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is
vasculitis
?
s Vasculitis
is
a
s heterogeneous
disorder
s Vascular
inammation
s Classied
:
s Cause
s Size
of
vessels
s Ab-associated
s Pathology
: primary, secondary : small, medium, large : ANCA, ANA : granulomatous, immune / pauci-immune
Classication
of
Vasculitis
Isolated
vasculitis
Systemic
vasculitis
s Primary
vasculitis
s Large
vessel
s Medium
vessel
s Small
vessel
s miscellaneous
s Secondary
vasculitis
s Infection
associated
s CNTD
associated
s Drug
induced
s Malignancy
associated
s Post
organ
transplant
Secondary
vasculitis
s Infection-associated
s Viral
:
HBV,
HCV,
HIV,
Herpes,
CMV
,
parvo
B19
s Bacteria
:
strep,
staph,
salmonella
s Mycobacteria
:
TB,
atypical
mycobacteria
s Syphylis
Secondary
vasculitis
s Infection-associated s CNTD-associated
s SLE
s MCTD
s Dermatomyositis
s RA
s Sjogrens
syndrome
Secondary
vasculitis
s Infection-associated s CNTD-associated s Drug induced
s Penicillin
s Sulfonamide
s Oral
contraceptive
agents
s Allopurinol
s PTU
s Hydralazine
Secondary
vasculitis
s Infection-associated s CNTD-associated s Drug induced s Malignancy-associated
Secondary
vasculitis
s Infection-associated s CNTD-associated s Drug induced s Malignancy-associated s Post-organ transplant
Large
s aorta and its major branches (and the corresponding vessels in the venous circulation in some forms of vasculitis, e.g., Behet's disease). s smaller than the major aortic branches s contain four elements: (1) an intima, (2) a continuous internal elastic lamina, (3) a muscular media,(4) an adventitia. s In clinical terms, medium vessel vasculitis is generally macrovascular (i.e., involves vessels large enough to be observed in gross pathologic specimens or visualized by angiography). s all vessels below macroscopic disease, includes capillaries, postcapillary venules, and arterioles s less than 500 in outer diameter s Glomeruli: dierentiated capillaries
Medium
Small vessel
Classications
s ACR
criteria
1990
s Chapel-Hill
concensus
conference
1994
s EULAR
1994: CHCC classication ( Chapel Hill Consensus Conference) BV Size Large Primary Vasculitis GCA TK Medium PAN KW Small WG CSS MPA HSP Ess Cryo Cut. LCV Histology Granulomatous arteritis of aorta and its branch ( Ext Carotid A.) Granulomatous arteritis of aorta and its branch Necrotizing vasculitis of medium and small BV Large, meduim and small arteritis( Coronary a.) Granulomatous necrotizing vasculitis (sm- M) Granulomatous necrotizing vasculitis (Eo- sm-M) Necrotizing vasculitis of small BV IgA-deposit vasculits of small BV Cryoglobulin-deposit vasclitis of small BV Isolated cutaneous LCV no systemic
Classications
primary
Large artery
secondary
Giant cell arteritis Aortitis asso with RA Takayasus arteritis syphilis Isolated CNS angiitis PAN Kawasaki
WC CSS MPA HSP Cutaneous LCV Essential mixed cryoglobulinemia Hepatitis B SLE Sjogrens syndrome Drug HIV Drug Hepatitis B, C
aorta
Classic PAN, Kawasaki large & medium Wegener, MPA, Churg Strauss
Henoch Schonlein Cryoglobulinemia Buergers disease Small vessel angiitis
small
arteriole capillary venule vein vena cava
aorta
Classic PAN, Kawasaki large & medium Wegener, MPA, Churg Strauss
Henoch Schonlein Cryoglobulinemia
small
arteriole capillary venule vein vena cava
aorta
large & medium
small
arteriole capillary venule vein vena cava
less common in Westerns aseptic meningoencephalitis cerebral vasculitis recurrent phlebitis, arteritis, synovitis
Behcets disease
Tissue
histopathology
s Granulomatous
inammation
s Immune
complex
-
mediated
s ANCA
associated
(pauci-immune)
Granulomatous
Inammation
s Giant
cell
arteritis
s Takayasu's
arteritis
s Cogan's
syndrome
s Wegener's
granulomatosis
s Churg-Strauss
syndrome
s Primary
angiitis
of
CNS[]
s Buerger's
disease
[]
s Rheumatoid
vasculitis
s Sometimes
granulomatous.
s Giant
cells
occur
within
inammatory
thrombi
(and
are
diagnostic
of
Buerger's
disease),
but
do
not
occur
within
the
blood
vessel
wall.
ANCA
associated
s WG
s CSS
s MPA
s PAN
Approach
to
vasculitis
vIdentify
common
clinical
features
of
vasculitis
vExclude
pseudo-vasculitis
(mimics)
vLook
for
causes
of
vasculitis
(secondary
vasculitis)
vDene
primary
vasculitis
(categorize
group)
vConrm
denite
diagnosis
(tissue
biopsy/angiogram)
vStart
appropriate
treatment
Approach
to
vasculitis
vIdentify
common
clinical
features
of
vasculitis
vExclude
pseudo-vasculitis
(mimics)
vLook
for
causes
of
vasculitis
(secondary
vasculitis)
vDene
primary
vasculitis
(categorize
group)
vConrm
denite
diagnosis
(tissue
biopsy/angiogram)
vStart
appropriate
treatment
Approach
to
vasculitis
vIdentify
common
clinical
features
of
vasculitis
vExclude
pseudo-vasculitis
(mimics)
vLook
for
causes
of
vasculitis
(secondary
vasculitis)
vDene
primary
vasculitis
(categorize
group)
vConrm
denite
diagnosis
(tissue
biopsy/angiogram)
vStart
appropriate
treatment
Mimic
Vasculitis
Infec&ous
diseases
Bacterial
endocardi-s
**
DGI
**
Pulmonary
histoplasmosis
Coccidioidomycosis
Syphilis
Lyme
disease
Rocky
Mountain
spo?ed
fever
Whipple's
disease
Drug
toxicity
Cocaine
**
Amphetamines
**
Ergot
alkaloids
**
Methysergide
Arsenic
Thrombosis
**
An-phospholipid
syndrome
TTP
Neoplasms
**
Atrial
myxoma
Lymphoma
Carcinomatosis
Miscellaneous
Atheroembolic
disease
**
Goodpasture's
syndrome
**
Sarcoidosis
Amyloidosis
Migraine
Cryobrinogenemia
Approach
to
vasculitis
vIdentify
common
clinical
features
of
vasculitis
vExclude
pseudo-vasculitis
(mimics)
vLook
for
causes
of
vasculitis
(secondary
vasculitis)
vDene
primary
vasculitis
(categorize
group)
vConrm
denite
diagnosis
(tissue
biopsy/angiogram)
vStart
appropriate
treatment
Secondary
vasculitis
Evaluate History s Infection-associated s CNTD-associated s Drug induced s Malignancy-associated s Post-organ transplant
Post-capillary
venules
Palpable
purpura
Urticarial
vasculitis
Arteriole small a.
Skin ulcer
EN
Livedo reticularis
Gangrene
Mononeuritis multiplex
Typical
Clinical
Manifestations
of
Large,
Medium,
and
Small
Vessel
Involvement
by
Vasculitis
Constitutional
symptoms:
fever,
weight
loss,
malaise,
arthralgias/arthritis
(all
vessel
sizes)
Large Limb claudication Asymmetric blood pressures Absence of pulses Bruits Aortic dilation Renovascular hypertension Medium Cutaneous nodules Ulcers Livedo reticularis Digital gangrene Mononeuritis multiplex Microaneurysms Renovascular hypertension Purpura Vesiculobullous lesions Urticaria Glomerulonephritis Alveolar hemorrhage Cutaneous extravascular necrotizing granulomas Splinter hemorrhages Uveitis/episcleritis/scleritis Small
Epidermiology
s AGE,
GENDER,
AND
ETHNICITY
s KW
:
80%
<
5
yrs
s GCA
:
never
occurs
in
<
50
yrs,
mean
age
72.
s HSP
:
s children
(90%)
self-limited
s adults,
higher
chronicity
&
poor
renal
outcome
Epidermiology
Disease GCA United States Elsewhere Age/Gender/Ethnic Predispositions Age >50, mean age 72/ Females 3:1/Northern European ancestry Age <40/Females 9:1/Asian Silk Route countries Slight male predominance Children of Asian ancestry Whites >> Blacks Only 10% of cases occur in adults 240/1 million 220-270/1 million (Olmsted County, (Scandinavian MN) countries) 3/1 million 3/1 million 7/1 million 100/1 million 4/1 million
[]
200-300/1 million (India) 3000/1 million (Turkey) 7/1 million (Spain) 900/1 million (Japan) 8.5/1 million (United Kingdom)
From Gonzalez-Gay MA, Garcia-Porrua: Epidemiology of the vasculitides. Rheum Dis Clin N Am 27:729-750, 2001.
Epidermiology
s GENES
s Familial
rare
s except
GCA
HLA-DRB10401
and
HLA-DRB10101,
tumor
necrosis
factor
microsatellite
polymorphisms
s Rheumatoid
vasculitis:
s HLA-DRB1
,genotypes
0401/0401,
0401/0404,
and
0401/0101
s HLA-C3
in
patients
lacking
HLA-DRB104
s WG
:
s functional
polymorphism,
620W,
in
the
intracellular
tyrosine
phosphatase
gene
PTPN22
Epidermiology
s ENVIRONMENT
s cigarette
smoking
s Buerger's
disease
and
rheumatoid
vasculitis
s IVDU
s Infection
s HBV
HCV
VZV
HIV
Investigations
1.
Exclude
mimic
2.
Assess
extent
of
vasculitis
3.
Conrm
diagnosis
4.
Identify
specic
type
of
vasculitis
Investigations
1.
Exclude
mimic
2.
Assess
extent
of
vasculitis
3.
Conrm
diagnosis
4.
Identify
specic
type
of
vasculitis
s H/C
s Hepatitis
prole
s Anti
HIV
s Anti
phspholipid
chanel
s ANA,
RF
s Echo
s Anti
GBM
Investigations
1.
Exclude
mimic
2.
Assess
extent
of
vasculitis
3.
Conrm
diagnosis
4.
Identify
specic
type
of
vasculitis
UA
CXR
EMG
/
NCV EEG/
LP
Investigations
1.
Exclude
mimic
2.
Assess
extent
of
vasculitis
3.
Conrm
diagnosis
4.
Identify
specic
type
of
vasculitis
s Biopsy
s Angiogram
Investigations
1.
Exclude
mimic
2.
Assess
extent
of
vasculitis
3.
Conrm
diagnosis
4.
Identify
specic
type
of
vasculitis
s ANCA
s Cryoglobulin
s Complement
(C3
C4
CH50)
s Eosinophilia
/
IgE
(CBC)
s Specic
biopsy
staining
:
s s s s Necrotizing
Granulomatous
inam
IgA
deposit
IC
deposit
C-ANCA
>95%
Proteinase-3
<5%
others
P-ANCA
aorta
Classic PAN, Kawasaki large & medium Wegener, MPA, Churg Strauss
Henoch Schonlein Cryoglobulinemia
small
arteriole capillary venule vein vena cava
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