Myosi&s:Ge#nginSyncwithyour
HealthcareteamNamitaGoyal,MDAssociateProfessorofNeurologyAssociateDirector,NeuromuscularCenterUCIrvine
TheMyosi&sAssocia&onKentucky
September7,2018
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OverviewofCareTeam:Checklist• Rheumatologist/Neurologist
• PrimaryCarePhysician(PCP)
• Pulmonologist/Respiratorytherapist
• Cardiologist
• Speech/Swallowtherapist
• Physicaltherapist/Occupa>onaltherapist
• Die>cian/Nutri>onist
• SocialWorker
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Neurologist/Rheumatologist:Ini>alEvalua>on:
• History/exam
• Diagnos>cevalua>on• Bloodtests:CKlevels• An>bodies• EMG• Musclebiopsy• MuscleMRI
• CancerScreeninginDermatomyosi>s• Espif>40yrsold• upto3-5yearsfromsymptomonset
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Myosi&sAn&bodies
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Myosi&sSpecificAn&bodies:Dermatomyosi&sAutoan&bodies
Mi-2
TIF1γ
NXP-2
MDA5
SAE
classic cutaneous manifestations, respond well to immunotherapy, low risk of cancer
high risk of malignancy, classic skin manifestations, “diffuse photoerythema, dusky red face”
subcutaneous calcifications, (in up to 25% of juvenile DM, but also adults), increased risk of malignancy
rapidly progressive ILD, (20-30% of Asian DM patients, less freq in Caucasians), skin ulcerations, tender palmar papules, oral ulcers, minimal muscle involvement (clinically amyopathic)
least frequent, <10%, dysphagia, skin disease, good prognosis
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Myosi&sSpecificAn&bodies(MSA):An&synthetaseAutoan&bodies
An>synthetasesyndrome:• Myosi>s,Inters>>allungdisease(ILD),inflammatory
arthri>s,fever,Raynaud’sphenomenon,mechanic’shands• Somehaveprominentskinrash
8Abs,mostcommonMSA,iden>fiedin35-40%ofmyosi>s
Jo-1 PL-12 PL-7 KS OJ EJ Zo Ha
most common, (in 15-20% of myositis) 90% have myositis, 50-75% ILD
50% have myositis, 90% ILD
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Myosi&sAssociatedAn&bodies(MAA):
Nonspecific,inmyosi>s&connec>ve>ssuediseases
• Ro52/TRIM21,PMScl,ribonucleoproteincomplex(RNP;U1RNP,U2RNP,U4/U6,RNP,U5RNP),Ku
Ro52
PMScl
Ku
most common, associated with ILD
seen in PM, systemic sclerosis (SSc), & PM/SSc overlap syndrome, associated with lung and esophageal involvement
in Overlap syndrome, frequent joint involvement, Raynaud’s and ILD
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MSA:Immune-MediatedNecro&zingAbsProminentmyofibernecrosiswithminimalinflamma>on
CK>1,000-10,000
An>-SRP
• Rapidly progressive onset • Very high CK levels • Dysphagia • Neck extensors • Severe weakness • May not respond well to
immunotherapy
• First described in context of statin exposure (2010)
• Also in statin-naïve • (Not found in self-limited statin
intolerance) • May require aggressive
immunotherapy or IVIg
An>-HMGCR
Rare,(<5%ofallmyosi>s)
(6-9%ofallmyosi>s)
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Neurologist/Rheumatologist:(follow-up)Subsequentvisits:
• History/exam
• Medica>onmanagement
• Responsetoimmunotherapy?• Adjustmedica>ons• Lackofresponse• Alterna>vetherapies/clinicaltrials?• Wrongdiagnosis?
• Adverseeffectsofmedica>ons• Checkbloodcounts,liver,kidney
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PrimaryCarePhysician:• Ageappropriatehealthscreening
• MonitorBloodsugars,Bloodpressure–ifonsteroids
• Bonedensitytest(VitaminDwithCalcium)
• CheckinwithPCPifnotfeelingwell,maynotmountfeverifimmunosuppressed
• Vaccina>ons
• HelpwithMood/An>depressant?
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Pulmonologist/Respiratorytherapist:• IfInters>>allungdisease(ILD)
• CTChest(toscreenandmonitorprogression)• Pulmonaryfunc>ontests
• NoninvasiveVen>la>on(BiPAP)• Quitebeneficialinrespiratoryinsufficiency• Difficultytolera>ng?
• Workwithrespiratorytherapisttoadjustmask/sepngs
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Cardiologist:
• Cardiomyopathy/cardiacarrhythmias
• Rare,butpoten>alcomplica>onofDM,An>-synthetasesyndromes
• Closemonitoring
• Medicalmanagement
• Echo/EKG
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Speech/SwallowTherapist:• Difficultyswallowing/dysphagia
• Canbeleadingcauseofmorbidity/mortality
• Upto1/3ofmyosi>spa>ents(espIBM)
• BariumSwallowingevalua>on• Candetectsubclinicalinvolvement• Evaluatesseverity
• Modifieddiet
• Ifsevere,G-tube• Reducesriskofaspira>onpneumonia
• Communica>ondevices
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Physical/Occupa&onaltherapists:• Riskoffalls
• Adap>veequipmentneeds• Footbrace(AFO),Cane,Walker,Scooter,Wheelchair• Showerchair• Hospitalbed
• Homesafetyevalua>on–railings,grabbars
• Roleofexercise• Nopain,nogain-NOTthemoqo!• Sta>onarycycling,pooltherapy(ifsafetogetin)• Don’texercisetothepointofpainorsignificantfa>gue
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Die&cian/Nutri&onist:• Well-balanceddiet
• Maintainingweight
• Ifonsteroids,weightgainisarecognizedconcern• Askforaconsultwithnutri>onist
• Ifdifficultyswallowing,weightlossisaconcern
• Withmuscleatrophy,weightlossmayoccur
• G-tube(whenseveredysphagia)–reducesriskofaspira>on• Maintaincaloriesandweight• Helpfulforpills• Hydra>on
• Cons>pa>on(common),espinimpairedmobility
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SocialWorker:
• Caregivingresources
• Homehealthservices
• Supportgroups
• Psychologists/psychotherapists
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OtherSpecialists:
• Dermatologist–dermatomyosi>spa>entswithsevereskininvolvement
• Gastroenterologist(GI)–ifPCPhasdifficultymanagingandmoresevereGIissues
• Psychiatrist
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Goal: Multidisciplinary Team Approach
Pa>ent
Neurologist/Rheumat
Respiratorytherapist
Physical/Occupational
Therapist
Swallow Therapist
SpeechTherapistSocialWorker
Die&cian
Medicalassistant/Nurse
Otherspecialists
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