Vasculitides
Fudan university
Zhongshan Hospital
Yuqiang
The term vasculitis refers
to a heterogeneous group of
disorders characterized
pathologically by evidence of
blood vessel inflammation and
clinically by a diverse set of
symptoms and signs,
Pathology
three types,
1,necrotize
2,granulomas
3,only inflammatory cellular
infiltration in the vessel wall
Cause to
1.vessel wall
angusty,
blunting,
fibrosis,
aneurysm
2,tissue
ischemia,
functional impaiement
CLASSIFICATION
1,histopathologic
( 1) necrotize
( 2) granulomas
( 3) leucocytoclasia
( 4) giant cell
2,clinical classific
? infectivity
? un-infectivity
3,clinical+ histopathologic
primarily
skin
systemic
secondary
Lie CLASSIFICATION
primarily
large-vessel
Takayasu artertis
giant cell artertis
Medium-vessel
polyarteritis nodosa
Churg-Struss syndrom
Wegener’s granulomatosis
small-vessel
Microscopic polyangiitis
Schonlein-Henoch syndrom
Secondary
infectivity
drug
connective tissue disease
associated with tumer
pathogenesis
1,infectivity
2,immune complex
3,antineutrophil cytoplasmic antibody
ANCA-PR3
ANCA-MPO
4.antibody attack vessel
AECA
GBM antibody
5.cell immune
6.understand
clinical manifestation
fever
debll
general malaise,
anaemia,
myosalgia,
Prompt,
fever
rash
joint pain
myosalgia
organ impaired
the Chapel Hill Consensus
Conference
1993
giant cell artertis,
Granulomatous arteritis of the aorts and
it’s major branches with a predilection for
the extracranial branch of the carotid artery,
Often involved the temporal artery,Usually
occues in associated with polymyalgia
rheumatica,
Triad,
1.temple pain 2.intermittence mandibular
movement dyskinesia 3,acroise
age\headache\intermittence mandibular
movement dyskinesia\temporal arteritis\scalp
pain\biopsy 3/6
Takayasu artertis
Granulomatous inflammation of the aorta and
its major branches.Usually occurs in patients
younger then 5o,
Age\limping\impulse attenuated\differential
pressure>20mmHg\noise\arteriography
3/6
polyarteritis nodosa
Necrotizing inflammation of medium-size or
small arteries without glomerulonephritis or
vasculitis in arterioles,capilaries,or venules
body weight drawdown>4kg \livedo reticularis
orchialgia\myosalgia\ NS,\ high BP \ Cr \ B
hepatitis\arteriography
3/10
Microscopic polyangiitis Necrotizing
vasculitis,with few or no immune
deposits,affecting small vessel (capillaries,
venules or arterioles), Necrotizing arteritis
involving small and medium-size arteries may
be present,Necrotizing glomerulnephritis is
very common,Pulmonary capillaritis often occurs,
Nephritis
lung damage
Wegener’s granulomatosis
Granulomatous inflammation involving
respiratory tract,And necrotizing vasculitis
affecting small to medium-size
vessels(capillaries,venules,arterioles and
arteries),Necrotizing glomerulonephritis is
common,
Triad,
Upper airway
lung
nephritis
Churg-Struss syndrom
Eosinophil-rich and granulomatous
inflammation involving the respiratory tract,And
necrotizing vasculitis affacting small to medium-
size vessels,and associated with asthma and
eosinophilia,
Asthma\ Eosinophil-rich\neuritis\ lung
infiltrate\nasosinusitis\ Eosinophil infiltrate
4/6
BEH?ET’S DISEASE
it affects vessels of all sizes,
leukocytoclastic vasculitides
1,aphthous stomatitis
2,genital aphthous ulcers,
3,uveitis,
4,cutaneous vasculitis,
5,pin-prick test
2/4
GI disease,and meningoencephalitis,arthritis,
Auxiliary examination
1,ANCA
2,AECA
3,vasography
4,biopsy
DIAGNOSIS
under study and the presence of
pathognomonic clinical,laboratory,
imaging,or histopathologic features,
have been described in patients
secondary vasculitis
primary vasculitis
systemic vasculitides,
differential diagnosis
viral infection
malignancies
Treatment
1,secondary vasculitis
remove etiological factor
2,skin vasculitis
antihistamine drug +NSAIDs
3,systemic vasculitides
immune depressant
prognosis
Fudan university
Zhongshan Hospital
Yuqiang
The term vasculitis refers
to a heterogeneous group of
disorders characterized
pathologically by evidence of
blood vessel inflammation and
clinically by a diverse set of
symptoms and signs,
Pathology
three types,
1,necrotize
2,granulomas
3,only inflammatory cellular
infiltration in the vessel wall
Cause to
1.vessel wall
angusty,
blunting,
fibrosis,
aneurysm
2,tissue
ischemia,
functional impaiement
CLASSIFICATION
1,histopathologic
( 1) necrotize
( 2) granulomas
( 3) leucocytoclasia
( 4) giant cell
2,clinical classific
? infectivity
? un-infectivity
3,clinical+ histopathologic
primarily
skin
systemic
secondary
Lie CLASSIFICATION
primarily
large-vessel
Takayasu artertis
giant cell artertis
Medium-vessel
polyarteritis nodosa
Churg-Struss syndrom
Wegener’s granulomatosis
small-vessel
Microscopic polyangiitis
Schonlein-Henoch syndrom
Secondary
infectivity
drug
connective tissue disease
associated with tumer
pathogenesis
1,infectivity
2,immune complex
3,antineutrophil cytoplasmic antibody
ANCA-PR3
ANCA-MPO
4.antibody attack vessel
AECA
GBM antibody
5.cell immune
6.understand
clinical manifestation
fever
debll
general malaise,
anaemia,
myosalgia,
Prompt,
fever
rash
joint pain
myosalgia
organ impaired
the Chapel Hill Consensus
Conference
1993
giant cell artertis,
Granulomatous arteritis of the aorts and
it’s major branches with a predilection for
the extracranial branch of the carotid artery,
Often involved the temporal artery,Usually
occues in associated with polymyalgia
rheumatica,
Triad,
1.temple pain 2.intermittence mandibular
movement dyskinesia 3,acroise
age\headache\intermittence mandibular
movement dyskinesia\temporal arteritis\scalp
pain\biopsy 3/6
Takayasu artertis
Granulomatous inflammation of the aorta and
its major branches.Usually occurs in patients
younger then 5o,
Age\limping\impulse attenuated\differential
pressure>20mmHg\noise\arteriography
3/6
polyarteritis nodosa
Necrotizing inflammation of medium-size or
small arteries without glomerulonephritis or
vasculitis in arterioles,capilaries,or venules
body weight drawdown>4kg \livedo reticularis
orchialgia\myosalgia\ NS,\ high BP \ Cr \ B
hepatitis\arteriography
3/10
Microscopic polyangiitis Necrotizing
vasculitis,with few or no immune
deposits,affecting small vessel (capillaries,
venules or arterioles), Necrotizing arteritis
involving small and medium-size arteries may
be present,Necrotizing glomerulnephritis is
very common,Pulmonary capillaritis often occurs,
Nephritis
lung damage
Wegener’s granulomatosis
Granulomatous inflammation involving
respiratory tract,And necrotizing vasculitis
affecting small to medium-size
vessels(capillaries,venules,arterioles and
arteries),Necrotizing glomerulonephritis is
common,
Triad,
Upper airway
lung
nephritis
Churg-Struss syndrom
Eosinophil-rich and granulomatous
inflammation involving the respiratory tract,And
necrotizing vasculitis affacting small to medium-
size vessels,and associated with asthma and
eosinophilia,
Asthma\ Eosinophil-rich\neuritis\ lung
infiltrate\nasosinusitis\ Eosinophil infiltrate
4/6
BEH?ET’S DISEASE
it affects vessels of all sizes,
leukocytoclastic vasculitides
1,aphthous stomatitis
2,genital aphthous ulcers,
3,uveitis,
4,cutaneous vasculitis,
5,pin-prick test
2/4
GI disease,and meningoencephalitis,arthritis,
Auxiliary examination
1,ANCA
2,AECA
3,vasography
4,biopsy
DIAGNOSIS
under study and the presence of
pathognomonic clinical,laboratory,
imaging,or histopathologic features,
have been described in patients
secondary vasculitis
primary vasculitis
systemic vasculitides,
differential diagnosis
viral infection
malignancies
Treatment
1,secondary vasculitis
remove etiological factor
2,skin vasculitis
antihistamine drug +NSAIDs
3,systemic vasculitides
immune depressant
prognosis