inflammatory myositis
TRANSCRIPT
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Inflammatory myositis
-Prof. Rukmani M.D.
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Definition
A group of acquired inflammatory myopathies
affecting skeletal muscles«
Three groups Polymyositis
dermatomyositis
Inclusion Body myositis [IBM]
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Common Clinical Featur es
Progressive and sym. prox. muscle weakness; expt IBM [distal]
Fine motor movements relatively spared; except IBM ±inv. early
Ocular muscles spared in even untreated and advanced cases
Pharyngeal and neck muscles commonly involved
Respiratory muscles affected in advanced stages
Sensations preserved
Tendon reflexes preserves until late stages
Weakness can be accompanied by muscle wasting
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ExtraMuscular Manif estations
Systemic symptoms ± fever, malaise, wt loss,
arthralgias, raynaud¶s
Joint contractures
Dysphagia and GI symptoms
Catrdiac ± conduction defects, cardiomyopathies
Pulmonary - Pulmonary HT and ILD
Subcutaneous Calcifications
Arthralgias
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Overlap Syndromes
Inflammatory myopathies associated withconnective tissue disorders
DM most commonly asssociated with systemicsclerosis or MCD
R A, SLE, Sjogrens are very rarely associated
Overlap syndromes may have ANA, anti PM/Sclantibodies in addiation to Jo 1 antibodies
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Featur e Polymyositis Dermatomyositis IBM
Age at onset
Familial Assoc
Extra
Musc.manif.
Asssoc. Conn.
Tissue Dis
Malignancy
Viruses
Drugs
Parasites andbacteria
< 18 years
No
Yes
Yes
No
Yes
Yes
yes
Adult and childhood
No
Yes
Scleroderma and MCD
Yes
Unproven
Rarely
yes
>50 years
No
Yes
Yes
No
Yes
No
No
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Skin Manif estation-
Dermatomyositis
Heliotrope rash
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Gottron¶s sign and mechanic hands
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Shawl sign
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Nail &Cuticle changes
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Diagnosis
Clinical suspiscion
Creatinine Kinase
Needle EMG
Muscle Biopsy
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Criteria Polymyos. Dermatomyo. IBM
Myopathic
muscle
weakness
EMG findings
Muscle enzymes
Muscle Biopsy
Rash /Calcinosis
Yes
Myopathic
Elevated [upto 50fold]
Primary
inflammation with
CD8/MHC1complex and no
vacoules
Absent
Yes
Myopathic
Elevated [upto 50fold]
Perifascicular,
perimysial or
perivascular infilatrates
present
Yes, slow
onset, distal
Myopathic
with mixed
potentials
Elevated upto
10 fold
Primary
inflammation
with
CD8/MHC1 complex,
vacoulated
fibr es with
amyloid
Absent
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Tr eatment
1. Glucocorticoids Initial treatment of choice
Started at 1 mg/kg/day-continued for 3-4 weeks
Then tapered to alternate day regimen and then
maintained at lowest possible dose
Other immunosuppressives started if there is no
improvement by 3 months
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Other Drugs
Azothioprine- 3mg/kg/day
Methotrexate ± 7.5 mg/week Mycophenolate mofetil 2.5 mg/day
Anti CD20 antibodies ± Rituximab
Cyclosporine
Cyclophosphamide
Immunomodulators ± IV Ig 2g/kg divided doses
over 5 days
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