Transcript Blood Vessels-3

Blood Vessels-3
Dr Suneet Kumar
Vasculitis
•Inflammation & often
necrosis of blood vessels
•Predominant or sole
manifestation of disease/
only component of
disease
•May induce tissue
ischemia
• Polyarteritis nodosa
• Allergic granulomatosis
of Churg & Strauss
• Kawasaki disease
• Hypersensitivity
vasculitis
• Giant cell arteritis
(Temporal arteritis)
• Takayasu’s arteritis
• Buerger’s disease
• Wegener’s
granulomatosis
Polyarteritis nodosa (PAN)
• Necrotizing immune
vasculitis
• Epidemiology
– Young adults
– Male> female
• Distribution of disease
– Systemic necrotizing
vasculitis
• Any organ EXCEPT LUNG
• Kidney, heart, liver, GIT
– Small & medium sized
muscular arteries
• Cause
– Immune complex
deposition (type III
hypersensitivity)
– Activation of neutrophils &
monocytes by antineutrophil cytoplasmic
antibodies (P-ANCA); NOT
DIAGNOSTIC
• Associated conditions
– HBV antigenemia (30-50%)
– Hypersensitivity to drugs
• i.v amphetamines
Polyarteritis nodosa (PAN)
• Lesions are in different
stages of development
(acute or healing stage)
• Frequently only part of
vessel is involved
– Nodosa – Focal
aneurysm formation
• Segmental necrotizing
vasculitis
• 3 stages
• Acute lesions
– Fibrinoid necrosis and
neutrophils
• Healing lesions
– Fibroblast proliferation
• Healed lesions
– Nodular fibrosis & loss of
internal elastic lamina
• Sequele
– Thrombosis and infarction
– Multiple aneurysms
(kidneys, heart, GIT)
Polyarteritis nodosa (PAN)
•Symptoms
–Fever, wt loss, malaise
–Hematuria, renal failure
hypertension
–Abdominal pain, diarrhea, GI
bleeding
–Myalgia and arthralgia
•Signs
–Micro-aneurysms
–Pericarditis, myocarditis,
palpable purpura
• Diagnosis
– Arteriography
– Arterial biopsy
– Biopsy of palpable
nodules in skin or organ
• Prognosis
– Untreated- fatal in most
cases
– Treated- 90% long term
remission rate
Allergic granulomatosis
(Churg Strauss syndrome)
• Variant of PAN
associated with
– Asthma & peripheral
eosinophilia
• Pathology
– Systemic vasculitis of
upper & lower resp.
tract
– Granulomas
– P-ANCA positive
• Sites of involvement
– Lung, spleen, kidney
Kawasaki disease
• Epidemiology
– Commonly affects infants and
young children (age<4)
– Japan, Hawaii, and US
mainland
• Distribution of disease
– Large, medium-sized, and
small arteries
– Coronary artery commonly
affected
• Clinical features
– Fever
– Conjunctivitis; erythema and
erosions of oral mucosa
– Skin rash- Generalized
maculopapular
– Lymphadenopathy
• Pathology
– Acute necrotizing vasculitis of
small/medium-sized vessels
– Weakened vascular wall may
undergo aneurysm formation
• Etiology-Possible viral
• Complications
– Thrombosis, aneurysm, and
myocardial infarction
• Prognosis
– Self-limited course
– Mortality rate: 1-2% due to
rupture of a coronary
aneurysm or coronary
thrombosis
Hypersensitivity vasculitis
(Leucocytoclastic Vasculitis)
• Organs affected
– Post-capillary venules, mostly
skin →palpable purpura
– Can affect internal organs &
tissues
• Pathogenesis
– Immune complex deposition,
Type III hypersensitivity
– P-ANCA positive
• Causes
– Drugs: penicillin,
sulfonamides
– Bacterial: β hemolytic
Streptococcal Ag, Hepatitis B
Ag
• Microscopically:
– Fibrinoid necrosis of
vascular wall
– Extravasation of RBC’s
– Nuclear dust from PMN
breakdown
– Inflammation is at same
stage in all vessels
• Can resolve, recur, or
become chronic
Leucocytoclastic Vasculitis
GIANT CELL ARTERITIS
(Temporal arteritis)
• Most common
vasculitis
• Site of involvement
– Affects medium and
small arteries
– Usually branches of
carotid artery (temporal
& extra-cranial branches)
• Granulomatous
vasculitis
• Epidemiology
– Females > males
– Primarily affects elderly
population (> 50 years)
– Associated with HLA-DR4
• Distribution of disease
– Cranial arteries- temporal,
facial, ophthalmic
– Aortic arch- giant cells
aortitis (uncommon)
• Etiology
– ? autoimmune reaction to
elastic fibers in vessel wall
GIANT CELL ARTERITIS
(Temporal arteritis)
Morphology:
•Nodular thickenings with
reduction of lumen
•Segmental granulomatous
vasculitis
–with multinucleate giant cells
•Fragmentation of internal
elastic lamina
•Intimal fibrosis with luminal
narrowing
Clinically:
•Throbbing unilateral headache
•Jaw claudication / Facial pain
•Visual disturbances
–Ophthalmic artery occlusion
•Polymyalgia rheumatica
–Systemic flu-like symptoms
–Proximal muscle pain
–Peri-articular pain
•ESR- High
•Temporal arterial biopsy (2-3
cm) -Positive in 60% cases
GIANT CELL ARTERITIS
(Temporal arteritis)
• Treatment
– Corticosteroids and antiinflammatory agents
• Prognosis
– Treated- dramatic response to
steroids
– Untreated- blindness due to
occlusion of ophthalmic artery
TAKAYASU’S ARTERITIS
(Pulseless disease)
• Ocular disturbances
• Marked weakening of
pulses in upper extremities
• Epidemiology
•
•
– Most common in Asia
– Young and middle-aged
women (15-45 years)
Distribution of disease
– Medium sized to large
arteries
– Aortic arch and major
branches
Etiology
– Unknown
• Pathology
– Vasculitis
– Fibrous thickening of aorta
with narrowing of lumina
• Gross
–Narrowing of orifices
–Irregular fibrous thickening of
wall
• Microscopy
–Granulomatous vasculitis
–Massive intimal fibrosis
TAKAYASU’S ARTERITIS
(Pulseless disease)
•
•
•
•
•
•
Fever
Arthritis
Night sweats
Myalgia
SKIN nodules
Ocular disturbances
• Visual defects, retinal
hemorrhages, total
blindness
• Weaker pulses in UL
– Coldness or numbness of
fingers
• Variable course
– Rapid progression
– Quiescent stage within 1-2
years
• Sequele
– Collagenous fibrosis
involving all layers,
especially intima
– Aortic valve insufficiency
• Due to involvement of
aortic root leading to
dilation
– Myocardial infarction
• Due to a narrowing of the
coronary ostia
TAKAYASU’S ARTERITIS
(Pulseless disease)
• Diagnosis
– Distinctions among
active giant cell lesions
of aorta are based
largely on age of patient
– Most giant cell lesions of
aorta in young patients
are designated Takayasu
arteritis
BUERGER’S DISEASE
(Thromboangitis obliterans)
•Age: 25-50 years
•Etiology: Cigarette smoking
•Site
–Radial & tibial arteries;
adjacent nerves & veins
•C/F
–Intermittent claudication
–Superficial nodular phlebitis
–Raynaud’s phenomenon
–May lead to distal gangrene
• Pathology
– Thrombosis,
organization,
recanalization
– Micro-abscess
– Granulomas
– Fibrosis of vascular
bundle
BUERGER’S DISEASE
(Thromboangitis obliterans)
WEGENER’S GRANULOMATOSIS
• Epidemiology
–
–
–
–
Rare
Males > females
Age
Peak incidence 40-60 yrs
• Distribution of disease
– Focal necrotizing vasculitis
with granulomas
– Classically involves nose,
sinuses, lungs & kidneys
– Small size arteries & veins
Classic triad
• Acute necrotizing
granulomatous
inflammation
• Focal necrotizing
vasculitis
• Necrotizing
glomerulonephritis
WEGENER’S GRANULOMATOSIS
Clinical features
•Cough, dyspnea,
hemoptysis
•Chronic sinusitis
•Perforation of nasal
septum
•Otitis media
•Mastoiditis
•Hematuria
•Microscopy
–Fibrinoid necrosis
–Neutrophils
–Granulomas
•Lab findings
–C-ANCA
•Autoantibody against
proteinase 3
•Correlates with disease
activity
–Urine examination
Raynaud’s phenomenon & Disease
• Raynaud’s phenomenon
• Arterial insufficiency of
digital vessels in response
to cold or increased
emotion
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–
–
–
–
–
–
TAO
Takayasu’s arteritis
Cryoglobulinemia
Ergot poisoning
Thoracic outlet syndrome
Cold agglutinin disease
CREST Syndrome
Raynaud disease
(Primary Raynaud phenomenon)
• Clinical features
– Young woman
– Episodic small artery
vasospasm in
extremities, nose or ears
– Blanching and cyanosis
of fingers or toes
– Precipitated by cold
temperature/emotions
– No underlying disease
or pathology
Henoch-Scholein purpura
• Immune vasculitis
• Usually occurs in
children following URTI
• Most common vasculitis
in children
• IgA-C3
immunocomplexes
deposit in vessel wall
• IgA nephropathy
(Berger’s disease) may
be part of the systemic
complex
• Palpable purpura
– Lower extremities,
buttocks
• Polyarthritis
• Abdominal pain
– Sometimes with malena
• Renal disease
– Hematuria
Question
•
A.
B.
C.
D.
E.
A 70-year-old woman, a non-smoker, is bothered by
left sided headaches for several months. She does
not seek medical attention. She then notices
decreased visual acuity in her left eye. Her vital signs
show T 35.9 C, P 74, R 14, and BP 125/85 mm Hg. She
is found to have an erythrocyte sedimentation rate
of 94 mm/hr. Her CBC shows a WBC count of
7540/microliter. Her visual problem is most likely to
be a complication of:
Wegener's granulomatosis
Polyarteritis nodosa
Raynaud's disease
Giant cell arteritis
Thromboangitis obliterans
Question
•
A 45-year-old woman has had increasing malaise for
several weeks. Her serum creatinine is 3.8 mg/dL
with urea nitrogen of 40 mg/dL. Her serum
antinuclear antibody test is negative, but her antineutrophil cytoplasmic autoantibody test is
positive. She has an episode of hemoptysis. A chest
radiograph reveals a reticulonodular pattern of
infiltrates. A lung biopsy reveals necrotizing
granulomas with vasculitis.
A.
B.
C.
D.
E.
Wegener's granulomatosis
Thromboangiitis obliterans
Polyarteritis nodosa
Hypersensitivity angiitis
Giant cell arteritis