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8/3/2019 Lec 2- Blood Vessels
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Characteristics Structures Involved Clinical Features Lab Diagnosis Treatment
Giant Cell
Arteritis
- Age >50 yrs
Def: An acute and
chronic granulomatous
inflammation
- nodular thickenings
affected artery develop
these nodular
thickenings and may
become thrombosed
ischemia
(palpably firm andpainful)
Vasculitis produces this
b/c thrombus Ischemia
- Temporal artery (pain,
head ache),
- Ophthalmic artery-
blindness, diplopia
- Branches of external
carotid arteries: TIA (
episodes last< 24 hours).
Stroke (>24 hours)
- Polymyalgia
rheumatica (joint &
muscle pain), jaw pain
(caludication), abrupt
headaches, weight loss,
anorexia, loss of vision
and tenderness*.
- Temporal headache,
sudden diplopia.
Cannot see a thrombus
here…maybe it is gone
- ↑ ESR
Characteristics Structures Involved Clinical Features Lab Diagnosis Treatment
Takayasu
Arteritis
- Age <40 yrs
- Female
- pulseless
disease
- Granulomatous vasculitis
- branches of aortic arch,
renal, pulmonary and
Ophthalmic artery.
- Renal artery
Sec. hypertension (
↑Renin level)
1. aneurysm
2. narrowing
3. Occlusion
- BP and weaker
pulses in the upper
extremities ( carotid,
radial, ulnar) than in
the lower= pulseless
disease*.
- Ocular disturbances.
Giant Cell
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Characteristics Structures Involved Clinical Features Lab Diagnosis
Wegner
Granulomastosis
- aka…
Necrotizing
(granulomatous)
vasculitis
Triangle of death
1. Hole in the hard palate
(eroding the bone) systemic
involvement
2. Giant cell granuloma
3. Renal and Respiratory
system involved
- affects capillaries,
venules, arterioles, and
arteries.
- Giant cells is the blood vessels in
lung, kidney
- Death within 1 year if
untreated (malignant course).
- Nasopharynx:
- Mucosal ulceration,
sinusitis*, rupture of nasal
septum/ bleeding.
- Lung:
Pneumonitis/condolidation=
Hemoptysis*.
1. Mucosal ulcer in the
nose
2. Sinusitis
3. Bleeding from nasal
septum
- Kidney:
- Crescentic
Glomerulonephritis → acute
renal failure (hematuria*+
elevated BUN, creatinine).
- 20
Hypertension.
- c-ANCA* is positive in the serum in
95% & is used to monitor disease
activity.
dd TB bcz see caseous necrosis
- giant cell seen in both but in this case
it is found around the blood vessels
NOT tissue
It’s a quick course….2-3 months
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Characteristics Structures Involved Clinical Features Lab Diagnosis
Polyarteritis
Nodosa (PAN)
•It is a Systemic vasculitis.
–Hepatitis B surface
antigen present (HBsAg)*.
–Typically ANCA negative
PAN produces aneurysm
of the renal artery! Similar
to that seen in the
Wegenar.
- Involves Kidney, skin
and other organs….
Except the lungs***
Age: all age group
Course: E pisodic illness*.
Others:-
1.Malaise, fever of unknown cause, and
weight loss;
2.Hypertension, Abdominal pain and
melena (bloody stool)
3.*Skin: Palpable skin Purpura ( but this is
more common in leukocytoclastic vascuitis)
Morphology: : Patchy fibrinoid
necrosis (bright pink, fibrin-like,
refractile intimal deposit)* of intima
and adventitia with thrombosis.
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Characteristics Structures Involved Clinical
Features
Lab Diagnosis
Churg- Strauss
Syndrome
(variant of PAN)
•Features:
Involve pulmonary vasculature
Eosinophilia
Asthma
Morphologically is similar to PAN
pANCA is positive
MORPHOLOGY similar to PAN
PAN Churg- Strauss
Syndrome
No
Eosinophilia
Eosinophilia
Kidney inv. No Kidney inv
NO Lung Involves LUNG
pANCA neg. pANCA pos.
-
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Characteristics/ Etiology Structures Involved Clinical Features Lab Diagnosis
Immune Complex
Vasculitis:
AKA (Microscopic
polyarteritis,
Hypersensitivity,
OR
Leukocytoclastic
Vasculitis.)
•Etiology: reaction to an antigen
such as –Drugs (e.g., penicillin),
–Microorganisms (e.g.,
streptococci) or
–Malignancy
- pANCA is positive
•Affects arterioles, capillaries, andvenules-vessels. (venules… like in
Wegners)
•Deposition of antigen-antibody
complex in vessels wall.
•Skin: Palpable skin Purpura ( this
is more common than PAN),
hemoptysis, arthralgia, muscle painor weakness.
•Morphology: neutrophilic
vasculitis*.
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Characteristics/ Etiology Structures Involved Clinical Features Lab Diagnosis
Henoch-
Schonlein
Purpura
- Type of Leukocytoclastic vasculitis
-
•IgA mediated Skin purpura (vasculitis).
•Dermal IgA deposit: Dermatitis
herpetiformis.
•IgA deposition in kidney= recurrenthematuria.
•Abdominal manifestations:
–Pain, vomiting, and intestinal bleeding;
nonmigratory arthralgia. Negative for p-
anca.
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Characteristics/ Etiology Structures Involved Clinical Features Lab Diagnosis
Thromboangiitis
Obliterance
- AKA (Buerger
Disease)
•Thrombus and abscess in the
artery.
•Age & sex: middle aged(
before 35 years)
•Men > women
•There is a very close
association with smoking*. Tx
Stop smoking!
•Site: tibial and radial arteries.
- Early: Fingers show Raynaud
phenomenon and feet show
claudication.
- Late: Gangrene in some
In upper limbs: Raynaud
Phenomenon
In lower limbs: Claudication
Raynaud Phenomenon:
Paroxysmal color change:
whitebluered
Two types: –Primary = Vasoconstriction due
to cold, stress etc= neurological.
–Secondary: due to pathological
obstruction of artery as seen in
Buerger disease, scleroderma,
SLE etc.
Pathophysiology: Smoking Nicotine endothelial injury
thrombus. --> ischemia
gangreene (tibial/radial)
Arrow is showing ABSCESS!
(puss)
- Lumen occluded by a
thrombus with two
microabscesses (arrow)
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Characteristics/ Etiology Structures Involved Clinical Features Lab Diagnosis
Kawasaki Disease •Age: young children •Location: coronary artery →
AneurysmMyocardial infarction
•Clinical: conjunctivitis +
maculopapular skin rash +
lympadenopathy.
Looks like viral infection
Coronary artery aneurysm may
occur.
Characteristics/ Etiology Structures
Involved
Clinical Features Lab Diagnosis
Systemic Lupus
Erythematosus •Fibrinoid necrosis is present. Like PAN.
•Antibodies in SLE: –Anticardiolipin Ab.→ deep VENOUS thrombosis
(aka- Antiphospholipid syndrome) present.
–ds-DNA ANA, anti-smith ANA- not involved in DVT.
Antiphospholipin syndrome of DVT
1. SLE, 2. DVT 3. Anticardiolipin Ab.
- Fibrinoid necrosis seen here.
IT is an exudate made out of
fibrinogen.
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Characteristics/ Etiology Structures Involved Clinical Features Lab Diagnosis
Varicose Veins Abnormally dilated, tortuous veins
produced by
1.Prolonged, increased intraluminal
pressure (valve incompetence).2.Loss of vessel wall support-
muscle weakness- paralysis.
3.Weak valve or genetic
•Esophageal varices: seen in
patients who have cirrhosis of the
liver and its attendant portalhypertension.
•Hemorrhoids:
–varicose dilation of the
hemorrhoidal plexus of veins at
the anorectal junction: produce
fresh blood during defecation.
–Caused by repeated
pregnancies, straining at stools
(by cocaine).
Clinically silent (NO PAIN)
Varicos veine: without
THROMBUS and is NOT
painful/tender OF SUPERFICIAL
VEINS (i.e great saphenus).
Thrombophlebitis: vericose vein
with THROMBUS and has
Inflmmation and is TENDER and
PAINFUL.
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Characteristics/ Etiology Structures Involved Clinical Features Lab Diagnosis
Deep Vein
Thrombosis
•Cause: these veins usually don’t
form thrombus b/c of the fast flow.
Viscocity of the blood is elevated
due to: hypercoagulable states: – Adenocarcinoma , pregnancy,
immobilization, bone fracture* and
others.
- Viscosity of the blood is elevated
due to the
- Iliac, femoral, popliteal veins. - All 3 veins: asymptomatic, leg
swelling, erythema in some
cases.
Complications: Thrombus
acute cor pulmonale (RHF).
Also: Pulmonary infarction
which is acute and quick death.
Note: thrombus. Infarcted
area becomes RED.
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Characteristics/ Etiology Structures Involved Clinical Features Lab Diagnosis
Angiosarcoma
Tumor of the
blood vessel
• Age: often in older adults
• Marker: CD31, CD34, or vWF, FVIII• Carcinogen: Arsenic, polyvinyl
chloride (plastic factory),
Thorotrast.
- Most commonly in the SKIN and
LIVER (others: soft tissue, breast).
-
•Morphology: proliferatingvascular channels,
hyperchromatic spindle cells
(cancerous) and RBC.
Characteristics/ Etiology Structures
Involved
Clinical Features Lab Diagnosis
Kaposi’s
Sarcoma
Agent: HIV and KSHV-8
-If it occurs in the SKIN= Kaposi’s sarcoma. It is
basically an angiosarcoma, of the skin.
- HIV infectionImmune system is depressed
herpes virus comes in to produce this sarcoma- Vimentin is a nonspecific marker here.
Pathogenesis: KSHV proteins Incorporate in host
genome and:
1.May disrupt the control of cellular proliferation
(cell cycle S PHASE) by inactivating Rb gene , and
2.Prevent apoptosis of endothelial cells, through
the production of p53 inhibitors.
- It is a dermal
angiosarcoma.
•Clinical : Multiple growing red
to purple skin plaques or
nodules or on the hard palate
(Kaposi’s does not look like a
cancer clinically (despite itbeing cancer)…it looks like a
skin rash!!!.
In perineal, lower limbs and
oral cavity.
NON-painful and does not look
like cancer. Covers your whole
skin very rapidly.
•Morphology: sheets of plump,
proliferating hyper chromatic spindle
cells & RBC (CD31, CD34 and vWF,
Factor 4 and 8, Vimentin…) and slit like
spaces
Note: white slit
like spaces. Lots
of spaces & RBCs
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