IDIOPATHIC INFLAMMATORY

MYOPATHY
 IDIOPATHIC INFLAMMATORY MYOPATHY
 Inflammatory myopathy (inflammatory muscle disease or myositis) is disease
featuring weakness and inflammation of muscles and (in some types) muscle
pain. The cause of much inflammatory myopathy is unknown (idiopathic), and
such cases are classified according to
their symptoms and signs and electromyography, MRI and laboratory findings. It
can also be associated with underlying cancer. The main classes of idiopathic
inflammatory myopathy are polymyositis (PM), dermatomyositis (DM),
and inclusion-body myositis (IBM).

 It is an inflammatory disease of the muscle are a heterogeneous group of

disorders characterized by proximal muscle weakness and non-suppurative
inflammation of the skeletal muscle.

 Although the condition can be diagnosed at any age, idiopathic inflammatory

myopathy most commonly occurs in adults between ages 40 and 60 years or in
children between ages 5 and 15 years.
 It can also be associated with underlying cancer. The main classes
of idiopathic inflammatory myopathy are polymyositis (PM),
dermatomyositis (DM), and inclusion- body myositis (IBM).
 The cause of inflammatory myopathy is unknown (idiopathic), and
such cases are classified according to their signs and symptoms and
electromyography, MRI and laboratory findings.

 The primary symptom of idiopathic inflammatory myopathy is

muscle weakness, which develops gradually over a period of weeks
to months or even years. Other symptoms include joint pain and
general tiredness (fatigue)

 Myopathy is a muscle disease unrelated to any disorder of

innervation or neuromuscular junction. Etiologies vary widely.
 ETIOLOGY
 Idiopathic myopathies are thought to result from immune-mediated
phenomena including sarcoidosis with myopathy, polymyositis and
dermatomyositis. Some IM are associated with connective tissue disease,
systemic lupus, rheumatoid arthritis.
Infectious causes include the following:
 Trichinosis
 Cysticercosis (taenia solium)
 Toxoplasmosis
 Human immunodeficiency virus
 Influenza
 Staphylococcus aureus muscle infection (frequent cause of polymyositis)
 WHAT ARE MYOPATHIES?
 Myopathy is the medical term for muscle disease. Some muscle diseases
occur when the body’s immune system attacks muscles. The results is
misdirected inflammation, hence the name inflammatory myopathies. This
damages muscle tissue and makes muscle weak.
People with IM may have these features:
 Weakness in the large muscles around neck, shoulders and hips
 Trouble climbing stairs, getting up from a seat, or reaching for objects
overhead
 Choking while eating or aspiration (intake) of food into the lungs
 Shortness of breath and cough
Muscle inflammation and weakness occur in both conditions while patients
with dermatomyositis also have a rash. This rash most often appears as purple
or red spots on the upper eyelids or as scaly, red bumps over the knuckles,
elbows and knees. Children with the disease in the skin called CALCINOSIS.
WHAT CAUSES MYOPATHIES AND WHO GETS THEM?
 There are many causes of muscle disease other than inflammation. They
include infection, muscle injury due to medicine, inherited diseases that
affect muscle function, disorders of electrolyte levels and thyroid disease. It
is unknown what causes inflammatory myopathies. A top theory is that
something goes wrong in the immune system, which leads to attack of the
“self” and muscle inflammation.
 Polymyositis and dermatomyositis occur in about one person per 100,000.
 All ages can get these diseases.
 Weakness in the large muscles around neck, shoulders and hips.
 Myopathy almost always causes loss of muscle strength.
 Some patients have rashes or breathing problems.
 SIGNS AND SYMPTOMS
 Common signs & symptoms are muscle weakness, impaired function in
activities of daily life and rarely , muscle pain and tenderness without
weakness should consideration of other causes.
 Malaise, fatigue
 Symmetric proximal muscle weakness
 Dark-colored urine/ fever
 Normal level of consciousness
 Gottron papules in dermatomyopathies: pink to violaceaous scaly over
areas over knuckles, elbows and knees
Laboratory Testing
 Creatinine kinase levels with isoenzymes
 Level of electrolytes, calcium and magnesium
 Serum creatinine and blood urea nitrogen levels
 Complete blood count
 HOW ARE MYOPATHIES DIAGNOSED?
 A doctor suspects myopathy when patients complain of trouble doing
tasks that require muscle strength or when they get certain rashes or
breathing problems. Most people with myopathy have little or no pain
in their muscles. A doctor will do muscle strength exam to find if true
muscle weakness is present. The following test may then be needed?
 A blood test to measure the level of various muscle enzymes and
myositis-specific antibodies
 An electromyogram- often referred to as an EMG- to gauge electrical
activity in muscle
 A biopsy of a weak muscle (a small piece of muscle tissue is removed
for testing)
 Magnetic resonance imaging- more often called MRI to try to show
abnormal muscle.
 Classification and diagnosis
 There are a number of known causes of myopathy, and it is only once
these have been ruled out that a clinician will assign an idiopathic
inflammatory myopathy (IIM) syndrome to a case. The usual criteria for a
diagnosis of PM are weakness in muscles of the head, neck, trunk, upper
arms or upper legs; raised blood serum concentrations of
some muscle enzymes such as creatine kinase; unhealthy muscle changes
on electromyography; and biopsy findings of muscle cell degeneration
and regeneration and chronic inflammatory infiltrates in muscle cells. If
heliotrope (purple) rash or Gottron's papules are also present, then the
diagnosis is DM. In DM, myositis may not be clinically apparent but
detectable via biopsy or MRI. If the criteria for PM are met but muscle
weakness also affects the hands and feet or is not accompanied by pain
IBM should be suspected, and confirmed when muscle cell biopsy
reveals cytoplasmic vacuoles fringed by basophilic granules and
inflammatory infiltrate comprising mostly CD8 T
lymphocytes and macrophages; and electron microscopy reveals
filamentous inclusions in both cytoplasm and nucleus
 Polymyositis and dermatomyositis
 In severe cases of PM and DM with systemic signs, an initial three to five days on intravenous
corticosteroid (methylprednisolone) may be used; but normally treatment begins with a single
daily (after breakfast) high dose of oral corticosteroid (prednisone). After a month or so the
strength of every second day's dose is very gradually reduced over three to four months, to
minimize the negative effects of the prednisone. When a high dose of prednisone cannot be
reduced without losing muscle strength, or when prednisone is effective but it is producing
significant complications, "steroid sparing" oral immunosuppressants such as azathioprine,
mycophenolate mofetil, methotrexate and cyclosporine, may be used in combination with
reduced prednisone. Some of these steroid sparing drugs can take several months to
demonstrate an effect.[4]

 To minimize side effects, patients on corticosteroids should follow a strict high-protein, low-
carbohydrate, low-salt diet; and with long-term corticosteroid use a daily calcium supplement
and weekly vitamin D supplement (and a weekly dose of Fosamax for postmenopausal women)
should be considered.[4]

 For patients not responding to this approach there is weak evidence supporting the use of
intravenous immunoglobulin, ciclosporin, tacrolimus, mycophenolate mofetil and other agents;
and trials of rituximab have indicated a potential therapeutic effect
 Treatment and Management
 There have been few randomized treatment trials, due to the relative rarity
of inflammatory myopathies. The goal of treatment is improvement
in activities of daily living and muscle strength. Suppression of immune
system activity (immunosuppression) is the treatment strategy. Patients with
PM or DM almost always improve to some degree in response to treatment,
at least initially, and many recover fully with maintenance therapy. (If there
is no initial improvement from treatment of PM or DM, the diagnosis should
be carefully re-examined.) There is no proven effective therapy for IBM, and
most IBM patients will need assistive devices such as a cane, a walking
frame or a wheelchair. The later in life IBM arises, the more aggressive it
appears to be. Such treatments may include the following:
 Physical Therapy
 Bracing
 Surgery
 Supportive: management of airway, breathing, circulation and hydration
 NURSING MANAGEMENT
 The history of the clients receiving high dose steroid
therapy focuses on teaching them about the potential
side effects of long term prednisone. Client should be
aware that they have an increased risk of infection or
they should monitor and report any manifestations such
as low grade fever, chills and joint pains to their primary
caregivers. Other long term side effects include facial
edema, increased appetite and the development of
diabetes mellitus, osteoporosis and avascular necrosis.
THANK YOU FOR LISTENING!
PREPARED BY:
DHANICA MAE D. BANTACULO
SUBMITTED TO:
MRS. PORTIA LAPIDARIO RN,
MAN, MD