MYOSITIS
Zia ul Haq
Jawad Munir
Definition
Sabacute or slowly progressive, symmetrical
weakness primarily affecting the proximal
limb and trunk muscles.
Classification of Myositis
Polymyositis
Dermatomyositis
Poly/Dermato-myositis associated with
Malignancy
Poly/Dermato-myos...
EPIDEMIOLOGY
Annual Incidence: 2-10 case /million
Peak age : 10-15 and 45-55 years old
Female: Male ratio: 3:1 total
Black...
CLINICAL MANIFESTATIONS
Weakness of muscles
-shoulder girdle
- pelvic girdle
- neck flexors
Myalgia is minimal
Constitutio...
CLINICAL MANIFESTATIONS
Rheumatic: Arthralgia/Arthritis 20-70%
Pulmonary: Interstitial Lung Disease 10%
GIT: Esophageal Dy...
Myositis-PROGNOSIS
5-YEAR SURVIVAL IS AROUND 85% IN PM,
DM, PM/DMASSOC. CTD.
MUCH LOWER IN PM/DMASSOC.
MALIGNANCY.
Polymyositis
A persistent inflammatory muscle disease that
causes weakness of the skeletal muscles, which
control movement...
Polymyositis
Can occur at any age,
Adults -30s, 40s or 50s.
Blacks
Women
Polymyositis
signs and symptoms usually develop gradually,
over weeks or months.
Remissions - rare
Remissions: periods dur...
Signs and symptoms
appear gradually,
Progressive muscle weakness
Difficulty swallowing (dysphagia)
Difficulty speaking
Mil...
Signs and symptoms
affects the muscles closest to the trunk,
particularly hips, thighs, shoulders, upper arms
and neck.
we...
Complications
Dysphagia
Which in turn may cause weight loss and malnutrition.
Aspiration pneumonia
Shortness of breath or ...
Associated conditions
Polymyositis is often associated with other
conditions that may cause further complications
of their...
DIAGNOSTIC CRITERIA
1. PROXIMAL MOTOR WEAKNESS:
symmetric, proximal muscles
2. HIGH SERUM MUSCLE ENZYMES:
CPK, aldolase, m...
DIAGNOSTIC CRITERIA
3. EDT= electro-diagnostic tests
NEUROPATHIC
DISORDER
PM/DM
-Poly-phasic action
potentials
-long durat...
DIAGNOSTIC CRITERIA
4. MUSCLE BIOPSY:
biopsy a clinically weak muscle, contralateral to an
abnormal muscle ( by EDT), MRI ...
LABORATORY TESTS
HIGH MUSCLE ENZYMES:- CPK
ELEVATED ESR , CRP:- 50%
POSITIVE ANA:- 50-80%
AUTOANTIBODIES:-
anti- RNP (MCTD...
Myositis-specific
AUTOANTIBODIES
ANTI Jo-1 part of ANTI SYNTHETASE Ab’s
Antibodies to the antigen- Aminoacyl-tRNA
syntheta...
Treatment
1. STEROIDS
2. IMMUNOSUPPRESSIVE AGENTS:
methotrexate, azathioprine, cytoxan, cellcept
3. IMMUNOMODULATORY AGENT...
Cytokines in Inflammation
Pro-inflammatory Anti-inflammatory
TNFa
IL-1b
sTNFR
IL-10
IL-1Ra
Macrophage or
Activated T Cell
Soluble TNFa
Target Cell
TNFa
Receptor
Signal Induction
Synthesis and Function of
TNFa
Tran...
Adapted with permission from Choy EHS, Panayi GS. N Engl J Med. 2001;344:907-916.
Copyright © 2001 Massachusetts Medical S...
Humira- The first fully human
antiTNFa
Chimeric
Antibody
70% Human
Humanized
Antibody
95% Human
Fully Human
Antibody
100% ...
Treatment
corticosteroid
When muscle strength improves, usually in 4 to 6
weeks, the medication is slowly tapered off.
Mai...
Treatment
Intravenous gamma globulin
IVIG
is a purified blood product that contains healthy
antibodies from thousands of b...
Other immunosuppressive
medicine
Tacrolimus (Prograf)
is a transplant-rejection drug that may work to inhibit
the immune s...
Biological therapies
Rituximab (Rituxan)
Tumor necrosis factor (TNF) inhibitors
etanercept (Enbrel) and infliximab (Remica...
Nursing management
Coping and support
Educate about the illness
Balance Rest and exercise
Myositis associated with skin diseases
Dermatomyositis
a muscle disease characterized by inflammation
and a skin rash. It is a type of inflammatory
myopathy.
5 -...
Symptoms
Dysphagia
Muscle weakness, stiffness, or soreness
Purple or violet colored upper eyelids
Purple-red skin (violace...
Symptoms
The muscle weakness may appear suddenly or develop
slowly over weeks or months. may have difficulty raising
arms ...
reddish-purple (violaceous) rash
reddish-purple
(violaceous) rash.
The rash is named after
the tendency of plants to
grow ...
purple (violaceous) plaques
The appearance of purple
(violaceous) plaques on
the knees may be
associated with
dermatomyosi...
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 ...
violet-colored inflammation (erythema) over the
knuckles
Maria Carmela L. Domocmat, RN, MSN
periungual erythema
Candida paronychia produced periungual
erythema, edema and nail fold maceration.
Maria Carmela L. Domo...
Dx Exams
CPK & aldolase
ECG
Electromyography
Magnetic resonance imaging (MRI)
Muscle biopsy
Maria Carmela L. Domocmat, RN,...
Treatment
Corticosteroids
Immunosuppressants
When muscle strength gets better – taper off
corticos
However, most people ta...
Outlook (Prognosis)
Some recover and have symptoms completely
disappear - especially in children.
In adults, death may res...
Possible Complications
Acute renal failure
Cancer (malignancy)
Inflammation of the heart
Joint pain
Lung disease
Maria Car...
DIFFERENTIAL DIAGNOSIS
-MYOPATHY- DRUG/TOXIN
-NEURO-MUSCULAR
-ENDOCRINE DISEASE
-INFECTIOUS MYOSITIS
-METABOLIC STORAGE MY...
ANTI-SYNTHETASE SYNDROME
associated with anti-Jo1 antibodies with acute onset of PM>> DM
disease.
Associated with ILD -40-...
Steroid
response
prognosisHLAClinical
association
prevalenceAb’s
moderatemoderateDR3Anti-
synthetase
syndrome
20-50%
(PM)
...
Poly/Dermato-myositis
associated with malignancy
Associated neoplasms present within the first 2
years of PM/DM followup
I...
Polymyositis Dermatomyositis
of 48

Polymyositis Dermatomyositis

It is a slide show about the Inflammatory Muscle Diseases with particular focus on Polymyositis and Dermatomyositis.
Published on: Mar 4, 2016
Published in: Health & Medicine      
Source: www.slideshare.net


Transcripts - Polymyositis Dermatomyositis

  • 1. MYOSITIS Zia ul Haq Jawad Munir
  • 2. Definition Sabacute or slowly progressive, symmetrical weakness primarily affecting the proximal limb and trunk muscles.
  • 3. Classification of Myositis Polymyositis Dermatomyositis Poly/Dermato-myositis associated with Malignancy Poly/Dermato-myositis associated with Connective Tissue Disease Inclusion Body myositis Amyopathic Dermatomyositis
  • 4. EPIDEMIOLOGY Annual Incidence: 2-10 case /million Peak age : 10-15 and 45-55 years old Female: Male ratio: 3:1 total Black:White : 4:1
  • 5. CLINICAL MANIFESTATIONS Weakness of muscles -shoulder girdle - pelvic girdle - neck flexors Myalgia is minimal Constitutional symptoms-fatigue, fever, weight loss
  • 6. CLINICAL MANIFESTATIONS Rheumatic: Arthralgia/Arthritis 20-70% Pulmonary: Interstitial Lung Disease 10% GIT: Esophageal Dysmotility 10-30% Cardiac: Conduction Blocks and Arrhythmia Vascular: Raynaud’s Phenomenon 20-40% Skin : Rash, Livedo Reticularis
  • 7. Myositis-PROGNOSIS 5-YEAR SURVIVAL IS AROUND 85% IN PM, DM, PM/DMASSOC. CTD. MUCH LOWER IN PM/DMASSOC. MALIGNANCY.
  • 8. 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.
  • 9. Polymyositis Can occur at any age, Adults -30s, 40s or 50s. Blacks Women
  • 10. Polymyositis signs and symptoms usually develop gradually, over weeks or months. Remissions - rare Remissions: periods during which symptoms spontaneously disappear
  • 11. Signs and symptoms appear gradually, Progressive muscle weakness Difficulty swallowing (dysphagia) Difficulty speaking Mild joint or muscle tenderness Fatigue Shortness of breath
  • 12. Signs and symptoms affects the muscles closest to the trunk, particularly hips, thighs, shoulders, upper arms and neck. weakness is symmetrical worsens over time. As muscle weakness progresses, difficult to climb stairs, rise from a seated position, lift objects or reach overhead.
  • 13. Complications Dysphagia Which in turn may cause weight loss and malnutrition. Aspiration pneumonia Shortness of breath or respiratory failure. Calcinosis Calcium deposits in muscles, skin and connective tissues
  • 14. 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.
  • 15. DIAGNOSTIC CRITERIA 1. PROXIMAL MOTOR WEAKNESS: symmetric, proximal muscles 2. HIGH SERUM MUSCLE ENZYMES: CPK, aldolase, myoglobin, AST, ALT, LDH
  • 16. 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 abnormalnormalNCV
  • 17. DIAGNOSTIC CRITERIA 4. MUSCLE BIOPSY: biopsy a clinically weak muscle, contralateral to an abnormal muscle ( by EDT), MRI directed. a. Perivascular and endomysial inflammation CD8+ T cells in PM, CD8+, CD4+ T and B cells in DM b. b. Muscle fiber necrosis and regeneration
  • 18. LABORATORY TESTS HIGH MUSCLE ENZYMES:- CPK ELEVATED ESR , CRP:- 50% POSITIVE ANA:- 50-80% AUTOANTIBODIES:- anti- RNP (MCTD) anti-PM/Scl (OVERLAP)
  • 19. 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 particle In 5% of PM ANTI Mi-2 in 10% of DM.
  • 20. Treatment 1. STEROIDS 2. IMMUNOSUPPRESSIVE AGENTS: methotrexate, azathioprine, cytoxan, cellcept 3. IMMUNOMODULATORY AGENTS: IVIG, Plasmapheresis 4. REHABILITATION
  • 21. Cytokines in Inflammation Pro-inflammatory Anti-inflammatory TNFa IL-1b sTNFR IL-10 IL-1Ra
  • 22. Macrophage or Activated T Cell Soluble TNFa Target Cell TNFa Receptor Signal Induction Synthesis and Function of TNFa Transmembrane TNFa Receptor-Bound TNFa
  • 23. 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 blockade Activation of anti-inflammatory pathways Cytokine receptor Soluble receptor Monoclonal antibody Monoclonal antibody Receptor antagonist Anti-inflammatory cytokine Suppression of inflammatory cytokines No signal No signal Inflammatory signal
  • 24. Humira- The first fully human antiTNFa Chimeric Antibody 70% Human Humanized Antibody 95% Human Fully Human Antibody 100% Human Mouse Human Chimeric Humanized Fully Human 24
  • 25. Treatment corticosteroid When muscle strength improves, usually in 4 to 6 weeks, the medication is slowly tapered off. Maintenance therapy with prednisone may be continued indefinitely. DMARDs - If unresponsive to corticosteroids methotrexate and azathioprine,
  • 26. Treatment Intravenous gamma globulin IVIG is 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
  • 27. Other immunosuppressive medicine Tacrolimus (Prograf) is a transplant-rejection drug that may work to inhibit the immune system. Immunosuppressants, cyclophosphamide (Cytoxan) and cyclosporine (Gengraf, Neoral, Sandimmune),
  • 28. Biological therapies Rituximab (Rituxan) Tumor necrosis factor (TNF) inhibitors etanercept (Enbrel) and infliximab (Remicade),
  • 29. Nursing management Coping and support Educate about the illness Balance Rest and exercise
  • 30. Myositis associated with skin diseases
  • 31. 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
  • 32. Symptoms Dysphagia Muscle weakness, stiffness, or soreness Purple or violet colored upper eyelids Purple-red skin (violaceous) rash SOB Maria Carmela L. Domocmat, RN, MSN
  • 33. 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
  • 34. 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
  • 35. purple (violaceous) plaques The appearance of purple (violaceous) plaques on the knees may be associated with dermatomyositis. Maria Carmela L. Domocmat, RN, MSN
  • 36. Gottron's sign Red, thickened, scaly skin over the knuckles Maria Carmela L. Domocmat, RN, MSN
  • 37. 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
  • 38. violet-colored inflammation (erythema) over the knuckles Maria Carmela L. Domocmat, RN, MSN
  • 39. periungual erythema Candida paronychia produced periungual erythema, edema and nail fold maceration. Maria Carmela L. Domocmat, RN, MSN
  • 40. Dx Exams CPK & aldolase ECG Electromyography Magnetic resonance imaging (MRI) Muscle biopsy Maria Carmela L. Domocmat, RN, MSN
  • 41. Treatment Corticosteroids Immunosuppressants When muscle strength gets better – taper off corticos However, most people take prednisone indefinitely. 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
  • 42. Outlook (Prognosis) Some recover and have symptoms completely disappear - 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
  • 43. Possible Complications Acute renal failure Cancer (malignancy) Inflammation of the heart Joint pain Lung disease Maria Carmela L. Domocmat, RN, MSN
  • 44. DIFFERENTIAL DIAGNOSIS -MYOPATHY- DRUG/TOXIN -NEURO-MUSCULAR -ENDOCRINE DISEASE -INFECTIOUS MYOSITIS -METABOLIC STORAGE MYOPATHIES -MITOCHONDRIAL MYOPATHY -OTHERS
  • 45. 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
  • 46. Steroid response prognosisHLAClinical association prevalenceAb’s moderatemoderateDR3Anti- synthetase syndrome 20-50% (PM) Anti- Jo-1 poorbad (cardiac) DR5Severe PM5% (PM) Anti- SRP goodgoodDR7Classical DM 5-10% (DM) Anti- Mi-2 Myositis-specific AUTOANTIBODIES
  • 47. 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 lymphoma Routine screening in DM

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