![Page 1: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/1.jpg)
Recognition, Recognition, Investigation and Investigation and Treatment of Treatment of MyopathiesMyopathies
Hanni BoumaHanni Bouma
August 14, 2013August 14, 2013
![Page 2: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/2.jpg)
OverviewOverview
Statin-induced myopathyStatin-induced myopathy Idiopathic inflammatory Idiopathic inflammatory
myopathiesmyopathies– DermatomyositisDermatomyositis– PolymyositisPolymyositis– Inclusion body myositisInclusion body myositis
![Page 3: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/3.jpg)
Etiological Etiological Classification of Classification of MyopathiesMyopathies HereditaryHereditary Muscular DystrophiesMuscular Dystrophies
– Duchenne’s Duchenne’s MyotoniasMyotonias ChannelopathiesChannelopathies Congenital Congenital
MyopathiesMyopathies Metabolic MyopathiesMetabolic Myopathies
– Pompe’s diseasePompe’s disease Mitochondrial Mitochondrial
myopathiesmyopathies
AcquiredAcquired Inflammatory Inflammatory
myopathiesmyopathies– PM, DM, IBMPM, DM, IBM
EndocrineEndocrine– thyroidthyroid
Associated with Associated with other systemic other systemic illnessillness
Drug-induced and Drug-induced and toxic myopathiestoxic myopathies– EtOH, steroids, EtOH, steroids, statinsstatins
![Page 4: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/4.jpg)
Statin-induced Statin-induced MyopathyMyopathy 1.5-3% of statin users in RCTs and 10-
13% of participants enrolled in prospective clinical studies develop myalgias; rates of myositis lower (~0.1-0.5%) & dose-dependent
Mean duration of statin therapy before Mean duration of statin therapy before onset of Sx.: onset of Sx.: 6 months6 months
Mean duration of myalgias after Mean duration of myalgias after stopping statin therapy: stopping statin therapy: 2 months2 months
![Page 5: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/5.jpg)
![Page 6: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/6.jpg)
QuestionsQuestions
What if a patient develops a What if a patient develops a myopathy after several years of myopathy after several years of taking a statin?taking a statin?
Are some statins more likely to Are some statins more likely to cause muscle damage? Which cause muscle damage? Which ones?ones?
![Page 7: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/7.jpg)
![Page 8: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/8.jpg)
![Page 9: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/9.jpg)
ManagementManagement Significant muscle Sx.: discontinue Significant muscle Sx.: discontinue
statinstatin Asymptomatic but with CK>10x ULN: Asymptomatic but with CK>10x ULN:
discontinue statindiscontinue statin Rhabdo: no statins at any time due to Rhabdo: no statins at any time due to
risk of recurrence risk of recurrence If requires a statin but muscle toxicity If requires a statin but muscle toxicity
other than rhabdo: discontinue statinother than rhabdo: discontinue statin– Once Sx. have resolved and the CK has Once Sx. have resolved and the CK has
returned to baseline, can try returned to baseline, can try pravastatinpravastatin or or fluvastatinfluvastatin with careful monitoring with careful monitoring
![Page 10: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/10.jpg)
QuestionsQuestions
When are EMG or muscle biopsy When are EMG or muscle biopsy necessary in suspected statin necessary in suspected statin myopathy?myopathy?
Is Coenzyme Q10 helpful?Is Coenzyme Q10 helpful?
![Page 11: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/11.jpg)
Statin-associated Statin-associated necrotizing myopathynecrotizing myopathy
Myopathy which persists or Myopathy which persists or progresses after stopping statinprogresses after stopping statin
Linked to autoantibodies against Linked to autoantibodies against HMG-CoA reductaseHMG-CoA reductase
Distinct muscle biopsy findings:Distinct muscle biopsy findings:– macrophagocytic infiltrate engulfing macrophagocytic infiltrate engulfing
necrotic muscle fibers necrotic muscle fibers Responds to immune therapyResponds to immune therapy
![Page 12: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/12.jpg)
Statin-associated Statin-associated necrotizing myopathynecrotizing myopathy
![Page 13: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/13.jpg)
Onto the inflammatory Onto the inflammatory myopathies…myopathies…
![Page 14: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/14.jpg)
DM: ClinicalDM: Clinical
Slow, progressive, symmetric limb-girdle Slow, progressive, symmetric limb-girdle weaknessweakness
Activity-induced muscle painActivity-induced muscle pain Rash usually accompanies or precedes Rash usually accompanies or precedes
weakness (but not always)weakness (but not always) Associated features:Associated features:
– Adults: Myocarditis, ILD, vasculitis, other Adults: Myocarditis, ILD, vasculitis, other CTDs (RA, Scl, CREST)CTDs (RA, Scl, CREST)
– Children: Contractures, subQ calcinosis, Children: Contractures, subQ calcinosis, intestinal ulceration intestinal ulceration
MalignancyMalignancy: : adenocarcinomas, ovarian, adenocarcinomas, ovarian, breast, lung, lymphoma/leukemia breast, lung, lymphoma/leukemia
![Page 15: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/15.jpg)
![Page 16: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/16.jpg)
![Page 17: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/17.jpg)
DM: InvestigationsDM: Investigations
CK normal (20-30%) or increased up to CK normal (20-30%) or increased up to 50x 50x
ANA+ (24-60%) ANA+ (24-60%) Myositis specific antibodies: Myositis specific antibodies:
– Mi-2Mi-2 (15%) (15%) acute onset, nailfold ulcers & good response to acute onset, nailfold ulcers & good response to
therapytherapy– Anti-Jo-1Anti-Jo-1 (~20%) (~20%)
ILD, mechanic’s hands, arthritis, Raynaud’sILD, mechanic’s hands, arthritis, Raynaud’s EMGEMG Muscle biopsyMuscle biopsy MRIMRI
![Page 18: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/18.jpg)
Other Investigations: DMOther Investigations: DM Increased risk of Ca. within first 2-3 yrs of diagnosisIncreased risk of Ca. within first 2-3 yrs of diagnosis
– Treatment of malignancy sometimes improves muscle Treatment of malignancy sometimes improves muscle strengthstrength
– Malignancy workup in all patients: Malignancy workup in all patients: CT CAPCT CAP MammogramMammogram Breast & pelvic examsBreast & pelvic exams ColonoscopyColonoscopy And/OR PET scanAnd/OR PET scan
CXR, High res CT chest (ILD)CXR, High res CT chest (ILD) EKG (myocardial inv’t) or Echo if CHFEKG (myocardial inv’t) or Echo if CHF Swallowing assessment if dysphagiaSwallowing assessment if dysphagia
![Page 19: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/19.jpg)
PolymyositisPolymyositis
““Diagnosis of exclusion” Diagnosis of exclusion” – Often mistaken diagnosis of PM in cases of Often mistaken diagnosis of PM in cases of
DM w/o rash (yet) or IBM w/o inclusions on DM w/o rash (yet) or IBM w/o inclusions on biopsybiopsy
AdultsAdults with prox symmetric weakness: with prox symmetric weakness: limb girdle distribution + neck flexorslimb girdle distribution + neck flexors
Also ass’d with other autoimmune Also ass’d with other autoimmune disordersdisorders
Myocarditis, arthritis, Raynaud’s, ILDMyocarditis, arthritis, Raynaud’s, ILD
![Page 20: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/20.jpg)
IBMIBM Most common myopathy Most common myopathy
> 50 yo> 50 yo Insidious onset; Dx. Insidious onset; Dx.
usually several yrs after usually several yrs after onsetonset
Early dysphagiaEarly dysphagia Different pattern of Different pattern of
weakness: weakness: – Distal UE, Prox LEDistal UE, Prox LE– Early atrophy & Early atrophy &
weakness of WF, FF & weakness of WF, FF & quadsquads
– Hip girdle, TA musclesHip girdle, TA muscles
![Page 21: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/21.jpg)
EMG findingsEMG findings (all IM)(all IM)
Fibs, PSWs, Fibs, PSWs, CRDs at restCRDs at rest
Increased Increased insertional insertional activityactivity
![Page 22: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/22.jpg)
Why fibs?Why fibs?
1) Distal, healthy portion of muscle fibre gets separated from the part attached to the endplate2) Infarction of small intramuscular nerve twigs by surrounding interstitialinflammation
![Page 23: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/23.jpg)
Polyphasic, low Polyphasic, low amplitude, short amplitude, short duration MUPs with duration MUPs with voluntary activationvoluntary activation
Rapid recruitment Rapid recruitment of MUPs w/ full of MUPs w/ full interference interference pattern of low pattern of low amplitude on weak amplitude on weak effort effort
EMG findings
![Page 24: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/24.jpg)
Muscle biopsy Muscle biopsy findings…findings…
![Page 25: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/25.jpg)
Diagnosis?Diagnosis?
![Page 26: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/26.jpg)
PMPM
Endomysial mononuclear inflammatory cell infiltrate invading and surrounding non-necrotic muscle fibres
Mediated by CD8+ T-cells which attack Mediated by CD8+ T-cells which attack muscle fibresmuscle fibres
![Page 27: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/27.jpg)
![Page 28: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/28.jpg)
DMDM Humorally-mediated Humorally-mediated
microangiopathymicroangiopathy
1) Perifascicular necrosis/atrophy 2) Perivascular & perimysial inflammation: macrophages, B cells, CD4+ cells
![Page 29: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/29.jpg)
![Page 30: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/30.jpg)
IBMIBM
Similar to PM: CD8+ T cells & macrophagesSimilar to PM: CD8+ T cells & macrophages
Same features as PM + rimmed vacuoles + amyloid depositsModified Gomori trichrome stain
![Page 31: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/31.jpg)
Is it possible to have IBM without Is it possible to have IBM without inclusions on biopsy?inclusions on biopsy?
QuestionQuestion
![Page 32: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/32.jpg)
MRIMRI
•DM: inflammation mainly in anterior muscle compartments w/ preserved muscle mass•PM/IBM: fatty infiltration/muscle atrophy in all muscle groups
![Page 33: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/33.jpg)
Treatment of DM & PMTreatment of DM & PM Overall lack of “EBM” to guide Overall lack of “EBM” to guide
treatment; we don’t know:treatment; we don’t know:– Which second lineWhich second line therapies are most therapies are most
beneficialbeneficial– The doses required to see an effectThe doses required to see an effect– The best time to initiate 2The best time to initiate 2ndnd or 3 or 3rdrd line line
agentsagents– If some agents are more effective in If some agents are more effective in
certain types of myositiscertain types of myositis
![Page 34: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/34.jpg)
Treatment: Step 1Treatment: Step 1Initiate corticosteroidsInitiate corticosteroids
Treatment of choice in DM & PM:Treatment of choice in DM & PM:– Majority of patients will improve with Majority of patients will improve with
pred, but response may be pred, but response may be incompleteincomplete
Start prednisone at ~1 mg/kg/day up Start prednisone at ~1 mg/kg/day up to 100 mg qd to 100 mg qd
In severe weakness, treatment often In severe weakness, treatment often initiated w/ short course of IV initiated w/ short course of IV Solumedrol 1 g x 3 days prior to predSolumedrol 1 g x 3 days prior to pred
![Page 35: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/35.jpg)
Treatment: Step 1Treatment: Step 1Post-initiation of steroidsPost-initiation of steroids
Close clinical F/U q2-4 weeks initiallyClose clinical F/U q2-4 weeks initially Maintain dose until muscle strength Maintain dose until muscle strength
normalizes, improvement plateaus, normalizes, improvement plateaus, or CK normalizes (at least 4-6 wks at or CK normalizes (at least 4-6 wks at high dose)high dose)
Then Then slowslow taper: by taper: by 5 mg q2-3 5 mg q2-3 weeksweeks, below 20 mg by 2.5 q2wks, below 20 mg by 2.5 q2wks
![Page 36: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/36.jpg)
Treatment: Step 1Treatment: Step 1Side effect considerations for steroidsSide effect considerations for steroids
Monitor fasting glucose, K+ levelsMonitor fasting glucose, K+ levels Septra for PCP prophylaxisSeptra for PCP prophylaxis
– If concurrent ILD or pred + other If concurrent ILD or pred + other immunosuppressantimmunosuppressant
Bone density scan at baseline & qyearlyBone density scan at baseline & qyearly Calcium 1 g/day + Vit D 1000 IU/dayCalcium 1 g/day + Vit D 1000 IU/day Bisphosphonate used if postmenopausalBisphosphonate used if postmenopausal Record BP at each visit (accelerated HTN & Record BP at each visit (accelerated HTN &
renal failure is a risk) renal failure is a risk) – Coexistence of scleroderma & other MCTDsCoexistence of scleroderma & other MCTDs
Periodic eye exams for glaucoma & cataractsPeriodic eye exams for glaucoma & cataracts
![Page 37: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/37.jpg)
QuestionQuestion
What should I do if there is no What should I do if there is no response after an adequate trial response after an adequate trial of high dose prednisone?of high dose prednisone?
![Page 38: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/38.jpg)
QuestionQuestion
How can I tell if the patient is How can I tell if the patient is weaker because of refractory weaker because of refractory disease or because of chronic disease or because of chronic steroid use?steroid use?
![Page 39: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/39.jpg)
Treatment: Step 2Treatment: Step 2Add immunosuppressantAdd immunosuppressant
Indications:Indications:– Moderate or severe weaknessModerate or severe weakness– Other organ system inv’t (ILD, Other organ system inv’t (ILD,
myocarditis)myocarditis)– Increased risk of steroid complications Increased risk of steroid complications
(diabetic, OP, postmenopausal women)(diabetic, OP, postmenopausal women)– Failure to significantly improve after 2-4 Failure to significantly improve after 2-4
months of steroidsmonths of steroids– Any pt expected to need steroids for 10-12 Any pt expected to need steroids for 10-12
mos or moremos or more
![Page 40: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/40.jpg)
Treatment: Step 2Treatment: Step 2ImmunosuppressionImmunosuppression
Options:Options:– AzathioprineAzathioprine – MethotrexateMethotrexate– IVIG IVIG – CellceptCellcept– CyclophosphamidCyclophosphamid
ee
Generally used Generally used as 3as 3rdrd line, if line, if refractory to refractory to other Rx.:other Rx.:– Rituximab Rituximab – PLEX PLEX – CiclosporineCiclosporine– TacrolimusTacrolimus
![Page 41: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/41.jpg)
AzathioprineAzathioprine
Effective in DM/PM (retrospective Effective in DM/PM (retrospective studies), but takes 6-18 mos to studies), but takes 6-18 mos to workwork
Prior to starting, can screen for Prior to starting, can screen for TPMT deficiency (BM toxicity in TPMT deficiency (BM toxicity in homozygotes) or just monitor CBChomozygotes) or just monitor CBC
Begin at 50 mg/d, increase by 50 Begin at 50 mg/d, increase by 50 mg q2wks up to 2-3 mg/kg/dmg q2wks up to 2-3 mg/kg/d
![Page 42: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/42.jpg)
AzathioprineAzathioprineMonitoring & SEsMonitoring & SEs
Major SEs:Major SEs: 12% develop systemic 12% develop systemic rxn (fever, abdo pain, N/V) within rxn (fever, abdo pain, N/V) within first few wks requiring first few wks requiring discontinuation of drug; BM & liver discontinuation of drug; BM & liver toxicity, pancreatitis, teratogenicity, toxicity, pancreatitis, teratogenicity, oncogenicity, infectiononcogenicity, infection
LeukopeniaLeukopenia Monitor CBC, LFTs closelyMonitor CBC, LFTs closely
![Page 43: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/43.jpg)
MethotrexateMethotrexate
Most DM & PM respond to MTX Most DM & PM respond to MTX (retrospective studies only)(retrospective studies only)
Begin at 7.5 mg/wk po, increase Begin at 7.5 mg/wk po, increase gradually by 2.5 mg each week gradually by 2.5 mg each week up to 25 mg/wkup to 25 mg/wk
If no improvement after 1 month If no improvement after 1 month on 25 mg, switch to weekly subQ on 25 mg, switch to weekly subQ & increase dose by 5 mg qwk up & increase dose by 5 mg qwk up to 60 mg/wkto 60 mg/wk
![Page 44: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/44.jpg)
MethotrexateMethotrexateMonitoring & SEsMonitoring & SEs
Major SEs:Major SEs: alopecia, stomatitis, alopecia, stomatitis, pulmonary fibrosis, pulmonary fibrosis, teratogenicity, teratogenicity, oncogenicity, infection; renal, liver oncogenicity, infection; renal, liver & BM toxicity& BM toxicity
Avoid MTX in pts with ILD or anti-Avoid MTX in pts with ILD or anti-Jo-1+Jo-1+
Avoid MTX in heavy drinkersAvoid MTX in heavy drinkers Treat all pts with folate 5 mg qwkTreat all pts with folate 5 mg qwk
![Page 45: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/45.jpg)
IVIGIVIG
One prospective, double-blind, One prospective, double-blind, placebo-controlled study in 15 pts placebo-controlled study in 15 pts w/ DM showed significant w/ DM showed significant improvementimprovement
Little RCTLittle RCT evidence of benefit as evidence of benefit as monotherapy but plenty of monotherapy but plenty of anecdotal evidence that IVIG is anecdotal evidence that IVIG is effective, even aloneeffective, even alone
![Page 46: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/46.jpg)
CyclophosphamideCyclophosphamide
Used often if ILDUsed often if ILD SEs: infections, secondary SEs: infections, secondary
malignancies, hemorrhagic malignancies, hemorrhagic cystitis, sterilization, BM toxicity, cystitis, sterilization, BM toxicity, GI upset, alopeciaGI upset, alopecia– Usually given pulsed; higher risk of Usually given pulsed; higher risk of
cystitis pocystitis po
![Page 47: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/47.jpg)
Treatment: Step 3Treatment: Step 3If refractory to other modalities…If refractory to other modalities…
RituximabRituximab -> monoclonal Ab -> monoclonal Ab against CD20, depletes B cellsagainst CD20, depletes B cells– Warnings re: PML risk…Warnings re: PML risk…
![Page 48: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/48.jpg)
SISide Effects & Side Effects & MonitoringMonitoring
![Page 49: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/49.jpg)
Non-medical therapiesNon-medical therapies
PT & OTPT & OT Dietician consult if on steroidsDietician consult if on steroids Aerobic exercise programsAerobic exercise programs
– Prevents contracturesPrevents contractures– May help w/ steroid SEs (weight May help w/ steroid SEs (weight
gain, OP, type 2 fibre atrophy)gain, OP, type 2 fibre atrophy) Speech therapySpeech therapy
– Esp if concomitant dysphagiaEsp if concomitant dysphagia
![Page 50: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/50.jpg)
QuestionQuestion
What is the value of monitoring What is the value of monitoring serum CK levels in the treatment serum CK levels in the treatment of DM & PM?of DM & PM?
![Page 51: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/51.jpg)
QuestionQuestion
How does the treatment of IBM How does the treatment of IBM differ from that of PM & DM?differ from that of PM & DM?
![Page 52: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/52.jpg)
IBMIBM
Glucocorticoids have limited roleGlucocorticoids have limited role– In largest published series, muscle In largest published series, muscle
strength continued to deteriorate in strength continued to deteriorate in all of 25 pred-treated patients all of 25 pred-treated patients followed for at least 2 yrsfollowed for at least 2 yrs
– CK levels often normalize, but this CK levels often normalize, but this doesn’t correlate with clinical benefitdoesn’t correlate with clinical benefit
![Page 53: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/53.jpg)
IBM: suggested IBM: suggested approachapproach If ++inflammation seen on Bx., If ++inflammation seen on Bx.,
consider trial of steroids +/- consider trial of steroids +/- Imuran (3 mos) early in diseaseImuran (3 mos) early in disease
Discontinue all therapy if Discontinue all therapy if continued decline in strengthcontinued decline in strength
For most patients, For most patients, no treatmentno treatment
![Page 54: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/54.jpg)
Overview of the IMOverview of the IM
![Page 55: Recognition, Investigation and Treatment of Myopathies](https://reader031.vdocuments.site/reader031/viewer/2022020721/56814462550346895db0f703/html5/thumbnails/55.jpg)
ReferencesReferences
Dr. Erin O’FerrallDr. Erin O’Ferrall Amato & Barohn. Evaluation and treatment Amato & Barohn. Evaluation and treatment
of inflammatory myopathies. of inflammatory myopathies. Journal of Journal of Neurology, Neurosurgery & Psychiatry Neurology, Neurosurgery & Psychiatry 2009; 2009; 80: 1060-1068.80: 1060-1068.
Sathasivam & Lecky. Statin induced Sathasivam & Lecky. Statin induced myopathy. myopathy. BMJ BMJ 2008; 337: a2286.
Preston & Shapiro. Electromyography and neuromuscular disorders: Clinical-electrophysiologic correlates. 2nd ed. 2005.
Up to Date: Statin myopathy & Up to Date: Statin myopathy &