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