l muscle weakness in the setting

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1、A41Year-oldwomanwithproximalmuscleweaknessinthesettingof“rheumatoidarthritis”andanabnormalchestx-rayRheumatologyRoundsDecember15,2006LisaChristopher-Stine,MD,MPHAssistantProfessorofMedicineCo-Director,TheJohnsHopkinsMyositisCenterNoRelevantFinancialRelationshipswithCommercialInterestsDis

2、closuresLisaChristopher-Stine,MD,MPHObjectivesToreviewthehistorical,physicalexamination,andpathologicalfindingsofapatientwhopresentswithupperextremityproximalweaknessToexplorethedifferentialdiagnosisofthiscaseTogaininsightintothemeaningoftheassociatedautoantibodiesnotedToreviewthecurrent

3、literaturerelatedtothisclinicalentityToexploretherapeutictreatmentoptionsCasePresentationReferral:Proximalmyopathyinthesettingofadiagnosisofrheumatoidarthritis41year-oldwomanw/complicatedmedicalhistory1984:unrestrainedpassengerinMVA;legweakness;?UpperarmsorenessCirca1988:WheezingandSOB,a

4、ttributedtosmoking1988:c/oarmweakness,soreness;neuroevalHistory1988:CXRshowshilaradenopathynotedonpre-employmentphysicalexamination1989:Kneeandankleswelling;rheumevalrevealshighRFDxwithRAandRxw/naproxen1989:Dxwith“asthma”;Rxnebs,inhalers1988-1994:Notes“slowandsteadydeclineofmusclestreng

5、th”intheshouldergirdle1989:Leftquadmusclebxnondiagnostic1994:LeftnecklymphnodebiopsyHistory1994-2006Rapiddeteriorationinstrength;arthritisandarthralgias;enlarginggoiter;jawweakness1995:Secondmusclebxofdeltoid2000:?Rightfootdropnoted2005:ConsultationinJHUArthritisCenter2006:Referralformyo

6、pathyevaluationFamilyHistorySister:occularsarcoid,facialpalsy,arthritisMaternalcousinwithsarcoidMaternalgrandfather:RAMedicationsPrednisone(1994-present)MTXandCSA–shortdurationEtanercept2000-2002;restarted2wksagoCyclophosphamide2002-2004Mycophenolatemofetil2g/day12/04&12/05:IVIGx1courseP

7、hysicalExamination:GeneralHEENT:Canopenmouthonly2fingerbreadthsindiameterNeck:EnlargedgoiterwithpalpablenodulesHeart:RRR,nom/r/gLungs:CTAb/lwithoutwheeze,rhonchi,cracklesSkin:MinimalscalyrashonMCPsbilaterallyPhysicalexam-musculoskeletalWhenstands,swingsarmsfromsidetosidei