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RheumatologyMain91-100Q1A34-year-oldwomancomestotheclinicwithafacialrash,jointpainsrandoralulcers.Shehasnoassociatedfeverorothersystemicsymptomsandotherwisefeelswell.Medicalhistoryisunremarkable,andthepatient'sonlyconcurrentmedicationisanoralcontraceptive.Shedoesnotsmokeandconsumesalcoholonlyonsocialoccasions.Antinuclearandanti-double-strandedDHAantibodytitersareelevated.Creatinineandcomplementlevelsarenormal.Thepatientisdiagnosedwithsystemiclupuserythematosus,andtherapywithhydroxychloroquineisInitiated.Whichofthefollowingevaluationsshouldbedoneperiodicallywhilethepatientistakingthismedication?A.AudiometryB.EchocardiogramC.EyeexaminationD.LiverfunctionpanelE.PulmonaryfunctiontestsF.Thyroid-stimulatinghormoneA1Correctanswer: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.Q2A49-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-topoisomeraseIantibodiesA2Correctanswer: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-cycliccitruliinatedpeptideantibodiesareamorespec