vasculitisjeabnoii

8/4/2019 VasculitisJeabnoii

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Whatisvasculitis?

s Vasculitisisas heterogeneousdisorders Vascularinflammation

s Classified:s Cause :primary,secondarys Sizeofvessels :small,medium,larges Ab-associated :ANCA,ANAs Pathology :granulomatous,

immune/pauci-immune


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ClassificationofVasculitis

s Primaryvasculitiss Largevessels Mediumvessels Smallvessels miscellaneous

s Secondaryvasculitiss Infectionassociateds CNTDassociateds Druginduceds Malignancyassociateds Postorgantransplant

IsolatedvasculitisSystemicvasculitis


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Secondaryvasculitiss Infection-associated s Viral:HBV,HCV,HIV,Herpes,CMV,parvoB19

s Bacteria:strep,staph,salmonella

s Mycobacteria:TB,atypicalmycobacteria

s Syphylis


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Secondaryvasculitiss Infection-associateds CNTD-associated

s SLEs MCTDs Dermatomyositiss RAs Sjogrenssyndrome


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Secondaryvasculitiss Infection-associateds CNTD-associateds Drug induced

s Penicillins Sulfonamides Oralcontraceptiveagentss Allopurinols PTUs Hydralazine


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Secondaryvasculitiss Infection-associateds CNTD-associateds Drug induceds Malignancy-associated


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Secondaryvasculitiss Infection-associateds CNTD-associateds Drug induceds Malignancy-associateds

Post-organ transplant


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Larges aortaanditsmajorbranches(andthecorrespondingvesselsinthevenouscirculationinsomeformsof

vasculitis,e.g.,Behet'sdisease).

Mediums smallerthanthemajoraorticbranchess containfourelements:(1)anintima,(2)acontinuousinternalelasticlamina,(3)amuscularmedia,(4)an

adventitia.s Inclinicalterms,mediumvesselvasculitisisgenerallymacrovascular(i.e.,involvesvesselslargeenoughtobeobservedingrosspathologicspecimensorvisualizedbyangiography).

Smallvessels allvesselsbelowmacroscopicdisease,includescapillaries,postcapillaryvenules,andarterioles

s lessthan500inouterdiameters Glomeruli:differentiatedcapillaries


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Classificationss ACRcriteria1990s Chapel-Hillconcensusconference1994s EULAR


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AmericanCollegeofRheumatology

(ACR)1990criteria

s Classify7vascultissyndromess Giantcellarteritiss Takayasuartieritiss Henoch-Schonleinpurpuras Hypersensitivityvasculitiss Polyarteritisnodosas Churg-StraussSyndromes Wegenersgranulomatosis


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1994:CHCCclassification(ChapelHillConsensusConference)

BVSize PrimaryVasculitis Histology

Large GCA Granulomatousarteritisofaortaanditsbranch(ExtCarotidA.)

TK Granulomatousarteritisofaortaanditsbranch

Medium PAN NecrotizingvasculitisofmediumandsmallBV

KW Large,meduimandsmallarteritis(Coronarya.)

Small WG Granulomatousnecrotizingvasculitis(sm-M)

CSS Granulomatousnecrotizingvasculitis(Eo-sm-M)

MPA NecrotizingvasculitisofsmallBV

HSP IgA-depositvasculitsofsmallBV

EssCryo Cryoglobulin-depositvasclitisofsmallBV

Cut.LCV IsolatedcutaneousLCVnosystemic


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Classifications

primary secondary

Large artery Giant cell arteritis

Takayasus arteritis

Isolated CNS angiitis

Aortitis asso with RA

syphilis

Medium artery PAN

Kawasaki

Hepatitis B

Small andmedium vessel

WC

CSS

MPA

SLE

Sjogrens syndrome

Drug HIV

Small vessel HSPCutaneous LCV

Essential mixed

cryoglobulinemia

Drug

Hepatitis B, C

Miscellaneous:Cogan,Behcets,Relapsingpolychondritis


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aorta

large & medium

small

arteriole

capillary

venule

veinvena cava

Buergersdisease

Giant cell & Takayasu arteritis

Classic PAN, Kawasaki

Wegener, MPA, Churg Strauss

Henoch SchonleinCryoglobulinemia

Small vessel angiitis

Behcets disease, Relapsing polychondritis,Cogans


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leukocytoclastic vasculitiscutaneous vasculitishypersensitivity vasculitis


aorta

large & medium

small

arteriole

capillary

venule

veinvena cava






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aorta

large & medium

small

arteriole

capillary

venule

veinvena cava

Behcets disease

less common in Westernsaseptic meningoencephalitis

cerebral vasculitisrecurrent phlebitis, arteritis, synovitis


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Tissuehistopathology

s Granulomatousinflammations Immunecomplex-mediateds ANCAassociated(pauci-immune)


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GranulomatousInflammation

s Giantcellarteritiss Takayasu'sarteritiss Cogan'ssyndromes Wegener'sgranulomatosiss Churg-Strausssyndromes PrimaryangiitisofCNS[]s Buerger'sdisease[]s Rheumatoidvasculitiss Sometimesgranulomatous.s Giantcellsoccurwithininflammatorythrombi(andarediagnosticof

Buerger'sdisease),butdonotoccurwithinthebloodvesselwall.


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Immunecomplexassociatedvasculitis

s Anti-GBMdisease(Goodpasture'sdisease)s Henoch-Schnleinpurpura:IgA1s MixedCryoglobulinemia:IgG,IgM,,HCVs HBV-associatedPANs CNTD-associatedvasculitiss Hypersensitivityvasculitiss Erythemaelevatumdiutinum


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ANCAassociateds WGs CSSs MPAs PAN


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Approachtovasculitis

vIdentifycommonclinicalfeaturesofvasculitisvExcludepseudo-vasculitis(mimics)vLookforcausesofvasculitis(secondaryvasculitis)vDefineprimaryvasculitis(categorizegroup)vConfirmdefinitediagnosis(tissuebiopsy/angiogram)vStartappropriatetreatment


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ClinicalFeaturesSuggestingVasculitis

s FUOs Ischemicsymptom(esp.intheyoung)s Suspeciousrashs Mononeuritismultiplexs Unexplainedmultisysteminvolvements Unexplainedglomerulonephritiss Unexplainedinflammatorymyositis/arthritis


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ClinicalFeaturesSuggestingVasculitis

vSkin Palpablepurpura Livedoidvasculitis Urticarialvasculitis Panniculitis Chroniculcers Digitalgangrene

vNervoussystem PNSs Mononeuritismultiplexs Peripheralneuropathy

CNSs Strokes Seizures Visualdisturbances Cranialneuropathy


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Clinicalfeaturessuggestingvasculitis

vRespiratorytract Asthma,sinusitis Pulmonaryhemorrhage Pulmonaryinfiltrationornodules

vKidneys Renovascularhypertension Abnormalurinesediment Renalfailure

vGastrointestinalsystemBowelangina

Ischemicbowel GIhemorrhage

vMajorvesselsJawclaudication

Extremityclaudication Hypertension Aneurysm


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MimicVasculitisInfec&ousdiseases

Bacterialendocardi-s**DGI**PulmonaryhistoplasmosisCoccidioidomycosisSyphilisLymediseaseRockyMountainspo?edfeverWhipple'sdisease

DrugtoxicityCocaine**Amphetamines**Ergotalkaloids**MethysergideArsenic

Miscellaneous

Atheroembolicdisease**Goodpasture'ssyndrome**SarcoidosisAmyloidosisMigraineCryofibrinogenemia

Neoplasms**

AtrialmyxomaLymphomaCarcinomatosis

Thrombosis**

An-phospholipidsyndromeTTP


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SecondaryvasculitisEvaluate History

s Infection-associateds CNTD-associateds Drug induceds Malignancy-associateds Post-organ transplant


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Post-capillaryvenulesPalpablepurpura Urticarialvasculitis


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Arteriolesmalla.

Skinulcer


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Smallmediumsizeda.

Livedoreticularis

EN

Gangrene


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DDx:Erythemanodosums Infections Drugs Inflammations Rheumatos Malignancys Idiopathic


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Mononeuritismultiplex


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TypicalClinicalManifestationsofLarge,Medium,andSmallVesselInvolvementbyVasculitisConstitutionalsymptoms:

fever,weightloss,malaise,arthralgias/arthritis(allvesselsizes)

Large Medium SmallLimb claudication Cutaneous nodules PurpuraAsymmetric bloodpressures

Ulcers Vesiculobullous lesions

Absence of pulses Livedo reticularis UrticariaBruits Digital gangrene GlomerulonephritisAortic dilation Mononeuritis multiplex Alveolar hemorrhage

Renovascularhypertension Microaneurysms Cutaneous extravascularnecrotizing granulomasRenovascularhypertension

Splinter hemorrhages

Uveitis/episcleritis/scleritis


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ConsiderationsintheClassificationsofsystemicvasculitis

s Sizeofpredominantbloodvesselsaffecteds Epidemiologicfeaturess Ages Genders Ethnicbackground

s Patternoforganinvolvements Pathologicfeaturess Granulomatousinflammations ImmunecomplexdepositionVSpauci-immunehistopathologys Linearstainingalongglomerularbasementmembrane

s PresenceofANCA,anti-GBMAb,RFs Associatedinfection(hepatitisBorhepatitisC)


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Epidermiology

s AGE,GENDER,ANDETHNICITYs KW:80%


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EpidermiologyDisease United States Elsewhere Age/Gender/EthnicPredispositionsGCA 240/1 million

(Olmsted County,MN)

220-270/1 million(Scandinaviancountries)

Age >50, mean age 72/Females 3:1/NorthernEuropean ancestry

TK

3/1 million

200-300/1 million(India)

Age > BlacksHSP In children: 135-180/1 million; in adults:

13/1 millionOnly 10% of cases occur inadults

From Gonzalez-Gay MA, Garcia-Porrua: Epidemiology of the vasculitides. Rheum Dis Clin N Am27:729-750, 2001.


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Epidermiology

s GENESs Familialrares exceptGCAHLA-DRB10401andHLA-DRB10101,tumor

necrosisfactormicrosatellitepolymorphisms

s Polygenicandcomplex.s Singlegeneandvasculitiss HLA-B51withBehet'sdisease,80%ofAsianpatients

s Rheumatoidvasculitis:s HLA-DRB1,genotypes0401/0401,0401/0404,and0401/0101s HLA-C3inpatientslackingHLA-DRB104

s WG:s functionalpolymorphism,620W,intheintracellulartyrosine

phosphatasegenePTPN22


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Epidermiology

s ENVIRONMENTs cigarettesmokings Buerger'sdiseaseandrheumatoidvasculitis

s IVDUs Infections HBVHCVVZVHIV


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Investigations

1.Excludemimic

2.Assessextentofvasculitis

3.Confirmdiagnosis

4.Identifyspecifictypeofvasculitis


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Investigations

1.Excludemimic


3.Confirmdiagnosis


s H/Cs Hepatitisprofiles AntiHIVs Antiphspholipidchanels ANA,RFs Echos AntiGBM


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Investigations

1.Excludemimic


3.Confirmdiagnosis


UA

CXR

EMG/NCV

EEG/LP


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Investigations

1.Excludemimic


3.Confirmdiagnosis


s Biopsys Angiogram


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Investigations

1.Excludemimic


3.Confirmdiagnosis


s ANCAs Cryoglobulins Complement(C3C4CH50)s Eosinophilia/IgE(CBC)s Specificbiopsystaining:s Necrotizings Granulomatousinflams IgAdeposits ICdeposit


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AntineutrophilicCytoplasmicAb(ANCA)s firstdescribedin1982byDaviesandcolleagues:necrotizingpauci-immuneglomerulonephritiswhoweresuspectedofhavingviralinfections.

s Hallandcolleagues:ANCAwithsystemicvasculitis.s In1985,vanderWoudeandcolleagues:ANCA&WGs BenefitDDx smallmediumsizedvasculitis


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C-ANCA

50-70%myeloperoxidase(MPO)

20-50%others

P-ANCA

>95%Proteinase-3


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p-ANCAinselecteddisease


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leukocytoclastic vasculitiscutaneous vasculitishypersensitivity vasculitis


aorta

large & medium

small

arteriole

capillary

venule

vein

vena cava






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