Rheumatology-Main风湿病

RheumatologyMain91-100,Q1,A34-year-oldwomancomestotheclinicwithafacialrash,jointpainsrandoralulcers.Shehasnoassociatedfeverorothersystemicsymptomsandotherwisefeelswell.Medicalhistoryisunremarkable,andthepatientsonlyconcurrentmedicationisanoralcontraceptive.Shedoesnotsmokeandconsumesalcoholonlyonsocialoccasions.Antinuclearandanti-double-strandedDHAantibodytitersareelevated.Creatinineandcomplementlevelsarenormal.Thepatientisdiagnosedwithsystemiclupuserythematosus,andtherapywithhydroxychloroquineisInitiated.Whichofthefollowingevaluationsshouldbedoneperiodicallywhilethepatientistakingthismedication?A.AudiometryB.EchocardiogramC.EyeexaminationD.LiverfunctionpanelE.PulmonaryfunctiontestsF.Thyroid-stimulatinghormone,A1,Correctanswer:CThispatient;withanewdiagnosisofsystemiclupuserythematosus(SLE);haslimiteddisease,withskin,joint,andmucosalmanifestations.DiseaseactivityinSLEshouldbemonitoredwithperiodicbloodcounts,inflammatorymarkers(eg,erythrocytesedimentationrate),complementlevels,andanti-double-strandedDNAantibodytiters.Patientsshouldalsobescreenedregularlyforlupusnephritiswithurinalysis,serumcreatinine,andurineproteinassay.ThechoiceofdrugtherapyinSLEdependsonthedegreeoforganinvolvement.MostpatientswithactiveSLEbenefitfromhydroxychloroquine,asafeandweil-toleratedmedication.However,itcancauseretinaltoxicityonrareoccasions,withapotentialforirreversiblevisionloss.Thisismostcommonafter5-7yearsoftherapy.Forthisreason,patientstreatedwithhydroxychloroquineshouldhaveabaselineophthalmologicevaluation,withannualreassessmentbeginningafter5years.(ChoiceA)Ototoxicityisacommonadverseeffectofaminoglycosideantibioticsandplatinum-basedchemotherapeuticagents.Hearingimpairmenthasbeenreportedwithhydroxychloroquine,butroutineaudiometryisnotcurrentlyrecommended.(ChoiceB)Echocardiographyisusedtoassesslupusmyocarditisandcanidentifycardiotoxicityinpatientswithrheumatoidarthritistakingtumornecrosisfactorinhibitors(eg,etanercept).Cardiotoxicityisnotasignificantconcernwithhydroxychloroquine.(Choices0andE)Methotrexateisusedasasteroid-sparingagentinpatientswithmoderatetosevereSLE.Methotrexateisassociatedwithmultisystemtoxicityaffectingliver,lungs,mucosa,andbonemarrow.HepaticmarkersarefrequentlymonitoredinpatientswithSLEwhotakemethotrexate,buthydroxychloroquinedoesnotrequireroutinelivertesting.(ChoiceF)Amiodaronecancauseavarietyofendocrinopathies,includingbothhypo-andhyperthyroidism(forwhichperiodicthyroidfunctionmonitoringisrecommended).Educationalobjective:Hydroxychloroquineisusedforpatientswithactivesystemiclupuserythematosus.However,itcancauseretinaltoxicitywithprolongeduse.Patientstreatedwithhydroxychloroquineshouldhaveabaselineophthalmologicevaluationandperiodicreassessment.,Q2,A49-year-oldwomancomestotheofficeduetoswellingandstiffnessofthehandsforthepastseveralmonths.Forthelastyearshehasalsohadintermittentepisodesofpainandbluishdiscolorationofthefingers,especiallywhenexposedtocold.Thepatientisunabletoplaytennisonweekendslikeshedidbeforeduetofatigueandbreathlessness.Shetakespantoprazoleforheartburnandlisinoprilforhypertension.Thepatientdrinksalcoholonsocialoccasionsbutdoesnotsmokecigarettes.Temperatureis37.1(98.8);bloodpressureis140/90mmHg;andpulseis78/min.Physicalexaminationshowspuffinessofthehandsandskinthickeninginthefingers,asshownintheimagebelow.Lungauscultationrevealsbibasilarfineinspiratorycrackles.Whichofthefollowingtestsismostlikelytobepositiveinthispatient?A.AnticardiolipinantibodiesB.Anti-cycliccitrullinatedpeptideantibodiesC.AntimitochondrialantibodiesD.Anti-neutrophilcytoplasmicantibodiesE.Anti-smoothmuscleantibodiesF.Anti-topoisomeraseIantibodies,A2,Correctanswer:Systemicsclerosis(SSc,oftentermedscleroderma)ischaracterizedbyconnectivetissuethickeningduetofibroblastdysfunctionandIncreasedcollagenandgroundsubstanceproduction.Cutaneousfeaturesincludethickeningoftheskinthatbeginsinacralsites(handsandfeet)withedemathattransitionstodermalsclerosis(sclerodactyly)andobliterationofskinappendages(eg，hairfollicles;sweatglands).Vasculardysfunction(eg，Raynaudphenomenon)ispresentinmostpatients.Extradermalmanifestationsarecommon,especiallyinpatientswithmoreextensiveskininvolvement.Frequentcomplicationsincludeesophagealdysmotility,interstitiallungdisease(eg,fatigue,breathlessness,finecrackles),andhypertension(duetorenalinvolvement).ThediagnosisofSScisbasedprimarilyonclinicalmanifestationsandserologicmarkers.Antinuclearantibodiesarepresentinalmostallpatientsbutarenonspecific.Anti-topoisomeraseI(anti-Scl-70)andanti-RNApolymerase111arelesssensitivebutmorespecificandareassociatedwithextensivedisease.Anticentromereantibodiesmayalsobeseen,primarilyinpatientswithlimiteddisease.(ChoiceA)Cardiolipinisthemostcommonphospholipidsubstrateusedinantiphospholipidassays.Anticardiolipinantibodiesarepresentinpatientswithantiphospholipidsyndrome,characterizedbyarterialorvenousthrombosisandintrauterinefetaldemise.(ChoiceB)Rheumatoidfactorispositiveinanumberofautoimmunedisorders,mostprominentlyrheumatoidarthritis.Anti-cycliccitruliinatedpeptideantibodiesareamorespecificmarkerofrheumatoidarthritis.ThesemarkersmaybepositiveinpatientswhohaveanoverlapsyndromewithfeaturesofSSc,butmostpatientswouldhavemoreprominentjointinvolvement(eg,symmetricmorningstiffness).(ChoiceC)Antimitochondrialantibodiesareassociatedwithprimarybiliarycholangitis(primarybiliarycirrhosis),whichcanpresentwithfatigue,pruritus,andrightupperquadrantdiscomfortalongwithanelevatedserumalkalinephosphataselevel.(ChoiceD)Anti-neutrophilcytoplasmicantibodiesarepresentIngranulomatosiswithpolyangiitis(Wegenergranulomatosis),microscopicpolyangiitis,andotherformsofautoimmunevasculitis.(ChoiceE)Anti-smoothmuscleantibodiesareassociatedprimarilywithautoimmunehepatitis,whichcanpresentwitharangeofmanifestations,fromanasymptomaticelevationofaminotransferasestohepatosplenomegaly,cirrhosis,orliverfailure.Educationalobjective:Systemicsclerosisischaracterizedbyconnectivetissuethickeningduetofibroblastdysfunction.Cutaneousfeaturesincludethickeningoftheskinwithdermalsclerosisandvasculardysfunction(eg,Raynaudphenomenon).Extradermalmanifestationsincludeesophagealdysmotility,interstitiallungdisease,andhypertension.Anti-topoisomeraseI(anti-Scl-70)isaspecificserologicmarkerandisassociatedwithextensivedisease.,Q3,A45-year-oldwomancomestothephysicianwitha3-monthhistoryofexertionaldyspneaandnonproductivecough.Shehasalsobeenhavingdifficultyswallowing.HerothermedicalproblemsincludeRaynaudphenomenon,heartburn,andpoorlycontrolledhypertension.Theskinoverherhands,arms,andtrunkisthickandfirm.Whichofthefollowingisthemostlikelycauseofherpulmonarycomplaints?A.InterstitiallungdiseaseB.ParenchymalgranulomatousnodulesC.PleuraleffusionD.PulmonaryarterialhypertensionE.Restrictedchestwallmovement,A3,Correctanswer:AThispatient;withdysphagia,Raynaudphenomenon,andextensiveskinthickening,mostlikelyhasdiffusesystemicsclerosis(SSc).SScdisplaysextensiveheterogeneityinmanifestationsandseverity,thoughsomedegreeofskinthickeningisseenInalmostallpatients.Datientswithsclerosislimitedtotheskinofthehands,wrists,andface(limitedSSc)mayhaveesophagealdysmotiiityandvascularsymptoms(eg,Raynaudphenomenon),butextradermalmanifestationsareotherwiselimitedinmostcases.Incontrast,diffusecutaneousSSc,withdermalthickeninginvolvingtheproximalextremitiesortrunk,ismuchmorelikelytocauseextradermalcomplicationsandcarriesamoresevereprognosis.AwiderangeofpulmonarycomplicationscanoccurinpatientswithSSc.Theyaffectupto30%ofpatients,andaretheleadingcauseofdeathinSSc.ThemostcommonpulmonarycomplicationinpatientswithdiffuseSScisinterstitialfibrosis,whichdevelopsinabout40%ofpatients.(ChoiceB)Sarcoidosisaffectsthelungsin90%ofcases.Inadditiontononcaseatinggranulomas,patientswithsarcoidosiscandevelophilaradenopathy,fibrosis,bronchiectasis,andpleura!effusions.Dermalmanifestationsofsarcoidosismayincludeerythemanodosum,butsclerosisisuncommon.(ChoiceC)Pleuraleffusionsareacommonextra-articularmanifestationofrheumatoidarthritisbutareuncommoninpatientswithSSc.(ChoiceD)Pulmonaryarterialhypertension,clinicallysimilartoidiopathicpulmonaryhypertension,isapossiblebutlesscommoncomplicationofSSc.Itoccursinupto15%ofpatients,primarilyinthosewithlimitedSSc(eg，CRESTsyndrome).(ChoiceE)RestrictionofchestmovementsduetoextensivethoracicskinfibrosisisapossiblebutrarecomplicationofSSc.Educationalobjective:Patientswithdiffusesystemicsclerosis(SSc)frequentlyhaveextradermalorganinvolvement,especiallyofthelungs.ThemostcommonpulmonarycomplicationinpatientswithSScisinterstitialfibrosis.,Q4,A42-year-oldwomancomestotheofficewitha4-monthhistoryofheartburn.Shedescribesaperiodicstickingsensationinherchestduringmeals.Inaddition,thepatienthasrecentlybeenunabletoparticipateinhernormalexerciseroutineduetodyspneaonexertionandjointpaininherhandsandfeet.Shedoesnotusetobacco,alcohol,orillicitdrugs.Lungexaminationrevealsbilateralend-inspiratorycrackles.Endoscopicevaluationshowsmildhyperemiainthedistalesophagus.Esophagealmanometryshowslackofperistalticwavesinthelowertwo-thirdsoftheesophagusandasignificantdecreaseinloweresophagealsphinctertone.Whichofthefollowingisthemostlikelymechanismresponsibleforthispatientsmanometricfindings?A.DysfunctionofinhibitoryneuronsB.EosinophilicinfiltrationofesophagealmucosaC.LossofintramuralneuronsD.SmoothmuscleatrophyandfibrosisE.Striatedmuscleinflammation,A4,Correctanswer:DThispatienthasanumberoffindings(eg.esophagealdysmotility,fibroticlungdisease,arthralgias)consistentwithextradermalmanifestationsofsystemicsclerosis(SSc).ClassicearlyskinmanifestationsofSScincludethickeningorhardening,edema,andpruritus.However,ifskinsymptomsaremild,patientsmayfirstseekattentionduetogastrointestinal(Gl),joint,orrespiratorydisease.GlcomplicationsarecommoninSScandprimarilyaffecttheesophagus.SSccausessmoothmuscleatrophyandfibrosisintheloweresophagus.Commonsymptomsincludedysphagia,choking,heartburn,andhoarseness.Esophagealmanometryinaffectedpatientstypicallyshowshypomotilityandincompetenceoftheloweresophagealsphincter(LES).(ChoiceA)Dysfunctionofinhibitoryneuronscausesdiffuseesophagealspasmandpresentswithchestpainanddysphagiaratherthanheartburn.ManometryIscharacterizedbyperiodic,high-amplitude,non-peristalticcontractions.(ChoiceB)Eosinophilicesophagitisischaracterizedbyheartburnthatdoesnotrespondtostandardmedicationsforgastroesophagealrefluxdisease.Manometrymostoftenshowsesophagealhypercontractility.(ChoiceC)Achalasiapresentswithdysphagiaandregurgitationofundigestedfood.AsinSSc,manometryshowsaperistalsisinthedistalesophagus.However,achalasiacausesincreasedLESpressureandincompleteLESrelaxation,whereasSSccausesdecreasedLESpressure.(ChoiceE)Esophagealinvolvementmayoccurinpolymyositis.Itcaninvolveboththeupperandloweresophagusandischaracterizedbydysphagia,regurgitation,andaspiration.ManometryresultsareoftenfunctionallysimilartoSSc,butmostpatientswillhavesymmetricproximalmuscleweakness,notdistalarthralgias.Educationalobjective:Systemicsclerosiscancauseatrophyandfibrosisofthesmoothmuscleintheloweresophagus.ThisleadstodecreasedperistalsisanddecreasedtoneIntheloweresophagealsphincter.Typicalsymptomsincludeheartburnanddysphagia.,Q5,A29-year-oldwomancomestotheofficeduetoa6monthhistoryofnumbnessandpaininherupperarms.ThepainIsworseontheleftsideandisexacerbatedbyliftingorotheractivity.Associatedsymptomsincludefatigue,fleetingjointpains,anda5-kg(11-lb)weightloss.Thepatientsmedicalhistoryisunremarkable,andshedoesnotusetobaccoorillicitdrugs.SheemigratedfromVietnam5yearsagoandworksinafurniturerepairshop.Thepatientsbloodpressureis140/90mmHgintherightarmand90/55mmHgintheleftarm,andherpulseis78/minandregular.Conjunctivalandoralmucosaaremoistandwithoutlesions.Cardiopulmonaryexaminationshowsclearlungfieldsandnoheartmurmurs;however,abruitisheardintherightsupraclavicularfossa,andtheleftradialandbrachialpulsesaredecreased.Abdominalexaminationisunremarkable.Noskinlesionsarepresent.Completebloodcount,electrolytes,andrenalfunctionarenormal.Erythrocytesedimentationrateis40mm/hr.Whichofthefollowingisthemostlikelydiagnosisforthispatient?A.AorticdissectionB.CoarctationoftheaortaC.GiantcellarteritisD.KawasakidiseaseE.PolymyositisF.TakayasuarteritisG.Thromboangiitisobliterans,A5,Correctanswer:FThispatientwithexertionalarmpain(likelyclaudication),systemicsymptoms,andpulsedeficitshastypicalfeaturesofTakayasuarteritis,achroniclargearteryvasculitisthatpredominantlyaffectsAsianwomenage50.Typicalfindingsincludetemporalorscalptenderness,andmostpatientshavechronicpainandstiffnessoftheshouldersandhips(ie,polymyalgiarheumatica).(ChoiceD)KawasakidiseaseIsanacuteillnessofchildrencharacterizedbyfever,mucositis,conjunctivitis,cervicaladenopathy,rash,andedemaofthehandsandfeet.Itisaself-limiteddisorderandresolveswithin1-2weeks.(ChoiceE)Polymyositisischaracterizedbypainlessproximalmuscleweaknesswithelevatedserumlevelsofmuscleenzymes(eg,creatinekinase).Itdoesnotcausearterio-occlusivemanifestations.(ChoiceG)Thromboangiitisobliterans(Buergerdisease)occursprimarilyinmenwhoareheavysmokers.Findingsincludesuperficialthrombophlebitisandischemiaandgangreneofthedigits.Educationalobjective:TakayasuarteritisisalargearteryvasculitisthatismostcommoninyoungAsianwomen,initialsymptomsincludefever,arthralgias,andweightloss.Laterfeaturesincludearterio-occlusivesymptoms,bloodpressurediscrepancies,andpuisedeficits.CTandMRIcanrevealaneurysmformationorlumenalnarrowing.Treatmentincludessystemicglucocorticoids.,Q6,A75-year-oldwomancomestotheemergencydepartmentdueto1dayofseverelowbackpain.Thepainstartedafterliftingaturkeyfromthefreezerandworsenswithstanding,walking,andlyingonherback.Shehadnorecentfalls,lowerextremityweakness,orsensorylossinthelegs.Thepatienthasahistoryoftemporalarteritis,whichwasdiagnosedseveralmonthsagoandisbeingtreatedwithprednisone.Shedoesnotusetobaccooralcohol.Hertemperatureis36.7(96),bloodpressureis140/70mmHg,pulseis80/min,andrespirationsare16/min.Physicalexaminationrevealsmidlinetendernesswithpalpationorpercussionofthelumbarspine.Anklejerkreflexesareabsentbilaterally.Kneereflexesare2+inbothlegs.Babinskisreflexisdowngoingbilaterally.Musclestrengthis5/5inbothlegs.Bilateralstraightlegraiseto90degreesdoesnotincreasethepain.Whichofthefollowingisthemostlikelydiagnosisinthispatient?A.AnkylosingspondylitisB.EpiduralabscessC.HerniateddiskD.LumbosacralstrainE.MetastatictumorF.MultiplemyelomaG.SpinalstenosisH.Vertebralcompressionfracture,A6,Correctanswer:HThispatientspresentation-acutebackpainandpointtendernessafterlifting-suggestsavertebralcompressionfracture(VCF),whichoccursmostcommonlyintheelderly.RiskfactorsforVCFIncludetrauma(eg，fails),osteoporosis/osteomalacia,infection(eg,osteomyelitis),malignancywithbonemetastases,andmetabolicabnormalities(eg,hyperparathyroidism).PatientswithgradualonsetVCFcanbeasymptomatic.However,anacuteVCFcanpresentwithlowbackpainanddecreasedspinalmobilityaftersuddenbending,coughing,orlifting.Thepaintypicallyincreaseswithstanding,walking,orlyingontheback.Examinationcanshowtendernessattheaffectedlevel.RepeatedVCFcanleadtokyphosisandlossofstature.NontraumaticVCFismostcommonlyduetoosteoporosis,whichdecreasesbonedensityandincreasesfracturerisk.AlthoughmetastaticcancercancauseVCF,thispatientschronicsteroiduseandpostmenopausalstatusmakeadvancedosteoporosismorelikely(ChoiceE).Herabsentanklereflexescanbeduetonormalaging(mostcommonafterage70)andarenotclinicallysignificant.(ChoiceA)Ankylosingspondylitis(AS)typicallypresentswithpainandprogressivelylimitedbackmotion.ASismorecommoninyoungermen(age40).inaddition,thepainofASistypicallyworseinthemorningandImprovesthroughouttheday.(ChoiceB)Spinalepiduralabscessclassicallypresentswiththetriadoffever,severelocalizedbackpain,andneurologicdeficits(eg,motorweakness,paresthesias,bowel/bladderdysfunction).(ChoiceC)Herniateddiskusuallypresentswithradicularpainradiatingalongthethightobelowtheknee(ie,sciatica).Patientscanhaveapositivestraightlegraisetestduetothenerverootcompression.(ChoiceD)Lumbosacralstrainisusuallyseenafteraspecificeventoraction.Patientstypicallyhaveincreasedpainwithmovementanddecreasedpainwithrest.Inaddition,thepainistypicallyintheparaspinalareawithoutsignificanttendernesstopalpationofthevertebraasseeninthispatient.(ChoiceF)Multiplemyelomatypicallypresentswithanemia,bonepain(usuallybackorchest),fatigue,andweightloss.Laboratoryfindingsalsoshowelevatedcreatinineandhypercalcemia.However,thebackpaintendstobechronicratherthanacuteasseeninthispatient.(ChoiceG)Lumbarspinalstenosis(LSS)referstoanarrowingoftheintraspina!(central)canal,lateralrecess,orneuralforamen.Patientstypicallydeveloplowbackpainwithneurologicalsymptoms(eg，sensoryloss,weaknessinthelegs)thatisworsewithspinalextension(eg,walking,standing)butimprovedwithleaningforwardorlyingdown.Thispatientslackofsignificantneurologicalfindingsmakesthislesslikely.Educationalobjective:Avertebralcompressionfractureisacommoncomplicationofadvancedosteoporosis.Patientswithosteoporosiscandevelopacompressionfracturewithacutebackpainfollowingminima!trauma(eg,bending,coughing,lifting).Examinationmayshowpointtenderness,butneurologicalexaminationisusuallynormal.,Q7,A67-year-oldmancomestothephysiciandueto2daysofbackpain.Hewasmovingboxesinhisgaragewhenthepainstarted.Thepainisnotrelievedbylyingdownandincreasesinintensitywhenstrainingandcoughing.Hecannotsleepduringthenightduetopain,andhastakenseveralacetaminophentabletswithoutrelief.Hehasneverhadsuchpainbefore.Physicalexaminationshows2+symmetricalkneeandanklejerkreflexes.Straightlegraiseisnegative.Hehaspointtendernesstopalpationandpercussionalongthemidiineatthefourthlumbarvertebra.Whichofthefollowingisthemostlikelyunderlyingetiologyofthispatientscurrentcondition?A.ApophysealjointarthritisB.intervertebraldiskdegenerationC.LigamentoussprainD.LossofbonemineraldensityE.Nerverootdemyelinization,A7,Correctanswer:DThispatientspresentationacutebachpainandpointtendernessafterstrenuousactivitysuggestsavertebralcompressionfracture(VCF).PatientswithgradualonsetVCFcanbeasymptomatic.However,anacuteVCFcanpresentwithsuddenonsetoflowbackpainanddecreasedspinalmobilityafterbending,coughing,orlifting.Thepaintypicallyincreaseswithstanding,walking,orlyingontheback.Examinationcanshowten