Disorder

Vasculitis

Epidemiology/Etiol
ogy

Clinical/
Laboratory
Findings

Treatment

Takayasu arteritis
pulseless
disease

Large Vessel
Granulomatous
Aortic Arch

Young Asian
women and
children

No Upper

Corticosteroids

extremity pulse
>10 mmHg
discrepancy in

vessels

BP between
arms
Visual Defects
Stroke

Giant Cell
(Temporal) Arteritis

Large Vessel
Granulomatous
Involving
superficial
temporal and
ophthalmic
arteries

Adults > 50

Temporal
headache

Jaw claudication
I/L blindness
due to
ophthalmic
artery vasculitis

Polymyalgia

rheumatica is
commonly
associated (NL
CK)

ESR = useful
screening

Corticosteriods

Disorder

Vasculitis

Epidemiology/Etiol
ogy

Clinical/
Laboratory
Findings

Treatment

Polyarteritis
Nodosa (PAN)

Medium-sized

Middle-aged

Vessels are at

Corticosteroids
Cyclophosphami

vessels

Necrotizing
Involving renal,
coronary,
mesenteric
arteries

Pulmonary
arteries
SPARED

men

HBsAg
association
(immunocomple
x disease; Type
III HSV)

HCV less
common

UNK cause if
not one of above

ALL stages of
Acute and
Chronic
inflammation

Fever
(commonly
present; unk
origin)

Aneurysms

(produced by
focal vasculitis;
detected
w/angiography)

Organ infarction
in Kidneys (renal
failure,
hematuria),
Heart (acute
MI), Bowels
(bloody
diarrhea), Skin
(ischemic ulcer),
Testicle
(testicular pain)

de in resistant
cases

Disorder

Vasculitis

Epidemiology/Etiol
ogy

Clinical/
Laboratory
Findings

Kawasaki Disease

Medium-sized

Fever
Erythema and

vessel

Necrotizing
Coronary
arteries

Children
< 5 yo
Boys > girls
UNK cause
(likely infectious
cause in
genetically
susceptible ppl)

Leading cause
of acquired hear
disease in
children in
developed
countries

Asian children =

Thromboangitis
Obliterans
(Buerger Disease)

Medium-sized
vessels

digital vessel
thrombosis &
damage to
neurovascular
compartment

IV Ig
Aspirin
edema of hands Corticosteroids
and feet convalescing
w/desquamated
rash
adenopathy

oral erythema &

cracking of lips

Glossitis

(strawberryappearing
tongue)

AbNL ECG

Men

Lower extremity

Smokers
Genetic
mechanism

MC - Ashkenazi
Jews in Israel

High morbidity in
India, Korea,
and Japan

10% in females

(only after 2
unsuccessful
courses of IV Ig)

cervical

Highest
incidence
(Japan)

25-50 yo

Treatment

involvement
(100% cases)

resting pain on
forefoot

possible
ischemic ulcers
or gangrene of
foot/toes

Upper extremity
involvement (4050% cases)

upper limb

ischemia
w/ulceration and
gangrene
(amputation
common)

Raynaud
phenomenon

Smoking
cessation
essential

IV iloprost (PG
analogue)

Vasodilators (blockers, Cachannel

blockers)

Disorder

Vasculitis

Epidemiology/Etiol
ogy

Clinical/
Laboratory
Findings

Treatment

Raynaud Disease

Medium-sized

Young women
Exaggerated

Paroxysmal

Avoid cold

vessels

Digital vessels in
fingers and toes

tip of nose and

vasomotor
response to cold
or stress

ears in some
cases
Raynaud
Phenomenon

Medium-sized
vessels

Tip of nose and

ears in some
cases

Wegener
Granulomatosis

Medium and
Small-Sized
vessels

Necrotizing
involving Lung
(infarctions) and
Renal Vessels
(glomerulonephr
itis)

Ulceration and

temperatures
(wear gloves)

Ca-Channel
blockers
(nifedipine)

gangrene in
chronic cases

Adults (men and Systemic

women)

digital vessels in Secondary to

fingers and toes

digital color
changes (whiteblue-red
sequences)

other diseases
(SLE, CREST,
Systemic
sclerosis)

Sclerosis and
CREST

digital vasculitis
w/vessel fibrosis

dystrophic

calcification

ulceration
gangrene

41 yo = mean
Incidence equal
among genders

~ 90% present
with
Upper/Lower
airway
symptoms

Necrotizing
Granulomas in

Skin
URT

(nasopharynxsaddle nose
deformity,
chronic sinusitis,
trachea collapse)
LRT (cavitating
nodular lesions)

Necrotizing
vasculitis in

lungs (infarction,
hemoptysis)

Kidneys

(crescentic
glomerulonephriti
s)

Same as for
Raynaud Disease

Avoid cold

temperatures
(wear gloves)

Ca-Channel
blockers
(nifedipine)

Corticosteroids
Cyclophophamid
e

Disorder

Vasculitis

Epidemiology/Etiol
ogy

Clinical/
Laboratory
Findings

Treatment

c-ANCA
antibodies
(>90%)

correlate
erratically w/Tx

Microscopic
Polyangitis

Churg-Strauss
Syndrome

Small vessel
skin
lung
brain
GI tract
Kidneys
(postcapillary
venules and
glomerular
capillaries)

Children and
adults

Precipitated by
drugs,
infections, and
immune
disorders
(penicillin,
streptococci,
SLE)

skin

51 yo = mean
likely

lung

autoimmune

Small vessel

heart vessels

vessels at
SAME stage of
inflammation

Corticosteroids
and
cyclophosphamide

Palpable
purpura

glomerulonephrit
is (crescentic)

p-ANCA Abs
(>80%)

Allergic rhinitis
asthma
p-ANCA (70%)
eosinophilia

corticosteroids

Disorder

Vasculitis

Epidemiology/Etiol
ogy

Henoch-Schonlein
Purpura

Children 1-15 yo Often follows:

Corticosteroids
(if severe GI
some young
- Viral URI
disease or renal
adults
- GAS pharyngeal
disease are
Peak incidence
infection

Small vessel
skin
GI
Renal
Joint vessels

in Spring (rarely
summer)

Males > females

MC vasculitis in
children

MC Whites &
Asians than
blacks

IgAAnti-IgA
immunocomplex
es (Type III
HSR)

Clinical/
Laboratory
Findings

- Pathogens may

act as Ag trigger
that causes Ab
formation
leading to IC
formation (Type
III HSR)

Palpable purpura:
bottocks and
lower
extremities (95100%)

Polyarthritis
(80%)

Glomerulonephri
tis (80%)

Abd pain and

vomiting (85%)

GI bleeding
Recurrence in
1/3 of cases

Most =

spontaneous
recovery in 4 mo
w/o Tx

Treatment

present)

Disorder

Vasculitis

Epidemiology/Etiol
ogy

Clinical/
Laboratory
Findings

Treatment

Cryglobulinemia

Adults
- female > male

Cryglobulins:
- proteins in

Immunosuppressiv
e agents

Associations

- gel at cold

Small vessel
skin

(3:1)

GI
Renal
Different types
of
cryglobulinemia
(mixed,
monoclonal,
polyclonal)

with
HCV (>50%)

- Type 1 MPGN
- Multiple
Myeloma
(monoclonal
type)

Lymphoproliferat
ive disorders

- CT disorders

plasma

temperatures

- particularly in
those areas

exposed to cold
temperatures

Palpable
purpura

Acral cyanosis
of nose and ears

Raynaud
phenomenon

Glomerulonephri
tis (crescentic)

Arthritis
Abd pain

Disorder

Vasculitis

Epidemiology/Etiol
ogy

Clinical/
Laboratory
Findings

Infectious
Vasculitis

Small vessel
Skin vessels

Children and

RMSF:
Oral Doxycycline
- Dog tick or wood - Can treat

adults

involves
Microbial
Pathogens

Spotted Fever
(RMSF)
MC in SE,
followed by

tick transmission

children up to 10

of Rickettsia

days

rickettsii

Rocky Mountain

(Dermacentor
variables or
andersoni)

- Organisms
invade

south central

endothelial cells

states

producing
vasculitis

Fever (100%)
Petechiae
begin on palms
spread to trunk
50% appear 1st
day

- 80% appear by
5th day

- 10% no

petechiae

Treatment