RheumatologyMain61-70Q1A70-year-oldmancomestothephysicianforpainandstiffnessofhisneck,shoulders,andhipsforthelast3months.Hisstiffnessisworseinthemorningandlasts1-2hours.Healsocomplainsofgeneralmalaiseandarecentweightlossof3.17kg(7lb).Thepatienthasnoheadache,scalptenderness,visualsymptoms,orjawclaudication.Examinationshowsnoovertsynovitisatthejoints,withnormalpassiverangeofmotion.Thearteriesofthescalp,neck,andextremitiesarenormalonpalpationwithouttenderness.Laboratoryresultsareasfollows:Whichofthefollowingisthemostappropriatenextstepinmanagementofthispatient?A.MeasureantinuclearantibodiesandrheumatoidfactorlevelsB.Recommendlow-impactaerobicexerciseC.TemporalarterybiopsyD.Treatmentwithlow-doseprednisoneE.Treatmentwithnonsteroidalanti-inflammatorydrugA1Correctanswer:DInPMR;thephysicalexaminationisfrequentlyunremarkablewithpatientshavingnofocaltendernessorpainwithactiveorpassiverangeofmotion.Signsofinflammationinthejointsareabsent.Whenaskedtoidentifythelocationoftheirpain:patientstypicallyindicatethesofttissuesandnotthejoints.Low-doseglucocorticoidsarethetreatmentofchoiceforPMR(eg,prednisone10-20mgdaily).Rapidandthoroughreliefofsymptomsisexpected,andfailuretoimproverapidlyonprednisoneshouldcai!thediagnosisintoquestion.PMRisfrequentlyassociatedwithgiantcellarteritis(GCA),alsoknownastemporalarteritis.SymptomsofGCAincludeheadache,jawclaudication,visionloss,ortendernessoverthetemporalartery.IfGCAissuspected,patientsshouldbeconsideredforanexpeditedtemporalarterybiopsy(ChoiceC)andreceivesignificantlyhigherdosesofglucocorticoids(eg;prednisone40-60mgorhigherdaily).(ChoiceA)Antinuclearantibodyandrheumatoidfactorarescreeningtestsforsystemiclupuserythematosusandrheumatoidarthritis,respectively,andarefrequentlypositiveinanumberofotherinflammatoryandautoimmuneconditions.Thesetestshavepoorspecificityintheabsenceofcharacteristicsymptoms(eg,malarrash,metacarpophalangealsynovitis)andwouldbeunlikelytochangemanagementinthispatient.(ChoiceB)Fibromyalgia(FM)mostcommonlypresentsinyoungtomiddle-agedwomenwithwidespreadpain,fatigue,andcognitive/mooddisturbances.Patientshavepointmuscletendernessinareassuchasthemidtrapezius,lateralepicondyle,costochondraljunctioninthechest,andgreatertrochanter.RegularaerobicexerciseandgoodsleephygienearerecommendedforFM.However,constitutionalsymptoms(eg,weightloss)orelevatederythrocytesedimentationratearenotpresentinFM.(ChoiceE)Nonsteroidalanti-inflammatorydrugsareoccasionallyusefulinPMRformildbreakthroughdiscomfortorminorrelapseofsymptomswhiletaperingglucocorticoids.However,theyaremuchlesseffectivethanglucocorticoidsforinitialmanagementandarenotrecommended.Educationalobjective:Polymyalgiarheumatica(PMR)affectspatientsage50andischaracterizedbypainandstiffnessintheneck,shoulders,andpelvicgirdle,alongwithanelevatederythrocytesedimentationrate.ThetreatmentofchoiceforuncomplicatedPMRislow-doseprednisone,whichresultsinrapidreliefofsymptoms.Q2A45-year-oldwomancomestotheofficeduetoprogressiveweaknessforthepastseveralmonths.Thepatienthashaddifficultyliftingherfosterchildrenandgettinginandoutofhercar.Shealsoreportsdifficultyswallowingandhashadseveralepisodesofchokingwhiledrinkingwater.Shehasnomedicalproblems,butthereisahistoryofbreastcancerinhermother.Thepatientdoesnotusetobacco,alcohol,orillicitdrugs.Vitalsignsarewithinnormallimits,andshehasnormalsensationanddeeptendonreflexes.Strengthis4/5inthedeltoidandquadricepsmusclegroups,andthereisnolimitationinrangeofmotion.Physicalexaminationisotherwiseunremarkable.Erythrocytesedimentationrateis50mm/hr,andserumcreatinekinaseiselevated.TSHisnormal.Whichofthefollowingisthebestinitialtreatmentforthispatient?A.AmitriptylineB.BetainterferonC.CyclobenzaprineD.PrednisoneE.PyridostigmineF.RadiationtherapyG.RiluzoleA2Correctanswer:DThispatienthasfeaturesofpolymyositis,includingchronicproximalmuscleweakness,otherwisenormalneurologicfindings,andanelevatedcreatinekinase.Peakincidenceoccursatage40-50,andwomenaremorecommonlyaffectedthanmen.Proximalmuscleweaknesstypicallymanifestsasdifficultyclimbingstairs,gettingintooroutofalowchairorcar,orworkingwiththearmsoverhead(eg,combinghair).Involvementofupperesophagealmusculaturecancausedysphagiawithregurgitationandaspiration.Inflammatorymarkers(eg,erythrocytesedimentationrate)maybeelevated,andmostpatientshavedetectableautoantibodies(eg,antinuclearantibody,anti-Jo-1).Definitivediagnosisisestablishedonmusclebiopsy.Polymyositisresemblesdermatomyositisbutwithoutassociatedskinmanifestations.Initialremissioncanbeinducedwithglucocorticoids(eg,prednisone),andmostpatientsalsoreceiveaglucocorticoid-sparingagent(eg,methotrexate,azathioprine)tominimizethelong-termadverseeffectsoftreatment.Becausepolymyositisfrequentlyoccursasaparaneoplasticsyndrome,patientsshouldalsoreceiveage-appropriatecancerscreening.(ChoicesA)Amitriptylineisatricyclicantidepressantusedinanumberofchronicpaindisorders(eg,fibromyalgia).Fibromyalgiacancausefatigueandreluctancetoexercise,buttruemuscleweaknessisabsent.(ChoicesB,E,andG)Betainterferonissometimesusedinthetreatmentofmultiples