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Overview of Inflammatory Myositis Types

This document discusses inflammatory myositis, which includes three main types: polymyositis, dermatomyositis, and inclusion body myositis. The key clinical features are progressive, symmetric weakness of proximal muscles. Extra-muscular manifestations can include systemic symptoms, joint contractures, dysphagia, and cardiac or pulmonary involvement. Diagnosis involves clinical suspicion, elevated muscle enzymes, electromyography, and muscle biopsy showing primary inflammation. Treatment primarily consists of glucocorticoids with other immunosuppressants if no improvement after 3 months.

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177 views15 pages

Overview of Inflammatory Myositis Types

This document discusses inflammatory myositis, which includes three main types: polymyositis, dermatomyositis, and inclusion body myositis. The key clinical features are progressive, symmetric weakness of proximal muscles. Extra-muscular manifestations can include systemic symptoms, joint contractures, dysphagia, and cardiac or pulmonary involvement. Diagnosis involves clinical suspicion, elevated muscle enzymes, electromyography, and muscle biopsy showing primary inflammation. Treatment primarily consists of glucocorticoids with other immunosuppressants if no improvement after 3 months.

Uploaded by

drrajak99
Copyright
© Attribution Non-Commercial (BY-NC)
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as PPT, PDF, TXT or read online on Scribd

Inflammatory myositis

-Prof. Rukmani M.D.


Definition
 A group of acquired inflammatory myopathies
affecting skeletal muscles…
Three groups
 Polymyositis
 dermatomyositis
 Inclusion Body myositis [IBM]
Common Clinical Features
 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


ExtraMuscular Manifestations

 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
Overlap Syndromes
 Inflammatory myopathies associated with
connective tissue disorders

 DM most commonly asssociated with systemic


sclerosis or MCD

 RA, SLE, Sjogrens are very rarely associated

 Overlap syndromes may have ANA, anti PM/Scl


antibodies in addiation to Jo 1 antibodies
Feature Polymyositis Dermatomyositis IBM
Age at onset < 18 years Adult and childhood >50 years

Familial Assoc No No No

Extra Yes Yes Yes


Musc.manif.

Asssoc. Conn. Yes Scleroderma and MCD Yes


Tissue Dis

Malignancy No Yes No

Viruses
Yes Unproven Yes

Drugs Yes Rarely No

Parasites and yes yes No


bacteria
Skin Manifestation-
Dermatomyositis
 Heliotrope rash
 Gottron’s sign and mechanic hands
 Shawl sign
 Nail &Cuticle changes
Diagnosis
 Clinical suspiscion

 Creatinine Kinase

 Needle EMG

 Muscle Biopsy
Criteria Polymyos. Dermatomyo. IBM
Myopathic Yes Yes Yes, slow
muscle onset, distal
weakness
Myopathic Myopathic Myopathic
EMG findings with mixed
potentials
Muscle enzymes Elevated [upto 50 Elevated upto
Elevated [upto 50 10 fold
fold] fold]
Primary
Muscle Biopsy Primary Perifascicular, inflammation
inflammation with perimysial or with
CD8/MHC1 perivascular CD8/MHC1
complex and no infilatrates complex,
vacoules vacoulated
fibres with
βamyloid
Absent present Absent
Rash /Calcinosis
Treatment
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
 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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