SlideShare a Scribd company logo
1 of 77
InflammatoryMyositis
Dr. MohammadRehan
Contents
• Introduction
• History
• Classification
• Clinical Features
• Associated Conditions
• Pathogenesis
• Autoantibodies
• Antisynthetase syndrome
• Diagnosis
• Treatment
• Prognosis
Introduction
• Inflammatory Myopathies are sporadic disorders representing the
largest group of acquired and potentially treatable causes of skeletal
muscle weakness.
• Annual incidence ~ 1 in 100,000
• Women > Men , Polymyositis (PM) and Dermatomyositis (DM)
• Men > Women , Inclusion Body Myositis (IBM)
Age at onset for Inflammatory myopathies is different.
• PM > 18 years.
• DM affects both Juvenile population and Adulthood.
• IBM affects persons aged >50 years.
History
• In 1888 the first American biopsy documented polymyositis in ruling
out Trichinella.
• Heliotrope rash was first described in 1875 in France.
• In 1930 Gottron’s reported skin lesions .
• In 1967 the pathology of IBM was described.
Classification
• There is no internationally accepted classification system for
Inflammatory myopathies.
• For discussion purpose IM’s can be classified as :-
Polymyositis.
Dermatomyositis and Juvenile Dermatomyositis.
Inclusion Body Myositis.
Autoimmune Necrotising Myopathies.
Myositis associated with Collagen Vascular disorder.
Myositis associated with malignancy and Juvenile Dermatomyositis.
ClinicalFeatures
Polymyositis
• A rare, subacute inflammatory myopathy affecting adults and rarely
children.
• Progressive, symmetric proximal muscle weakness with sparing of
ocular and facial muscles.
• Pharyngeal and Neck Flexors, also involved causing dysphagia and
Head drop.
• Associated with Myalgia and Muscle tenderness in some cases
• In Advanced and Acute cases Respiratory muscles may be affected.
• Severe weakness if untreated leads to muscle wasting.
• Sensations and DTR’s remain intact.
ClinicalFeatures
Dermatomyositis
Subacute inflammatory myopathy with muscle weakness similar to
polymyositis with distinctive rash more often preceding the muscle
weakness.
DM:DermatologicManifestations
Heliotrope rash
• Periorbital violaceous erythema
with or without edema of eyelids
and periorbital tissue.
• Highly characteristic of DM
DM:DermatologicManifestations
Gottron’s Papules
• Violaceous flat
topped papules and
plaques over dorsal
aspect of
interphalangeal and
MCP joints
• Pathognomonic of DM,
seen in > 80%
patients with DM
DM:DermatologicManifestations
V- sign
• Macular violaceous
erythema over V-shaped
region of the neck and
upper chest.
• Sometimes rash is
pruritic.
DM:DermatologicManifestations
Shawl Sign
• Macular violaceous
erythema over
Nape,back and
shoulders.
• May show
photosensitivity
DM:DermatologicManifestations
Mechanic’s Hand
• Also considered
characteristic.
• hyperkeratosis,
scaling, and horizontal
fissuring of the palms
and fingers bilaterally.
• Can be a manifestation
of the Antisynthetase
syndrome.
DM:DermatologicManifestations
Nailfold Talengectasia
• Occur in 30 to 60 %
early in disease
AmyopathicandHypo-myopathic
Dermatomyositis
• Amyopathic DM : present with typical dermatological manifestations
of DM, with normal muscle strength, enzyme levels and EMG findings.
• Hypo-myopathic DM : Dermatological manifestations of DM with no
muscle weakness with mild elevation in Enzymes and Abnormal EMG &
Biopsy.
JuvenileDermatomyositis
Presents with clinical features similar to dermatomyositis with
following differences
 Age of onset ~ 7 years
 Less incidences of Malignancy and ILD.
 Increased risk for Vasculopathy, Lipodystrophy and calcinosis.
Associations: SOJIA ; Macrophage activation syndrome
 Less mortality.
InclusionBodyMyositis
• Symmetric or asymmetric weakness.
• Insidious onset.
• After 50 years, Male to female ratio of 3:1.
• Proximal and/or distal muscle involvement.
• Classically forearm flexors, finger flexors and Quadriceps.
• Facial and Pharyngeal muscles are more commonly involved than PM/DM.
Immune-MediatedNecrotizingMyopathy
• Subacute progressive proximal muscle weakness without a rash.
• Female to Male ratio 2:1.
• Generally develops more rapidly than with PM, and is markedly
severe.
• Associated myalgia and dysphagia can be seen.
• Associated with Paraneoplastic syndrome and Statin use.
ParaneoplasticNM
• Rare, rapid progression, association with Adenocarcinoma.
• Age > 40 years.
• Presentation in Fall or Winter.
• Antibodies against signal recognition particles (SRP) seen.
• Coexisting cardiomyopathy and ILD maybe present.
• Muscle Biopsy : Necrotic fibres infiltrated by macrophages and rare
T- cells
StatinInducedAutoimmuneNM(SANAM)
• Associated with statin use.
• Age ~ 46 to 89 years.
• Progress beyond 3 to 6 months after drug discontinuation.
• Require immunosuppresive therapy
AssociatedConditions
Systemic symptoms
e.g. fever, malaise ,wt. loss, arthralgia, Raynaud’s phenomenon
Joint contractures
mostly in DM and in children
Dysphagia
Cardiac
e.g. AV conduction defects, tachyarrythmias, DCMP, CHF
Interstitial lung diseases:
• Seen in 10-25% patients with PM/DM.
• Associated with Jo-1 antibodies.
 Malignancies:
• Risk increased in patients with DM only.
• Most common are ovarian, breast, colon cancer, melanoma and
non-Hodgkin lymphoma.
SC Calcifications
Arthralgias etc
AssociatedConditions
• Deforming arthritis of anti Jo-1
antibody patient.
Characteristics Polymyositis Dermatomyositis Inclusion Body
Myositis
Age at onset >18 yrs Adulthood and Childhood >50 yrs
Familial associations No No Yes, in some cases
Extramuscular
manifestations
Yes Yes Yes
Connective Tissue
Diseases
Yes Scleroderma and Mixed
CTD (Overlap Syndromes)
Yes in upto 20% of cases
Systemic AI Diseases Frequent Infrequent Infrequent
Malignancy No Yes in upto 15% of cases No
Drugs Yes Yes, rarely No
Parasites and Bacterias Yes No No
Viruses Yes Unproven Yes
FeaturesAssociatedwithInflammatory
Myopathies
Pathogenesis
• Both immune and non immune mechanism are
involved in pathogenesis.
• Immune mechanism involve T-cell, B-cells,Dendritic cells,
macrophages, cytokines and Antibodies.
• Non immune mechanisms involve endoplasmic reticulum stress ,
hypoxia and Autophagy.
ImmuneMechanism
• Immune Activators trigger
disease in myositis susceptibility
gene carriers.
• Environmental factors
may contribute
ImmuneMechanism
• Innate and Adaptive
responses are activated
in Lymph nodes
ImmuneMechanism
• Activated immune cells
enter general circulation,
and encounter with specific
antigen and penetrate
endothelia into local muscle
ImmuneMechanism
• In PM and IBM CD8+ Tcells
expressing perforin 1 and
Granzyme invade muscle fibres
expressing MHC 1 molecule
leading to muscle inflammation
and necrosis ( In IBM).
ImmuneMechanism
• In DM; CD4+ T cells ,
B-cells , Dendritic cells
and Macrophages
invade perivascular and
perimysial muscle
tissue.
MyositisSpecificAutoantibodies
Antisynthetasesyndrome
• Aminoacyl-tRNA synthetase is a cytoplasmic enzyme
involved in aminoacylation.
• The most common ARS is histidyl-RNAsynthetase,
also called Jo-1 (75%)
Antisynthetasesyndrome
• Myositis : Polymyositis or dermatomyositis
• Non-destructive polyarthritis of finger joints, wrists,
elbows or knees
• Fever in 80% of patients
• Mechanic’s hands : Thick cracked skin over the tips
and sides of the fingers
• Interstitial lung disease in over 70% of patients
• Raynaud’s phenomenon in 60% of patients
OtherMyositisSpecificAutoantibodies
Anti-PM-Sclantibodies
• Directed against a nucleolar macromolecular complex.
• Primarily polymyositis or dermatomyositis /scleroderma overlap.
• Strongly associated with HLA-DR3.
• Seen in 5-25% of patients with myositis
NonImmuneMechanism
HMGB1 : High mobility group protein B1
Diagnosis
• In 1975, Bohan and Peter used the following criteria for the diagnosis
and classification of PM and DM.
The Bohan and Peter Classification criteria
1. Symmetric proximal muscle weakness
2. Elevation of skeletal muscle enzyme levels.
3. Abnormal EMG results - Polyphasic, short, small motor unit potentials; fibrillation; positive
sharp waves; insertional irritability; and bizarre, high-frequency, repetitive discharges.
4. Muscle biopsy abnormalities -Degeneration/regeneration, perifascicular atrophy, necrosis,
phagocytosis, fiber size variation, and mononuclear inflammatory infiltrate
5. Typical skin rash of DM
DrawbacksofBohanandPetercriteria
• Case series and data developed from a single institution and based on
clinical observations.
• Only skin features were used to differentiate DM from PM.
• IBM was not recognized as a separate entity.
NewDiagnosticCriteria
• New diagnostic criteria considers :
1. Muscle Weakness.
2. Creatine Kinase
3. Electromyographic Findings.
4. Muscle biopsy.
5. Rash / Calcinosis.
MuscleWeakness
• In PM and DM there is progressive, symmetric proximal
muscle weakness with sparing of ocular and facial muscles.
• In IBM : Symmetric or asymmetric weakness, involving
the proximal and/or distal muscles classically forearm
flexors, finger flexors and quadriceps.
MuscleEnzymes
• In PM, Creatine Kinase is always elevated upto 5 – 50 times the ULN.
• In DM, CK is elevated upto 50 times the ULN, in 90% of patients.
While in others CK levels may be normal.
• In IBM, CK levels are elevated upto 10 times of UNL or
may be Normal.
• Necrotising Myopathy, CK is increased 10 times or more
EMGinMyopathies
• In acute myopathies EMG must be conducted about
3 weeks from the onset of symptoms to ensure good sensitivity.
• EMG of the muscle is done in two situations : at rest and at
voluntary activity.
• The Resting values also called spontaneous activity (not seen in
normal muscles) are of three types.
1.Fibrillations and Positive waves
2.High frequency discharges
3.Myotonic Discharges
EMGinMyopathies
• Fibrillations and Positive waves
These are small positive rhythmic electric potential typical of
denervation.
EMGinMyopathies
• High frequency discharges (Pseudomyotonic or Complex repetitive
discharges)
Rhythmic electric
potential with abrupt
beginning and end
typical of long standing
denervation.
EMGinMyopathies
Myotonic Discharges
• Electrophysiological representations of difficulty of relaxation
following voluntary muscle contraction.
EMGinMyopathies
• During Voluntary activity motor unit potentials (MUP) ie. are
recorded and MUP phase are studied
• ≥5, phases are called polyphasic.
• Acute myopathy: MUP are small in amplitude, short
duration and polyphasic.
• Chronic myopathy: MUP are large and of longer duration.
EMGinMyopathies
• EMG in IM show
• Increased insertional and spontaneous activity.
• Small-amplitude low-frequency fibrillation potentials
and positive sharp waves.
• Occasionally pseudomyotonic and complex repetitive
discharges indicating chronicity.
• Electrical myotonia : SANAM.
• Helpful in assessing relapse during treatment with
corticosteroids.
MusclebiopsyinPolymyositis
Endomysial inflammatory infiltrate (CD8+ MHC1 complexes) surrounding and
invading non-necrotic muscle fibers, with no vacuolization.
MusclebiopsyinDermatomyositis
Perifascicular and Perivascular infiltrates with perifascicular atrophy.
Inclusionbodymyositis
Basophilic rimmed vacuoles Vacuole filled with granules
Vacuolated muscle fibres infiltrated with CD8/MHC-1complexes. Beta-amyloid deposits and
cytochrome oxidase negative fibres may be seen.
NecrotizingMyopathy
Scattered necrotic myofibres with myophagocytosis in the paucity of T-lymphocytic
infiltration.
Polymyositis Dermatomyositis Inclusion Body
Myositis
Criterion Definite Probable
Myopathic Muscle
Weakness
Yes Yes Yes Yes, slow onset, early
involvement of distal
muscles, frequent falls
EMG Findings Myopathic Myopathic Myopathic Myopathic with mixed
potentials
Muscle Enzymes Elevated(upto
50 fold)
Elevated(upto
50 fold)
Elevated(upto 50 fold)
or Normal
Elevated(upto 10 fold)
or Normal
Muscle Biopsy Findings Primary
inflammation
with the
CD8/MHC-I
complex and no
vacuoles
Ubiquitous
MHC-I
expression
but
minimal
inflammati
on and no
vacuoles
Perifascicular,
perimysial, or
perivascular
infiltrates,
perifascicular
atrophy
Primary inflammation
with CD8/MHC-I
complex; vacuolated
fibres with -amyloid
deposits; cytochrome
oxygenase–negative
fibers; signs of chronic
myopathy
OtherInvestigations
• MRI-T1-weighted, T2-weighted, and sequences
using fat suppression techniques and short “tau” inversion recovery
(aka STIR), provides useful information to:
– diagnose myositis
– monitor treatment response,
– and identify a muscle site for biopsy.
• Muscle biopsy guided by MRI contains significantly more
inflammatory cells than the biopsy taken from MRI non-affected sites.
STIR imaging show
fibrosis or diffuse or
patchy signal symmetric
increase in the proximal
muscles and
intramuscular fascia
indicative of muscle
oedema due to
inflammation.
Other Investigations
Malignancy work up (for DM and PM)
• Chest , Abdomen and Pelvic CT scans
• Mammogram, Breast and Pelvic examination in women
• Colonoscopy in patients – Age >50 years or in those with
GI symptoms.
Other Investigations
In patients with IM:
• Chest X-ray, PFT and HRCT – especially in Jo1 positive
patients to rule out ILD.
• ECG- for myocardial involvement
• Echocardiogram- in patients with symptoms and signs of
heart failure.
• Video fluoroscopy- to objectively assess swallowing in
patients with dysphagia.
Treatment
Goals
• To eliminate inflammation.
• To restore muscle performance.
• To prevent chronic muscle disease.
• To prevent other organ system damage
• To regain quality of life
InitialTreatmentApproach
InitialTreatment
Approach Prednisone, 0.5-1 mg/kg/day.
for 2-4 wk
Concurrent with:
InitialTreatment
Approach Prednisone, 0.5-1 mg/kg/day.
for 2-4 wk
Concurrent with:
MTX, 15 mg/wk, to
Target dose of 25
mg/wk
Azathioprine, 2
mg/kgIBW
Twice daily
Mycophenolate mofetil,
500
mg
Twice daily. Increase by
500
mg/wk
until 1000 mg twice daily
InitialTreatment
Approach Prednisone, 0.5-1 mg/kg/day.
for 2-4 wk
Concurrent with:
MTX, 15 mg/wk, to
Target dose of 25
mg/wk
Azathioprine, 2
mg/kgIBW
Twice daily
Mycophenolate mofetil,
500
mg
Twice daily. Increase by
500
mg/wk
until 1000 mg twice daily
Taper prednisone every
2 wk
until completed:
60mg/d
40mg/d
30mg/d
25mg/d
20mg/d
17.5mg/d
15mg/d
12.5mg/d
10mg/d
7.5mg/d
5mg/d
2.5mg/d
Treatment
Initiate treatment with high dose corticosteroids and steroid sparing
agents.
• Prednisolone – Dose 0.5 to 1 mg/kg/day for 2- 4 weeks and then
taper off.
• Duration : 3 to 6 months.
• If no objective benefits after 3 months of high dose therapy switch
over to next immunosuppresive drug.
• Long term use of steroids may lead to steroid myopathy.
Treatment
• Azathioprine : Dose 2 mg/kg/day for 3 months.
• Before starting Azathioprine patient’s thiopurinemethyl- transferase
levels should be checked, otherwise it may lead to myelosuppression.
• Side Effects : Nausea, Loose stools, fever, and Liver toxicity
Treatment
• Methotrexate : Initial dose of 15 mg orally once weekly to a target
dose of 25 mg once weekly within 3 to 6 months.
• Folic acid supplementation to be given along.
• Side Effects : BM suppression, Liver toxicity, Pneumonitis.
• Should be used cautiously in patients with ILD
Treatment
• Mycophenolate Mofetil (MMF) : initial dose of 500mg BD to a dose of
1000mg BD over 4 weeks.
• Side Effects : Nausea, Loose stools, Leukopenia, elevation in liver
enzymes and Teratogenicity.
• Concurrent treatment : In Patients with ILD or on Prednisolone or
Immunosuppressive agents TMP-SMX prophylaxis is given for
Pneumocystis.
TreatmentMonitoring
• Within 2 to 4 weeks of starting MTX or azathioprine, a complete
blood cell count, liver enzyme function tests, and creatinine
measurement should be obtained once a month for 3 consecutive
months.
• Once a stable dosage is achieved, laboratory follow-up every 2 to 3
months thereafter is appropriate.
• DEXA scan is obtained at Baseline and Every year while patients
receive steroids.
SevereandRefractoryIM
• Myopathy refractory to conventional therapy or with severe organ-
threatening manifestations like ILD, severe dysphagia, notable weight
loss, severe rash, or weakness.
TreatmentofrefractoryIMorsevereorgan-
threateningIM
Methylprednisolone, 500-1000 mg/d IV (10-30 mg/kg/ day in JDM), for 1-3 days
IVIG, 1 g/kg (divided doses over 1-2 days) Repeated once monthly for 1-6 months
CPM, 0.6-1.0 g/m2 IV every 4 Wk or 1-2 mg/kg/day orally, for 3-12 months
Rituximab, 1000 mg repeated on day 15, Or 375 mg/m2 once weekly for 4 wk
CSA, 3.0-3.5 mg/kg/day
Treatment:IBM
• Treat patients of IBM with immunosuppression using the same
algorithms as used for DM and PM
• Goal is to suppress muscle inflammation, although the disease
typically is resistant to standardimmunotherapy.
Treatment:JDM
• Begin Prednisone at 2mg/kg to a max of 60mg/day.
• Subcutaneous MTX, at 15 mg/m2 once weekly is added at the onset.
SpecialConsiderations
Therapy for Skin disease.
• Avoid UV rays.
• Judicious use of Sunscreen with SPF of > 50.
• Topical steroids and Tacrolimus.
• Hydroxychloroquine at 200mg BD.
• For refractory cases MMF can be used.
SpecialConsiderations
Therapy for Calcinosis. Not satisfactory
• Drugs used are
• Diltiazem
• Colchicine
• Bisphosponates
• Intralesional Steroids.
• Abatacept
• Sodium Thiosulphate
Prognosis
• Older studies (before the availability of steroids) revealed a 50%
mortality from complications.
• Current estimates of mortality, excluding patients with malignancy, is
less than 10% at 5 years after initial diagnosis.
• DM has a favorable prognosis among all, IBM has least favorable
prognosis.
Poorprognosticfactors
• Older age
• Malignancy
• Delayed steroid treatment
• Dysphagia with aspiration
• ILD
TakeHomeMessage
• Inflammatory myopathies should be considered in all patients with
proximal muscle weakness.
• Diagnosis is made by Clinical findings, raised CPK, typical EMG and
muscle biopsynfindings.
• Treatment is by immunosuppression.
• Prognosis is generally good for DM and worst for IBM.
THANK YOU

More Related Content

What's hot

Idiopathic Inflammator Myopathie Dr.Mark Boulos June5 07fin
Idiopathic Inflammator Myopathie Dr.Mark Boulos June5 07finIdiopathic Inflammator Myopathie Dr.Mark Boulos June5 07fin
Idiopathic Inflammator Myopathie Dr.Mark Boulos June5 07fin
MedicineAndHealthNeurolog
 
Idiopathic inflammatory myopathy
Idiopathic inflammatory myopathyIdiopathic inflammatory myopathy
Idiopathic inflammatory myopathy
dattasrisaila
 

What's hot (20)

Polyarteritis nodosa
Polyarteritis nodosaPolyarteritis nodosa
Polyarteritis nodosa
 
Adult Still disease..pptx
Adult Still disease..pptxAdult Still disease..pptx
Adult Still disease..pptx
 
Sle
SleSle
Sle
 
Vasculitis
VasculitisVasculitis
Vasculitis
 
Systemic sclerosis..scleroderma
Systemic sclerosis..sclerodermaSystemic sclerosis..scleroderma
Systemic sclerosis..scleroderma
 
Dermatomyositis
DermatomyositisDermatomyositis
Dermatomyositis
 
Scleroderma
SclerodermaScleroderma
Scleroderma
 
Systemic Sclerosis
Systemic SclerosisSystemic Sclerosis
Systemic Sclerosis
 
Recent advances in idiopathic inflammatory myopathies by Dr. Naman Madaan
Recent advances in idiopathic inflammatory myopathies by Dr. Naman MadaanRecent advances in idiopathic inflammatory myopathies by Dr. Naman Madaan
Recent advances in idiopathic inflammatory myopathies by Dr. Naman Madaan
 
Dermatomyositis
DermatomyositisDermatomyositis
Dermatomyositis
 
Psoriatic arthropathy
Psoriatic arthropathyPsoriatic arthropathy
Psoriatic arthropathy
 
Dermatomyositis
DermatomyositisDermatomyositis
Dermatomyositis
 
Idiopathic Inflammator Myopathie Dr.Mark Boulos June5 07fin
Idiopathic Inflammator Myopathie Dr.Mark Boulos June5 07finIdiopathic Inflammator Myopathie Dr.Mark Boulos June5 07fin
Idiopathic Inflammator Myopathie Dr.Mark Boulos June5 07fin
 
Dermatomyositis Dr. Saad Raheem Abed
Dermatomyositis Dr. Saad Raheem AbedDermatomyositis Dr. Saad Raheem Abed
Dermatomyositis Dr. Saad Raheem Abed
 
Idiopathic inflammatory myopathy
Idiopathic inflammatory myopathyIdiopathic inflammatory myopathy
Idiopathic inflammatory myopathy
 
Bone tumors
Bone tumors   Bone tumors
Bone tumors
 
SERO-NEGATIVE ARTHRITIS
SERO-NEGATIVE ARTHRITISSERO-NEGATIVE ARTHRITIS
SERO-NEGATIVE ARTHRITIS
 
Dermatomyositis
DermatomyositisDermatomyositis
Dermatomyositis
 
Polymyositis Dermatomyositis
Polymyositis DermatomyositisPolymyositis Dermatomyositis
Polymyositis Dermatomyositis
 
Vasculitis
VasculitisVasculitis
Vasculitis
 

Similar to Inflammatory myositis

myopathy kireeti.pptx
myopathy kireeti.pptxmyopathy kireeti.pptx
myopathy kireeti.pptx
kireeti8
 
Exploring Polymyositis and Dermatomyositis: Understanding the Intricacies of ...
Exploring Polymyositis and Dermatomyositis: Understanding the Intricacies of ...Exploring Polymyositis and Dermatomyositis: Understanding the Intricacies of ...
Exploring Polymyositis and Dermatomyositis: Understanding the Intricacies of ...
muhammaduzair780907
 
Clinical aspects of neoplasia.pptx
Clinical aspects of neoplasia.pptxClinical aspects of neoplasia.pptx
Clinical aspects of neoplasia.pptx
MohammadFaisal565026
 

Similar to Inflammatory myositis (20)

inflammatorymyopathiesver2-160831211752.pptx
inflammatorymyopathiesver2-160831211752.pptxinflammatorymyopathiesver2-160831211752.pptx
inflammatorymyopathiesver2-160831211752.pptx
 
Dermatomyositis diskusi div adi alergi.pptx
Dermatomyositis diskusi div adi alergi.pptxDermatomyositis diskusi div adi alergi.pptx
Dermatomyositis diskusi div adi alergi.pptx
 
myopathy kireeti.pptx
myopathy kireeti.pptxmyopathy kireeti.pptx
myopathy kireeti.pptx
 
Exploring Polymyositis and Dermatomyositis: Understanding the Intricacies of ...
Exploring Polymyositis and Dermatomyositis: Understanding the Intricacies of ...Exploring Polymyositis and Dermatomyositis: Understanding the Intricacies of ...
Exploring Polymyositis and Dermatomyositis: Understanding the Intricacies of ...
 
Dermatomyositis PPT
Dermatomyositis PPTDermatomyositis PPT
Dermatomyositis PPT
 
Meningioma final
Meningioma finalMeningioma final
Meningioma final
 
Muscle disease final
Muscle disease finalMuscle disease final
Muscle disease final
 
Immune.pptx
Immune.pptxImmune.pptx
Immune.pptx
 
Approach to muscle disorders
Approach to muscle disordersApproach to muscle disorders
Approach to muscle disorders
 
Mctd final
Mctd finalMctd final
Mctd final
 
Multiple myeloma
Multiple myelomaMultiple myeloma
Multiple myeloma
 
Myositis
Myositis Myositis
Myositis
 
Cutaneous malignancies.pptx
Cutaneous malignancies.pptxCutaneous malignancies.pptx
Cutaneous malignancies.pptx
 
seminar on multiple myeloma.pptx
seminar on multiple myeloma.pptxseminar on multiple myeloma.pptx
seminar on multiple myeloma.pptx
 
Transvere myelitis
Transvere myelitisTransvere myelitis
Transvere myelitis
 
Brain tumor dr. abeer elsayed
Brain tumor dr. abeer elsayedBrain tumor dr. abeer elsayed
Brain tumor dr. abeer elsayed
 
122 Malignant gliomas anaplastic astrocytoma gb gliosarcoma
122 Malignant gliomas   anaplastic astrocytoma gb gliosarcoma122 Malignant gliomas   anaplastic astrocytoma gb gliosarcoma
122 Malignant gliomas anaplastic astrocytoma gb gliosarcoma
 
DERMATOMYOSITIS.pdf
DERMATOMYOSITIS.pdfDERMATOMYOSITIS.pdf
DERMATOMYOSITIS.pdf
 
Acute leukemias
Acute leukemiasAcute leukemias
Acute leukemias
 
Clinical aspects of neoplasia.pptx
Clinical aspects of neoplasia.pptxClinical aspects of neoplasia.pptx
Clinical aspects of neoplasia.pptx
 

More from Mohammad Rehan

More from Mohammad Rehan (11)

Uppergibleeding 150402032909-conversion-gate01-converted
Uppergibleeding 150402032909-conversion-gate01-convertedUppergibleeding 150402032909-conversion-gate01-converted
Uppergibleeding 150402032909-conversion-gate01-converted
 
Syncope 160319195211 (1)
Syncope 160319195211 (1)Syncope 160319195211 (1)
Syncope 160319195211 (1)
 
Sapho syndrome
Sapho syndromeSapho syndrome
Sapho syndrome
 
Rabies
RabiesRabies
Rabies
 
Plasmacelldisordersppt 111216180735-phpapp02
Plasmacelldisordersppt 111216180735-phpapp02Plasmacelldisordersppt 111216180735-phpapp02
Plasmacelldisordersppt 111216180735-phpapp02
 
Occupationallungdisease 160423173406
Occupationallungdisease 160423173406Occupationallungdisease 160423173406
Occupationallungdisease 160423173406
 
Inpatient hyperglycemia.ver3 (3)
Inpatient hyperglycemia.ver3 (3)Inpatient hyperglycemia.ver3 (3)
Inpatient hyperglycemia.ver3 (3)
 
Inflammatory bowel disease
Inflammatory bowel diseaseInflammatory bowel disease
Inflammatory bowel disease
 
Hyponatremiappt 170315180214
Hyponatremiappt 170315180214Hyponatremiappt 170315180214
Hyponatremiappt 170315180214
 
Basics of ecg
Basics of ecgBasics of ecg
Basics of ecg
 
Alcoholusedisorder 170517202616-converted
Alcoholusedisorder 170517202616-convertedAlcoholusedisorder 170517202616-converted
Alcoholusedisorder 170517202616-converted
 

Recently uploaded

Failure to thrive in neonates and infants + pediatric case.pptx
Failure to thrive in neonates and infants  + pediatric case.pptxFailure to thrive in neonates and infants  + pediatric case.pptx
Failure to thrive in neonates and infants + pediatric case.pptx
claviclebrown44
 
Difference Between Skeletal Smooth and Cardiac Muscles
Difference Between Skeletal Smooth and Cardiac MusclesDifference Between Skeletal Smooth and Cardiac Muscles
Difference Between Skeletal Smooth and Cardiac Muscles
MedicoseAcademics
 
💚Mature Women / Aunty Call Girl Chennai Escorts Service 💯Call Us 🔝 6367492432...
💚Mature Women / Aunty Call Girl Chennai Escorts Service 💯Call Us 🔝 6367492432...💚Mature Women / Aunty Call Girl Chennai Escorts Service 💯Call Us 🔝 6367492432...
💚Mature Women / Aunty Call Girl Chennai Escorts Service 💯Call Us 🔝 6367492432...
chaddanishu186
 

Recently uploaded (20)

Benefits of Chanting Hanuman Chalisa .pdf
Benefits of Chanting Hanuman Chalisa .pdfBenefits of Chanting Hanuman Chalisa .pdf
Benefits of Chanting Hanuman Chalisa .pdf
 
💚Gorgeous Mumbai Escorts Service 💯Call Us 🔝 9920874524 🔝💃Top Class Call Girl ...
💚Gorgeous Mumbai Escorts Service 💯Call Us 🔝 9920874524 🔝💃Top Class Call Girl ...💚Gorgeous Mumbai Escorts Service 💯Call Us 🔝 9920874524 🔝💃Top Class Call Girl ...
💚Gorgeous Mumbai Escorts Service 💯Call Us 🔝 9920874524 🔝💃Top Class Call Girl ...
 
parliaments-for-health-security_RecordOfAchievement.pdf
parliaments-for-health-security_RecordOfAchievement.pdfparliaments-for-health-security_RecordOfAchievement.pdf
parliaments-for-health-security_RecordOfAchievement.pdf
 
Gross Anatomy and Histology of Tongue by Dr. Rabia Inam Gandapore.pptx
Gross Anatomy and Histology of Tongue by Dr. Rabia Inam Gandapore.pptxGross Anatomy and Histology of Tongue by Dr. Rabia Inam Gandapore.pptx
Gross Anatomy and Histology of Tongue by Dr. Rabia Inam Gandapore.pptx
 
Borivali East * HiFi ℂall Girls Mumbai Phone No 9004268417 Elite ℂall Serviℂe...
Borivali East * HiFi ℂall Girls Mumbai Phone No 9004268417 Elite ℂall Serviℂe...Borivali East * HiFi ℂall Girls Mumbai Phone No 9004268417 Elite ℂall Serviℂe...
Borivali East * HiFi ℂall Girls Mumbai Phone No 9004268417 Elite ℂall Serviℂe...
 
Failure to thrive in neonates and infants + pediatric case.pptx
Failure to thrive in neonates and infants  + pediatric case.pptxFailure to thrive in neonates and infants  + pediatric case.pptx
Failure to thrive in neonates and infants + pediatric case.pptx
 
High Purity 99% PMK Ethyl Glycidate Powder CAS 28578-16-7
High Purity 99% PMK Ethyl Glycidate Powder CAS 28578-16-7High Purity 99% PMK Ethyl Glycidate Powder CAS 28578-16-7
High Purity 99% PMK Ethyl Glycidate Powder CAS 28578-16-7
 
VIP ℂall Girls Thane West Mumbai 9930245274 WhatsApp: Me All Time Serviℂe Ava...
VIP ℂall Girls Thane West Mumbai 9930245274 WhatsApp: Me All Time Serviℂe Ava...VIP ℂall Girls Thane West Mumbai 9930245274 WhatsApp: Me All Time Serviℂe Ava...
VIP ℂall Girls Thane West Mumbai 9930245274 WhatsApp: Me All Time Serviℂe Ava...
 
VIP ℂall Girls Korremula Hyderabad 9079923931 WhatsApp: Me All Time Serviℂe A...
VIP ℂall Girls Korremula Hyderabad 9079923931 WhatsApp: Me All Time Serviℂe A...VIP ℂall Girls Korremula Hyderabad 9079923931 WhatsApp: Me All Time Serviℂe A...
VIP ℂall Girls Korremula Hyderabad 9079923931 WhatsApp: Me All Time Serviℂe A...
 
Vesu * HiFi ℂall Girls Surat Phone No 8849756361 Elite ℂall Serviℂe Available...
Vesu * HiFi ℂall Girls Surat Phone No 8849756361 Elite ℂall Serviℂe Available...Vesu * HiFi ℂall Girls Surat Phone No 8849756361 Elite ℂall Serviℂe Available...
Vesu * HiFi ℂall Girls Surat Phone No 8849756361 Elite ℂall Serviℂe Available...
 
Unsatisfied Bhabhi ℂall Girls Surat Book Sneha 8849756361 Top Class ℂall Girl...
Unsatisfied Bhabhi ℂall Girls Surat Book Sneha 8849756361 Top Class ℂall Girl...Unsatisfied Bhabhi ℂall Girls Surat Book Sneha 8849756361 Top Class ℂall Girl...
Unsatisfied Bhabhi ℂall Girls Surat Book Sneha 8849756361 Top Class ℂall Girl...
 
Top 10 Most Beautiful Russian Pornstars List 2024
Top 10 Most Beautiful Russian Pornstars List 2024Top 10 Most Beautiful Russian Pornstars List 2024
Top 10 Most Beautiful Russian Pornstars List 2024
 
ABO Blood grouping in-compatibility in pregnancy
ABO Blood grouping in-compatibility in pregnancyABO Blood grouping in-compatibility in pregnancy
ABO Blood grouping in-compatibility in pregnancy
 
Lachesis Mutus- a Homoeopathic medicinel.pptx
Lachesis Mutus- a Homoeopathic medicinel.pptxLachesis Mutus- a Homoeopathic medicinel.pptx
Lachesis Mutus- a Homoeopathic medicinel.pptx
 
Top 10 Most Beautiful Chinese Pornstars List 2024
Top 10 Most Beautiful Chinese Pornstars List 2024Top 10 Most Beautiful Chinese Pornstars List 2024
Top 10 Most Beautiful Chinese Pornstars List 2024
 
Difference Between Skeletal Smooth and Cardiac Muscles
Difference Between Skeletal Smooth and Cardiac MusclesDifference Between Skeletal Smooth and Cardiac Muscles
Difference Between Skeletal Smooth and Cardiac Muscles
 
spinal cord disorders and paraplegia .
spinal cord disorders  and  paraplegia .spinal cord disorders  and  paraplegia .
spinal cord disorders and paraplegia .
 
TEST BANK For Guyton and Hall Textbook of Medical Physiology, 14th Edition by...
TEST BANK For Guyton and Hall Textbook of Medical Physiology, 14th Edition by...TEST BANK For Guyton and Hall Textbook of Medical Physiology, 14th Edition by...
TEST BANK For Guyton and Hall Textbook of Medical Physiology, 14th Edition by...
 
💚Mature Women / Aunty Call Girl Chennai Escorts Service 💯Call Us 🔝 6367492432...
💚Mature Women / Aunty Call Girl Chennai Escorts Service 💯Call Us 🔝 6367492432...💚Mature Women / Aunty Call Girl Chennai Escorts Service 💯Call Us 🔝 6367492432...
💚Mature Women / Aunty Call Girl Chennai Escorts Service 💯Call Us 🔝 6367492432...
 
Report Back from SGO: What’s the Latest in Ovarian Cancer?
Report Back from SGO: What’s the Latest in Ovarian Cancer?Report Back from SGO: What’s the Latest in Ovarian Cancer?
Report Back from SGO: What’s the Latest in Ovarian Cancer?
 

Inflammatory myositis

  • 2. Contents • Introduction • History • Classification • Clinical Features • Associated Conditions • Pathogenesis • Autoantibodies • Antisynthetase syndrome • Diagnosis • Treatment • Prognosis
  • 3. Introduction • Inflammatory Myopathies are sporadic disorders representing the largest group of acquired and potentially treatable causes of skeletal muscle weakness. • Annual incidence ~ 1 in 100,000 • Women > Men , Polymyositis (PM) and Dermatomyositis (DM) • Men > Women , Inclusion Body Myositis (IBM)
  • 4. Age at onset for Inflammatory myopathies is different. • PM > 18 years. • DM affects both Juvenile population and Adulthood. • IBM affects persons aged >50 years.
  • 5. History • In 1888 the first American biopsy documented polymyositis in ruling out Trichinella. • Heliotrope rash was first described in 1875 in France. • In 1930 Gottron’s reported skin lesions . • In 1967 the pathology of IBM was described.
  • 6. Classification • There is no internationally accepted classification system for Inflammatory myopathies. • For discussion purpose IM’s can be classified as :- Polymyositis. Dermatomyositis and Juvenile Dermatomyositis. Inclusion Body Myositis. Autoimmune Necrotising Myopathies. Myositis associated with Collagen Vascular disorder. Myositis associated with malignancy and Juvenile Dermatomyositis.
  • 7. ClinicalFeatures Polymyositis • A rare, subacute inflammatory myopathy affecting adults and rarely children. • Progressive, symmetric proximal muscle weakness with sparing of ocular and facial muscles. • Pharyngeal and Neck Flexors, also involved causing dysphagia and Head drop.
  • 8. • Associated with Myalgia and Muscle tenderness in some cases • In Advanced and Acute cases Respiratory muscles may be affected. • Severe weakness if untreated leads to muscle wasting. • Sensations and DTR’s remain intact.
  • 9. ClinicalFeatures Dermatomyositis Subacute inflammatory myopathy with muscle weakness similar to polymyositis with distinctive rash more often preceding the muscle weakness.
  • 10. DM:DermatologicManifestations Heliotrope rash • Periorbital violaceous erythema with or without edema of eyelids and periorbital tissue. • Highly characteristic of DM
  • 11. DM:DermatologicManifestations Gottron’s Papules • Violaceous flat topped papules and plaques over dorsal aspect of interphalangeal and MCP joints • Pathognomonic of DM, seen in > 80% patients with DM
  • 12. DM:DermatologicManifestations V- sign • Macular violaceous erythema over V-shaped region of the neck and upper chest. • Sometimes rash is pruritic.
  • 13. DM:DermatologicManifestations Shawl Sign • Macular violaceous erythema over Nape,back and shoulders. • May show photosensitivity
  • 14. DM:DermatologicManifestations Mechanic’s Hand • Also considered characteristic. • hyperkeratosis, scaling, and horizontal fissuring of the palms and fingers bilaterally. • Can be a manifestation of the Antisynthetase syndrome.
  • 16. AmyopathicandHypo-myopathic Dermatomyositis • Amyopathic DM : present with typical dermatological manifestations of DM, with normal muscle strength, enzyme levels and EMG findings. • Hypo-myopathic DM : Dermatological manifestations of DM with no muscle weakness with mild elevation in Enzymes and Abnormal EMG & Biopsy.
  • 17. JuvenileDermatomyositis Presents with clinical features similar to dermatomyositis with following differences  Age of onset ~ 7 years  Less incidences of Malignancy and ILD.  Increased risk for Vasculopathy, Lipodystrophy and calcinosis. Associations: SOJIA ; Macrophage activation syndrome  Less mortality.
  • 18. InclusionBodyMyositis • Symmetric or asymmetric weakness. • Insidious onset. • After 50 years, Male to female ratio of 3:1. • Proximal and/or distal muscle involvement. • Classically forearm flexors, finger flexors and Quadriceps. • Facial and Pharyngeal muscles are more commonly involved than PM/DM.
  • 19. Immune-MediatedNecrotizingMyopathy • Subacute progressive proximal muscle weakness without a rash. • Female to Male ratio 2:1. • Generally develops more rapidly than with PM, and is markedly severe. • Associated myalgia and dysphagia can be seen. • Associated with Paraneoplastic syndrome and Statin use.
  • 20. ParaneoplasticNM • Rare, rapid progression, association with Adenocarcinoma. • Age > 40 years. • Presentation in Fall or Winter. • Antibodies against signal recognition particles (SRP) seen. • Coexisting cardiomyopathy and ILD maybe present. • Muscle Biopsy : Necrotic fibres infiltrated by macrophages and rare T- cells
  • 21. StatinInducedAutoimmuneNM(SANAM) • Associated with statin use. • Age ~ 46 to 89 years. • Progress beyond 3 to 6 months after drug discontinuation. • Require immunosuppresive therapy
  • 22. AssociatedConditions Systemic symptoms e.g. fever, malaise ,wt. loss, arthralgia, Raynaud’s phenomenon Joint contractures mostly in DM and in children Dysphagia Cardiac e.g. AV conduction defects, tachyarrythmias, DCMP, CHF
  • 23. Interstitial lung diseases: • Seen in 10-25% patients with PM/DM. • Associated with Jo-1 antibodies.  Malignancies: • Risk increased in patients with DM only. • Most common are ovarian, breast, colon cancer, melanoma and non-Hodgkin lymphoma. SC Calcifications Arthralgias etc
  • 24. AssociatedConditions • Deforming arthritis of anti Jo-1 antibody patient.
  • 25. Characteristics Polymyositis Dermatomyositis Inclusion Body Myositis Age at onset >18 yrs Adulthood and Childhood >50 yrs Familial associations No No Yes, in some cases Extramuscular manifestations Yes Yes Yes Connective Tissue Diseases Yes Scleroderma and Mixed CTD (Overlap Syndromes) Yes in upto 20% of cases Systemic AI Diseases Frequent Infrequent Infrequent Malignancy No Yes in upto 15% of cases No Drugs Yes Yes, rarely No Parasites and Bacterias Yes No No Viruses Yes Unproven Yes FeaturesAssociatedwithInflammatory Myopathies
  • 26. Pathogenesis • Both immune and non immune mechanism are involved in pathogenesis. • Immune mechanism involve T-cell, B-cells,Dendritic cells, macrophages, cytokines and Antibodies. • Non immune mechanisms involve endoplasmic reticulum stress , hypoxia and Autophagy.
  • 27. ImmuneMechanism • Immune Activators trigger disease in myositis susceptibility gene carriers. • Environmental factors may contribute
  • 28. ImmuneMechanism • Innate and Adaptive responses are activated in Lymph nodes
  • 29. ImmuneMechanism • Activated immune cells enter general circulation, and encounter with specific antigen and penetrate endothelia into local muscle
  • 30. ImmuneMechanism • In PM and IBM CD8+ Tcells expressing perforin 1 and Granzyme invade muscle fibres expressing MHC 1 molecule leading to muscle inflammation and necrosis ( In IBM).
  • 31. ImmuneMechanism • In DM; CD4+ T cells , B-cells , Dendritic cells and Macrophages invade perivascular and perimysial muscle tissue.
  • 33. Antisynthetasesyndrome • Aminoacyl-tRNA synthetase is a cytoplasmic enzyme involved in aminoacylation. • The most common ARS is histidyl-RNAsynthetase, also called Jo-1 (75%)
  • 34. Antisynthetasesyndrome • Myositis : Polymyositis or dermatomyositis • Non-destructive polyarthritis of finger joints, wrists, elbows or knees • Fever in 80% of patients • Mechanic’s hands : Thick cracked skin over the tips and sides of the fingers • Interstitial lung disease in over 70% of patients • Raynaud’s phenomenon in 60% of patients
  • 36. Anti-PM-Sclantibodies • Directed against a nucleolar macromolecular complex. • Primarily polymyositis or dermatomyositis /scleroderma overlap. • Strongly associated with HLA-DR3. • Seen in 5-25% of patients with myositis
  • 37. NonImmuneMechanism HMGB1 : High mobility group protein B1
  • 38. Diagnosis • In 1975, Bohan and Peter used the following criteria for the diagnosis and classification of PM and DM. The Bohan and Peter Classification criteria 1. Symmetric proximal muscle weakness 2. Elevation of skeletal muscle enzyme levels. 3. Abnormal EMG results - Polyphasic, short, small motor unit potentials; fibrillation; positive sharp waves; insertional irritability; and bizarre, high-frequency, repetitive discharges. 4. Muscle biopsy abnormalities -Degeneration/regeneration, perifascicular atrophy, necrosis, phagocytosis, fiber size variation, and mononuclear inflammatory infiltrate 5. Typical skin rash of DM
  • 39. DrawbacksofBohanandPetercriteria • Case series and data developed from a single institution and based on clinical observations. • Only skin features were used to differentiate DM from PM. • IBM was not recognized as a separate entity.
  • 40. NewDiagnosticCriteria • New diagnostic criteria considers : 1. Muscle Weakness. 2. Creatine Kinase 3. Electromyographic Findings. 4. Muscle biopsy. 5. Rash / Calcinosis.
  • 41. MuscleWeakness • In PM and DM there is progressive, symmetric proximal muscle weakness with sparing of ocular and facial muscles. • In IBM : Symmetric or asymmetric weakness, involving the proximal and/or distal muscles classically forearm flexors, finger flexors and quadriceps.
  • 42. MuscleEnzymes • In PM, Creatine Kinase is always elevated upto 5 – 50 times the ULN. • In DM, CK is elevated upto 50 times the ULN, in 90% of patients. While in others CK levels may be normal. • In IBM, CK levels are elevated upto 10 times of UNL or may be Normal. • Necrotising Myopathy, CK is increased 10 times or more
  • 43. EMGinMyopathies • In acute myopathies EMG must be conducted about 3 weeks from the onset of symptoms to ensure good sensitivity. • EMG of the muscle is done in two situations : at rest and at voluntary activity. • The Resting values also called spontaneous activity (not seen in normal muscles) are of three types. 1.Fibrillations and Positive waves 2.High frequency discharges 3.Myotonic Discharges
  • 44. EMGinMyopathies • Fibrillations and Positive waves These are small positive rhythmic electric potential typical of denervation.
  • 45. EMGinMyopathies • High frequency discharges (Pseudomyotonic or Complex repetitive discharges) Rhythmic electric potential with abrupt beginning and end typical of long standing denervation.
  • 46. EMGinMyopathies Myotonic Discharges • Electrophysiological representations of difficulty of relaxation following voluntary muscle contraction.
  • 47. EMGinMyopathies • During Voluntary activity motor unit potentials (MUP) ie. are recorded and MUP phase are studied • ≥5, phases are called polyphasic. • Acute myopathy: MUP are small in amplitude, short duration and polyphasic. • Chronic myopathy: MUP are large and of longer duration.
  • 48. EMGinMyopathies • EMG in IM show • Increased insertional and spontaneous activity. • Small-amplitude low-frequency fibrillation potentials and positive sharp waves. • Occasionally pseudomyotonic and complex repetitive discharges indicating chronicity. • Electrical myotonia : SANAM. • Helpful in assessing relapse during treatment with corticosteroids.
  • 49. MusclebiopsyinPolymyositis Endomysial inflammatory infiltrate (CD8+ MHC1 complexes) surrounding and invading non-necrotic muscle fibers, with no vacuolization.
  • 50. MusclebiopsyinDermatomyositis Perifascicular and Perivascular infiltrates with perifascicular atrophy.
  • 51. Inclusionbodymyositis Basophilic rimmed vacuoles Vacuole filled with granules Vacuolated muscle fibres infiltrated with CD8/MHC-1complexes. Beta-amyloid deposits and cytochrome oxidase negative fibres may be seen.
  • 52. NecrotizingMyopathy Scattered necrotic myofibres with myophagocytosis in the paucity of T-lymphocytic infiltration.
  • 53. Polymyositis Dermatomyositis Inclusion Body Myositis Criterion Definite Probable Myopathic Muscle Weakness Yes Yes Yes Yes, slow onset, early involvement of distal muscles, frequent falls EMG Findings Myopathic Myopathic Myopathic Myopathic with mixed potentials Muscle Enzymes Elevated(upto 50 fold) Elevated(upto 50 fold) Elevated(upto 50 fold) or Normal Elevated(upto 10 fold) or Normal Muscle Biopsy Findings Primary inflammation with the CD8/MHC-I complex and no vacuoles Ubiquitous MHC-I expression but minimal inflammati on and no vacuoles Perifascicular, perimysial, or perivascular infiltrates, perifascicular atrophy Primary inflammation with CD8/MHC-I complex; vacuolated fibres with -amyloid deposits; cytochrome oxygenase–negative fibers; signs of chronic myopathy
  • 54. OtherInvestigations • MRI-T1-weighted, T2-weighted, and sequences using fat suppression techniques and short “tau” inversion recovery (aka STIR), provides useful information to: – diagnose myositis – monitor treatment response, – and identify a muscle site for biopsy. • Muscle biopsy guided by MRI contains significantly more inflammatory cells than the biopsy taken from MRI non-affected sites.
  • 55. STIR imaging show fibrosis or diffuse or patchy signal symmetric increase in the proximal muscles and intramuscular fascia indicative of muscle oedema due to inflammation.
  • 56. Other Investigations Malignancy work up (for DM and PM) • Chest , Abdomen and Pelvic CT scans • Mammogram, Breast and Pelvic examination in women • Colonoscopy in patients – Age >50 years or in those with GI symptoms.
  • 57. Other Investigations In patients with IM: • Chest X-ray, PFT and HRCT – especially in Jo1 positive patients to rule out ILD. • ECG- for myocardial involvement • Echocardiogram- in patients with symptoms and signs of heart failure. • Video fluoroscopy- to objectively assess swallowing in patients with dysphagia.
  • 58. Treatment Goals • To eliminate inflammation. • To restore muscle performance. • To prevent chronic muscle disease. • To prevent other organ system damage • To regain quality of life
  • 60. InitialTreatment Approach Prednisone, 0.5-1 mg/kg/day. for 2-4 wk Concurrent with:
  • 61. InitialTreatment Approach Prednisone, 0.5-1 mg/kg/day. for 2-4 wk Concurrent with: MTX, 15 mg/wk, to Target dose of 25 mg/wk Azathioprine, 2 mg/kgIBW Twice daily Mycophenolate mofetil, 500 mg Twice daily. Increase by 500 mg/wk until 1000 mg twice daily
  • 62. InitialTreatment Approach Prednisone, 0.5-1 mg/kg/day. for 2-4 wk Concurrent with: MTX, 15 mg/wk, to Target dose of 25 mg/wk Azathioprine, 2 mg/kgIBW Twice daily Mycophenolate mofetil, 500 mg Twice daily. Increase by 500 mg/wk until 1000 mg twice daily Taper prednisone every 2 wk until completed: 60mg/d 40mg/d 30mg/d 25mg/d 20mg/d 17.5mg/d 15mg/d 12.5mg/d 10mg/d 7.5mg/d 5mg/d 2.5mg/d
  • 63. Treatment Initiate treatment with high dose corticosteroids and steroid sparing agents. • Prednisolone – Dose 0.5 to 1 mg/kg/day for 2- 4 weeks and then taper off. • Duration : 3 to 6 months. • If no objective benefits after 3 months of high dose therapy switch over to next immunosuppresive drug. • Long term use of steroids may lead to steroid myopathy.
  • 64. Treatment • Azathioprine : Dose 2 mg/kg/day for 3 months. • Before starting Azathioprine patient’s thiopurinemethyl- transferase levels should be checked, otherwise it may lead to myelosuppression. • Side Effects : Nausea, Loose stools, fever, and Liver toxicity
  • 65. Treatment • Methotrexate : Initial dose of 15 mg orally once weekly to a target dose of 25 mg once weekly within 3 to 6 months. • Folic acid supplementation to be given along. • Side Effects : BM suppression, Liver toxicity, Pneumonitis. • Should be used cautiously in patients with ILD
  • 66. Treatment • Mycophenolate Mofetil (MMF) : initial dose of 500mg BD to a dose of 1000mg BD over 4 weeks. • Side Effects : Nausea, Loose stools, Leukopenia, elevation in liver enzymes and Teratogenicity. • Concurrent treatment : In Patients with ILD or on Prednisolone or Immunosuppressive agents TMP-SMX prophylaxis is given for Pneumocystis.
  • 67. TreatmentMonitoring • Within 2 to 4 weeks of starting MTX or azathioprine, a complete blood cell count, liver enzyme function tests, and creatinine measurement should be obtained once a month for 3 consecutive months. • Once a stable dosage is achieved, laboratory follow-up every 2 to 3 months thereafter is appropriate. • DEXA scan is obtained at Baseline and Every year while patients receive steroids.
  • 68. SevereandRefractoryIM • Myopathy refractory to conventional therapy or with severe organ- threatening manifestations like ILD, severe dysphagia, notable weight loss, severe rash, or weakness.
  • 69. TreatmentofrefractoryIMorsevereorgan- threateningIM Methylprednisolone, 500-1000 mg/d IV (10-30 mg/kg/ day in JDM), for 1-3 days IVIG, 1 g/kg (divided doses over 1-2 days) Repeated once monthly for 1-6 months CPM, 0.6-1.0 g/m2 IV every 4 Wk or 1-2 mg/kg/day orally, for 3-12 months Rituximab, 1000 mg repeated on day 15, Or 375 mg/m2 once weekly for 4 wk CSA, 3.0-3.5 mg/kg/day
  • 70. Treatment:IBM • Treat patients of IBM with immunosuppression using the same algorithms as used for DM and PM • Goal is to suppress muscle inflammation, although the disease typically is resistant to standardimmunotherapy.
  • 71. Treatment:JDM • Begin Prednisone at 2mg/kg to a max of 60mg/day. • Subcutaneous MTX, at 15 mg/m2 once weekly is added at the onset.
  • 72. SpecialConsiderations Therapy for Skin disease. • Avoid UV rays. • Judicious use of Sunscreen with SPF of > 50. • Topical steroids and Tacrolimus. • Hydroxychloroquine at 200mg BD. • For refractory cases MMF can be used.
  • 73. SpecialConsiderations Therapy for Calcinosis. Not satisfactory • Drugs used are • Diltiazem • Colchicine • Bisphosponates • Intralesional Steroids. • Abatacept • Sodium Thiosulphate
  • 74. Prognosis • Older studies (before the availability of steroids) revealed a 50% mortality from complications. • Current estimates of mortality, excluding patients with malignancy, is less than 10% at 5 years after initial diagnosis. • DM has a favorable prognosis among all, IBM has least favorable prognosis.
  • 75. Poorprognosticfactors • Older age • Malignancy • Delayed steroid treatment • Dysphagia with aspiration • ILD
  • 76. TakeHomeMessage • Inflammatory myopathies should be considered in all patients with proximal muscle weakness. • Diagnosis is made by Clinical findings, raised CPK, typical EMG and muscle biopsynfindings. • Treatment is by immunosuppression. • Prognosis is generally good for DM and worst for IBM.