类风关文档格式.docx
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类风关文档格式.docx
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Etiology:
Despitemanyyearsofintensiveinvestigations,theetiologyofRAremainsobscure.Therecentfindingofanassociationwithcertainallelesofhemajorhistocompatibilitycomplexsuggeststhatgeneticfactorscaninfluencetheexpressionofthedisease,perhapsbytheireffectsontheimmunologicphenomenathatplayanimportantroleindiseasepathogenesis.BesidesthatcertainbacteriaandvirusaresuspectedtobethetriggerofRA.
Rheumaticfactor:
(RF)
RFareantibodieswithspecificitytoantigenicdeterminantsontheFcportionofhumanoranimalIgG.StandardflocculationtestsdetectIgMRFintheseraofapproximately70%ofadultpatientsofRA.RFpositivecanalsobefoundinotherdiseasesoreveninoldhealthypeople.HightiterofRFisgenerallyassociatedwithmoresevereandactivejointdisease,thepresenceofnodules,greaterfrequencyofsystemicmanifestationsofRAandapooreroutcome.Currently,themostpopularnotionthatRFariseasantibodiesto“altered”autologousIgG.AlterationisthoughttooccurwhenanativeIgGantibodymoleculecombineswithitsspecificantigen,whichchangestheconfigurationoftheIgGmoleculerevealingneworpreviouslyhiddenantigenicdeterminants,therebyrenderingitanautoimmunogen.
BesidesRA,IgMRFfrequentlypresentinfollowingdiseases:
Sjogren’ssyndrome,systemiclupuserythematosus,progressivesystemicsclerosis,polymyositis/dermatomyositis,bacterialendocarditis,tuberculosis,syphilis,infectioushepatitis,schistosomiasis,normalindividualespeciallyaged,diffuseinterstitialpulmonaryfibrosis,cirrhosisofliver,chronicactivehepatitisandsarcoidosis.
RFusuallyabsentinosteoarthritis,ankylosingspondylitis,gout,chondrocaleinosis,psoriaticarthritis,enteropathicarthritisReiter’ssyndromeandsuppurativearthritis.
TheexactbiologicalroleofRFisunknown.Antiviralpropertieshavebeenascribedtothem.Itisfoundthattheycanaffecttheinflammatoryresponsebyenhancingcomplementfixation,byalteringthepropertiesofimmunecomplexes,orbyrenderingthembymoresusceptibletophagocyticcells.Theselattercharacteristicsareprobablyimportantinsynovialtissuesandfluids.
Pathology:
Thesynovialmembraneistheprimarysiteofinvolvement.Itbecomesthickened,withprominentvillousformationandeffusionofinflammatoryfluidintothejointcavity.Therearediffuseexudationandinflammatorycellinfiltration.Lininglayerincreasesindepth.Inthesubintimallayer,TcellspredominantlyCD4+cellsconstitute30-50%ofsynovialtissuecellsandclusterinperivasularaccumulations.Themostcharacteristicpathologyintherheumatoidjointisthepannusformation,asheetofinvasivecellulartissue,incontinuitywithsynoviallininglayerandpresumablyderivedfromit,whicherodescartilageandboneatthemarginofthejoint.
Theextra-articularpathologyincludesrheumatoidnodulesandvasculitis.Thenodulesconsistsofaninnercoreoffibrinoidnecrosis,asurrounding“fence”madeuppredominantlyofmacrophagesandanouterareaoflooselyorganizedperivascularchroniclymphoidinfiltration.
Pathogenesis:
RAisautoimmunereactionbynature.ItinvolvesbothTandBlymphocytesaswellassomecytokineslikeTNFα,IL-I.Unknownantigenismodifiedandpresentedbyantigen-presentingcellstoactivateThelpercells,whichinturnsecretemanycytokinestostimulateBcellsresultinginthemassproductionofimmunoglobulinincludingRF.Allthesechangeswillcreateaninflammatoryprocess.
Clinicalfeatures:
1.Articularandothersynovialdisease
TheonsetofRAisfrequentlyheraldedbyprodromalsymptomssuchasfatigue,anorexia,weightloss,weakness,,andgeneralizedachingandstiffness.Approximately20%ofpatientshaveanabruptonsetofpolyarthritis,oftenaccompaniedbyfeverandprostration.Themanifestationsarepain,stiffnesslimitationofmotionandthesignsofinflammation.Morningstiffnesslastingmorethanhalfanhourmaybeoneoftheimportantcluesfordiagnosis.Inearlydiseasejointlimitationisusuallycausedbypainwhilelatermaybecauseofcapsularfibrosis,musclecontractureorbony/fibrousankylosis.Themostcommonlyinvolvedinitiallyarethesmalljointsofhands,wrists,knees,andfeet.Itisusuallybilateral,symmetricalandpolyarticularinvolved.Asthediseasebecomesestablished,thearthritisspreadstotheelbows,shoulders,sternoclavicularjoints,hips,anklesandsubtalarjoints.Lesscommonlythetemporomandibularandcricoarytenoidjointsareaffected.Clinicallysignificantspinalinvolvementisusuallylimitedtotheuppercervicalarticulations.
Inearlystage,swellingoftheproximalinterphalangeal(PIP)jointsproducesaspindle-shapedappearanceofthefingers.Bilateralsndsymmertricalswellingofthemetacarpophalangeal(MCP)joints,particularlythesecondandthird,isverycommonandremainslongafterPIPjointinflammationhassubsided.Thedistalinterphalangeal(DIP)jointsaremoreoftenspared.Asthediseaseprogresses,laxityofsofttissuesincreasesandunderthepressureofregularuse,typicalhanddeformitiesdevelop.Ulnardeviationofthefingersisparticularlycommon,oftenaccompaniedbypalmarsubluxationoftheproximalphalanges.HyperextensionofthePIPjointsinconjunctionwithflexionattheDIPjointconstitutestheswanneckdeformity.TheboutonnieredeformityisaflexiondeformityofthePIPjoints.InvolvementofthethumbscauseshyperextensionoftheinterphalangealjointsandflexionattheMCPjointswitharesultantlossofpinch.
WristdiseaseisalmostinvariableaccompanimentofRA.Activesynovitiscanbeobservedatthedorsumofthewristasaboggy.Softtissueswelling,synovitishypertrophyandtenosynovitisonthevolaraspectmaycompressthemediannervebeneaththetransversecarpalligament,producingacarpaltunnelsyndromewithparesthesiaanddysesthesiaofthethumb,second,third,andradialaspectofthefourthdigits.Thethenareminencemayatrophy.
Hipjointsarelessinvolved.Afrequentcomplaintisdiscomfortinthegroin;
lessoftenthepainisreferredtothebuttocksorlowerback.Swellingandtendernessaredifficulttonoteandhipdiseasemayberecognizedonlybecauseofgaitabnormalitiesorlimitationofjointmotion.
Thekneeisamongthemostfrequentlyaffectedjointsandisresponsibleformuchdisability.Synovialhypertrophyandchroniceffusioncanbepronounced.Aregularaccompanimentofkneeinvolvementisquadricepsatrophy,oftenofgreatseverity.
Feetandanklearthritiscreateanumberofvexingproblemsduetopainandlimitationofflexionandextension.
Symptomsofintermittentneckpainandstiffnessarefrequent.Thespinalcordmaybecompromisedbyanteriordislocationofthefirstcervicalvertebrae.Verticalsubluxatoncaninducetorsionandcompressionofthevertebralarterieswithsymptomsofvertebrobasilarinsufficiencyandsyncopeondownwardgaze.
Extra-articulardisease:
Rheumatoidnoduleappearsatsometimeinapproximately20-25%ofthepatients.Theyareoftenassociatedwithseropositive(RF)andperhapswithamoresevereanddestructivearthritis.Nodulesareroundorovalmassinsubcutaneousordeeperconnectivetissues,varyingfromlessthan0.5cmtoseveralcentimetersindiameter.Peri-articularstructuresandareassubjectedtomechanicalpressurearecommonsitesespeciallytheolecranonbursae,extensorsurfaceoftheforearmandtheAchillestendon.
Vasculitis:
thepathogenesisofrheumatoidvasculitisisuncertain,indirectevidencesuggeststheparticipationofimmunecomplexes.Theymaytakemanyformsascapillarritis,venulitisorinrarecasewidespreadnecrotizingarteritis.Theyproducethesymptomsofdigitalgangrene,skinulcerandnecrosis,sensorimotorneuropathyandvisceralinfarction.
Cardiacmanifestations:
pericarditisoccursinabout40%atautopsy.Lesscommonaregranulomatouslesions,similartorheumatoidnodules,involvingtheepicarduim,myocardiumandvalves;
focalinterstitialmyocarditis;
andarteritisofcoronaryvessels;
occasionallyvalvularinsufficiencyorconductionabnormalitiesarerecognizedduringlife.
Pleuropulmonarymanifestations:
Rheumatoidpleuraldiseaseisusuallyasymptomatic.Pleuraleffusionmayhappenin10%ofthepatientswithhydrothorax.Solitaryormultiplenodulesareoccasionallyseeninthechestfilmespeciallythosewithsubcutaneousnodules.Diffuseinterstitialfibrosisisnotrare.InlatestageitmayprogresstothehoneycombappearanceinCTscanwithamarkeddecreaseinpulmonaryfunction.
PatientswithhightiterofRFprobablydevelopsecondarySjogren’ssyndrome.Theremaybecornealandconjunctivallesions.
LymphnodeenlargementiscommoninRApatients.Palpablesplenomegalyoccursinupto10%ofpatients.Felty’ssyndromereferstothoseRApatientswhohavebothsplenomeaglyandleukopenia.RecurrentGrampositiveinfectionarecommonandfrequentlyrespondpoorlytoantibiotics.
Labfi
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