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MYOSITISZia ul HaqJawad Munir
Definition
Sabacute or slowly progressive, symmetrical weakness primarily affecting the proximal limb and trunk muscles.
Classification of Myositis
Polymyositis DermatomyositisPoly/Dermato-myositis associated with
MalignancyPoly/Dermato-myositis associated with
Connective Tissue DiseaseInclusion Body myositisAmyopathic Dermatomyositis
EPIDEMIOLOGY
Annual Incidence: 2-10 case /millionPeak age : 10-15 and 45-55 years old
Female: Male ratio: 3:1 total Black: White : 4:1
CLINICAL MANIFESTATIONS
Weakness of muscles -shoulder girdle - pelvic girdle - neck flexorsMyalgia is minimal
Constitutional symptoms-fatigue, fever, weight loss
CLINICAL MANIFESTATIONS
Rheumatic: Arthralgia/Arthritis 20-70%
Pulmonary: Interstitial Lung Disease 10%
GIT: Esophageal Dysmotility 10-30%Cardiac: Conduction Blocks and
ArrhythmiaVascular: Raynaud’s Phenomenon 20-
40%Skin : Rash, Livedo Reticularis
Myositis-PROGNOSIS
5-YEAR SURVIVAL IS AROUND 85% IN PM, DM, PM/DM ASSOC. CTD.
MUCH LOWER IN PM/DM ASSOC. MALIGNANCY.
Polymyositis
A persistent inflammatory muscle disease that causes weakness of the skeletal muscles, which control movement.
Medically, polymyositis is classified as a chronic inflammatory myopathy — one of only three such diseases.
Polymyositis
Can occur at any age, Adults -30s, 40s or 50s. BlacksWomen
Polymyositis
signs and symptoms usually develop gradually,over weeks or months.
Remissions - rare
Remissions: periods during which symptoms spontaneously disappear
Signs and symptoms
appear gradually,Progressive muscle weakness Difficulty swallowing (dysphagia) Difficulty speakingMild joint or muscle tenderness FatigueShortness of breath
Signs and symptoms
affects the muscles closest to the trunk, particularly hips, thighs, shoulders, upper arms and neck.
weakness is symmetricalworsens over time.As muscle weakness progresses,
difficult to climb stairs, rise from a seated position, liftobjects or reach overhead.
Complications
DysphagiaWhich in turn may cause weight loss and malnutrition.
Aspiration pneumoniaShortness of breath or respiratory failure.
CalcinosisCalcium deposits in muscles, skin and connective tissues
Associated conditions
Polymyositis is often associated with other conditions that may cause further complications of their own, or in combination with polymyositis symptoms. Associated conditions include:Raynaud's phenomenon.
Other connective tissue diseases. Cardiovascular disease.Lung disease.
DIAGNOSTIC CRITERIA
1 .PROXIMAL MOTOR WEAKNESS: symmetric, proximal muscles
2 .HIGH SERUM MUSCLE ENZYMES: CPK, aldolase, myoglobin, AST,
ALT, LDH
DIAGNOSTIC CRITERIA
3 .EDT= electro-diagnostic tests
NEUROPATHIC DISORDER PM/DM
-Poly-phasic action potentials
-long duration-large amplitude
-Poly-phasic action potentials
-short duration-low amplitude
EMG
abnormal normal NCV
DIAGNOSTIC CRITERIA
4 .MUSCLE BIOPSY: biopsy a clinically weak muscle, contralateral to an
abnormal muscle ( by EDT), MRI directed.
a. Perivascular and endomysial inflammationCD8+ T cells in PM,
CD8+, CD4+ T and B cells in DM
.bb. Muscle fiber necrosis and regeneration
LABORATORY TESTS
HIGH MUSCLE ENZYMES:- CPKELEVATED ESR , CRP:- 50%POSITIVE ANA:- 50-80%
AUTOANTIBODIES-: anti- RNP (MCTD)
anti-PM/Scl (OVERLAP)
Myositis-specific AUTOANTIBODIES
ANTI Jo-1 part of ANTI SYNTHETASE Ab’s Antibodies to the antigen- Aminoacyl-tRNA synthetase, in 20-50% of PM>>DM ANTI SRP = anti signal recognition particleIn 5% of PM
ANTI Mi-2 in 10% of DM .
Treatment
1 .STEROIDS 2 .IMMUNOSUPPRESSIVE AGENTS:
methotrexate, azathioprine, cytoxan, cellcept3 .IMMUNOMODULATORY AGENTS:
IVIG, Plasmapheresis4 .REHABILITATION
Cytokines in Inflammation
Pro-inflammatory Anti-inflammatory
TNFaIL-1b
sTNFRIL-10
IL-1Ra
Macrophage or Activated T Cell
Soluble TNFa
Target Cell
TNFa Receptor
Signal Induction
Synthesis and Function of TNFa
Transmembrane TNFa
Receptor-Bound TNFa
Adapted with permission from Choy EHS, Panayi GS. N Engl J Med. 2001;344:907-916.Copyright © 2001 Massachusetts Medical Society. All rights reserved.
Inhibition of Cytokines
Inflammatory cytokine
Normal interaction Neutralization of cytokines
Receptor blockadeActivation of
anti-inflammatory pathways
Cytokine receptor
Soluble receptor
Monoclonal antibody
Monoclonal antibody
Receptor antagonist
Anti-inflammatorycytokine
Suppression ofinflammatorycytokines
No signal
No signal
Inflammatory signal
Humira- The first fully human antiTNFa
Chimeric Antibody
70% Human
Humanized Antibody
95% Human
Fully Human Antibody
100% HumanMouse
Human
Chimeric Humanized Fully Human
24
Treatment
corticosteroidWhen muscle strength improves, usually in 4 to 6weeks, the medication is slowly tapered off.Maintenance therapy with prednisone may becontinued indefinitely.
DMARDs - If unresponsive to corticosteroids
methotrexate and azathioprine,
Treatment
Intravenous gamma globulinIVIGis a purified blood product that contains healthy antibodies from thousands of blood donors.
The healthy antibodies in IVIG can block the damaging antibodies that attack muscle in polymyositis.
Repeat infusions q 6-8 weeks
Other immunosuppressivemedicine
Tacrolimus (Prograf)is a transplant-rejection drug that may work to inhibitthe immune system.
Immunosuppressants,cyclophosphamide (Cytoxan) and cyclosporine
)Gengraf, Neoral, Sandimmune,(
Biological therapies
Rituximab (Rituxan)Tumor necrosis factor (TNF) inhibitorsetanercept (Enbrel) and infliximab (Remicade),
Nursing management
Coping and support Educate about the illness Balance Rest and exercise
Myositis associated with skin diseases
Dermatomyositis
Dermatomyositis
a muscle disease characterized by inflammation and a skin rash. It is a type of inflammatory myopathy.
5 - 15 and adults age 40 - 60.Women
Polymyositis is a similar condition, but the symptoms occur without a skin rash.
Maria Carmela L. Domocmat, RN, MSN
Symptoms
DysphagiaMuscle weakness, stiffness, or soreness Purple or violet colored upper eyelids Purple-red skin (violaceous) rashSOB
Maria Carmela L. Domocmat, RN, MSN
Symptoms
The muscle weakness may appear suddenly or develop slowly over weeks or months. may have difficulty raising arms over head, rising from a sitting position, and climbing stairs.The rash may appear over the face, knuckles, neck,shoulders, upper chest, and back.
Maria Carmela L. Domocmat, RN, MSN
reddish-purple (violaceous) rash
reddish-purple(violaceous) rash.The rash is named after the tendency of plants to grow toward the sun (heliotropic) and is characteristic of dermatomyositis.
Maria Carmela L. Domocmat, RN, MSN
purple (violaceous) plaques
The appearance of purple (violaceous) plaques on the knees may be associated with dermatomyositis.
Maria Carmela L. Domocmat, RN, MSN
Gottron's sign
Red, thickened, scaly skin over the knuckles
Maria Carmela L. Domocmat, RN, MSN
Heliotrope eyelids
eyelids develop a brown (violaceous - rather than red) color.Heliotrope eyelids and Gottron's papules on the knuckles are characteristic findings in dermatomyositis.
Maria Carmela L. Domocmat, RN, MSN
violet-colored inflammation (erythema) over theknuckles
Maria Carmela L. Domocmat, RN, MSN
periungual erythema
Candida paronychia produced periungualerythema, edema and nail fold maceration.
Maria Carmela L. Domocmat, RN, MSN
Dx Exams
CPK & aldolaseECGElectromyographyMagnetic resonance imaging (MRI) Muscle biopsy
Maria Carmela L. Domocmat, RN, MSN
Treatment
CorticosteroidsImmunosuppressantsWhen muscle strength gets better – taper off corticosHowever, most people take prednisoneindefinitely.If the condition is associated with a tumor, the muscle weakness and rash may improve when the tumor is removed.
Maria Carmela L. Domocmat, RN, MSN
Outlook (Prognosis)
Some recover and have symptoms completelydisappear - especially in children.
In adults, death may result from severe and prolonged muscle weakness,
malnutrition, pneumonia, or lung failure. The major causes of death are cancer (malignancy) and lung disease.
Maria Carmela L. Domocmat, RN, MSN
Possible Complications
Acute renal failure Cancer (malignancy) Inflammation of the heart Joint painLung disease
Maria Carmela L. Domocmat, RN, MSN
DIFFERENTIAL DIAGNOSIS
-MYOPATHY- DRUG/TOXIN-NEURO-MUSCULAR-ENDOCRINE DISEASE-INFECTIOUS MYOSITIS-METABOLIC STORAGE MYOPATHIES -MITOCHONDRIAL MYOPATHY-OTHERS
ANTI-SYNTHETASE SYNDROME
associated with anti-Jo1 antibodies with acute onset of PM>> DM disease.
Associated with ILD -40-60%, deforming and non-erosive arthritis, Mechanic’s hands and Raynaud’s phenomenon
Steroid response
prognosis HLA Clinical association
prevalence Ab’s
moderate moderate DR3 Anti-synthetase syndrome
20-50%)PM(
Anti-Jo-1
poor bad)cardiac(
DR5 Severe PM 5%)PM(
Anti-SRP
good good DR7 Classical DM
5-10%)DM(
Anti-Mi-2
Myositis-specific AUTOANTIBODIES
Poly/Dermato-myositis associated with malignancy
Associated neoplasms present within the first 2 years of PM/DM followup
In PM- 10 %In DM- 15 %Reports of: carcinoma-lung, stomach,
ovary lymphomaRoutine screening in DM