/ pregnancy
Churge-Strauss Syndrome is characterized by?
- Affecting those with allergies or asthma
- Some type of organ damage
- MPO-ANCA
- Myocardial involvment
Cystic Hygroma
Congenital Lymphangioma
- often found on neck or axilla of children
- *associated w/ Turner’s syndrome (45, X)
Cavernous Hemangiomas (characteristics)
- Do not regress
- More infiltrative and intimately involved w/ surrunding tissue
- large blood filled spaces
- *associated w/ Von Hippel-Lindau syndrome
Pathogenesis of Atherosclerosis
- Endothelial damage allows lipids (LDL and cholesterol) to enter and accumulate in the intima
- Lipids are oxidized and consumed by macrophages forming foam cells
- Inflammation and healing leads to deposition of ECM and smooth muscle proliferation
Risk Factors for HTN
- Age
- Race (increased in African Americans)
- obesity
- stress
- high-salt intake
Cause of Henoch-Schonlein Purpura? Prognosis?
- IgA-immune complex deposits in the mesangium (capillaries of the glomerulus of kidney)
- usually secondary to viral upper respiratory infection (URI)
*Generally benign (good prognosis)
Angiosarcomas of the Liver are associated with exposures to:
- Polyvinyl Chloride (PVC) in plastics
- Arsenical pesticides
- Thorotrast (radioactive contrast agent)
Presentation of dissection
sharp tearing chest pain that radiates to back
nonobstructive calcification of the media within muscular (medium-sized) arteries
Monckeberg’s (medial calcific sclerosis)
*ASYMPTOMATIC
Function of endothelial cells:
- Maintain non-thrombotic state (requires normal laminar flow and BP)
- Modulates inflammation
- Affect growth of other cells (Smooth muscle cells)
- Produce NO
- Produce contracting factors (endothelin)
Name the main infectious agents that cause vasculitis (Bacterial and Fungal)
Bacterial: Pseudomonas
Fungi: Aspergillis and Mucor species
Causes of HTN
- Idiopathic (>90%)
- Renal disease
- Aortic abnormalities (Coarctation + rigidity)
- Acute Stress
- Pheochromocytoma
Main complication of Dissection
Cardiac tamponade
- Also
- rupture => fatal hemorrhage
- obstruction of branching arteries (coronary or renal)
Complications of AAA
*1. Rupture (risk based on size) - may lead to fatal hemorrhage
- embolism
- impingement on adjacent structures
Vasculitis associated with Hepatitic C?
Cyroglobulinemia
Most common vasculitis of childeren?
Henoch-Schonlein Purpura
Hyaline vs hyperplastic arteriolosclerosis?
Histologically
Hyaline = thickened intima and narrowed artery
Hyperplastic = fibrinoid necrosis of artery (onion skinning apperance)
