Clinical features
Treatment
Fever, fatigue, weight
loss, headache, facial
pain, and ocular
symptoms (diplopia,
blindness)
Corticosteroids
E
a
s
y
sy
Ea
Corticosteroids and
immunosuppressive
therapy
Gross: Segmental
lesions; superimposed
Involves renal and
thrombosis. Micro:
visceral arteries (heart,
Transmural mixed
GIT, liver) but spares
Medium to small
1/3 of patients have
Typically young adults,
inflammatory infiltrate pulmonary circulation;
muscular
chronic Hepatitis B; not
but affects all ages
(neutrophils and
hypertension, abdominal
arteries
associated with ANCAs
mononuclear cells);
pain, blood per rectum,
fibrinoid necrosis; older
muscular pain, and
lesions show fibrous
peripheral neuritis
thickening
Immunosuppressive
therapy; 25% relapse
rate
h
2
s
<50 yrs; common in
Japanese ethnicity
ee
Large to
medium
se
e
Gross: Marked wall
thickening with
Weakened pulses/BP in
narrowed or obliterated
upper extremities;
Autoimmune etiology;
lumen. Micro: Intense
ocular disturbances
primarily affects aortic transmural mononuclear
(retinal hemorrhage,
arch and its major
infiltrate; giant cells;
blindness); pulmonary
branches
granulomatous
hypertension; MI;
inflammation; intimal
systemic hypertension
hyperplasia; patchy
medial necrosis
t
Kawasaki Disease
(Mucocutaneous lymph
node syndrome)
Morphology
M
a
y
d
g
e
o
l
o
Wi
Polyarteritis Nodosa
(PAN)
Pathogenesis
Gross: Patchy nodular
thickening. Micro:
T-cell mediated immune
Intimal thickening;
response to
granulomatous
Older adults (~50 yrs); uncharacterized vessel inflammation in media
most common vasculitis wall antigen; elevated
(macrophages,
in this group
anti-endothelial and
lymphocytes,
anti-smooth muscle
multinucleated giant
antibodies
cells); fragmentation of
internal elastic lamina;
adventitial fibrosis
h
t
a
P
Takayasu Arteritis
(Pulseless disease)
Large to
medium
Demographics
Path
Giant Cell (Temporal)
Arteritis
Vessel size
W
ti
Vasculitis Type
A h m ed Kd
Dense transmural
Acute febrile illness;
inflammatory infiltrate;
conjunctival/oral
Triggered by viral
fibrinoid necrosis (less erythema and blistering;
infections; delayed-type
prominent than PAN);
hand/foot edema;
IVIG or aspirin (both
hypersensitivity;
Large to medium 80% in children <4 yrs
obstructive intimal
cervical lymph node
lower coronary artery
autoantibodies against
thickening when healed;
enlargement;
disease risk)
endothelial and smooth
coronary artery
cardiovascular
muscle cells
aneurysms in untreated
involvement (20% of
cases
untreated cases)
Dr. Ahmed Kdees
Treatment
Affects skin, mucous
membranes, lungs
(hemoptysis), kidneys
(hematuria, proteinuria),
brain, heart, GIT, and
muscles; palpable
cutaneous purpura
Immunosuppression and
removal of offending
agents
E
a
s
y
Segmental fibrinoid
Antibody response to drugs
necrosis of media;
(penicillin), microbes
neutrophilic infiltration
(streptococci), or tumor
with leukocytoclastic
proteins; immune complex
activity (karyorrhexis);
deposition or secondary
absence of granulomatous
ANCAs (MPO-ANCAs)
inflammation
Clinical features
Typically middle-aged
men
Triad: Necrotizing
granulomas (respiratory
Cell-mediated
tract), necrotizing
hypersensitivity to
infectious/environmental granulomatous vasculitis,
and focal
antigens; 95% positive for
necrotizing/crescentic
PR3-ANCAs
glomerulonephritis
Chronic sinusitis; lung
nodules and cavitation
(hemoptysis);
hematuria/proteinuria;
renal failure; high
mortality (80%) if
untreated
Steroids,
cyclophosphamide, TNF
inhibitors, and Rituximab
Heavy smokers, <35 yrs;
Medium to small
common in Israeli,
(typically tibial and
Indian, and Japanese
radial)
populations
Sharply segmental
transmural vasculitis;
luminal thrombosis with
microabscesses rimmed by
granulomatous
inflammation; involves
adjacent veins and nerves
Raynaud phenomenon;
instep claudication (foot
pain with exercise);
superficial nodular
phlebitis; extremity
ulceration and gangrene
Smoking cessation,
vasodilators, and
sympathectomy
h
Rare
Various
s
2
se
e
Small
Immune response to
tobacco extracts;
associated with certain
HLA haplotypes
Hyperresponsiveness to
allergic stimuli; associated
with MPO-ANCAs
Small-vessel necrotizing
vasculitis; extravascular
necrotizing granulomas;
intense infiltration by
eosinophils
Asthma, allergic rhinitis, lung
infiltrates, peripheral
eosinophilia, palpable purpura,
Corticosteroids and
and GI bleeding; cardiac
immunosuppressive therapy
involvement (60%) is a major
cause of death
Bacteria or fungi (e.g.,
Aspergillus, Mucor);
spreads from local tissue
or hematogenously (e.g.,
emboli from endocarditis
Inflammation caused by
direct infection
Complications include
mycotic aneurysms,
thrombosis, and infarction
of supplied tissues
ee
Small to medium
t
Infectious Vasculitis
Morphology
M
a
y
d
g
e
o
l
o
Not specified
Wi
Churg-Strauss Syndrome
(Allergic granulomatosis and
angiitis)
Pathogenesis
sy
Ea
Thromboangiitis Obliterans
(Buerger Disease)
Small (capillaries,
small arterioles)
h
t
a
P
Wegener Granulomatosis
Demographics
Path
Microscopic Polyangiitis
(Hypersensitivity or
Leukocytoclastic vasculitis)
Vessel size
W
ti
Vasculitis Type
A h m ed Kd
Not-specified
Not-specified
Dr. Ahmed Kdees
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