Yuliarni Syafrita Bagian Neurolog FK Unand. Polymyositis is an idiopathic inflammatory myopathy ...
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Transcript of Yuliarni Syafrita Bagian Neurolog FK Unand. Polymyositis is an idiopathic inflammatory myopathy ...
Yuliarni SyafritaBagian Neurolog
FK Unand
Polymyositis is an idiopathic inflammatory myopathy
causes symmetrical, proximal muscle weakness
elevated skeletal muscle enzyme levels
General◦ group of disorders◦ proximal muscle weakness◦ nonsuppurative inflammation skeletal muscle◦ prevalence
5 cases/mil/year 2 to 1 F>M 40-60 yrs pediatric variant 5-15-yrs
Epidemiology Idiopathic inflammatory myopathies are
relatively rare diseases. incidence in the United States that ranges
from 0.5-8.4 cases per million population. more common in the US within the black
population, with the estimated black-to-white incidences for polymyositis and dermatomyositis being 5:1 and 3:1, respectively.
Internationally, polymyositis is less common among Japanese persons.
usually affects adults older than 20 years, especially those aged 45-60 years.
Polymyositis rarely affects children. observed in children, usually those aged 5-
14 years In most patients, polymyositis responds well
to treatment, although residual weakness occurs in approximately 30% of patients
usually present with symmetrical, proximal muscle weakness in the upper and lower extremities.
Patients with polymyositis may report muscle pain and tenderness, which may be confused with symptoms of polymyalgia rheumatica.
all of the muscles usually involved Muscle weakness may fluctuate from week
to week or from month to month.
Dysphagia secondary to oropharyngeal and esophageal involvement occurs in about one third of patients with polymyositis and is a poor prognostic sign.
Facial and bulbar muscle weakness is extremely rare in individuals with polymyositis.
Etiology :• Polymyositis is an immune-mediated
syndrome .• secondary to defective cellular immunity • commonly associated with other systemic
autoimmune diseases
General◦ criteria
proximal muscle weakness elevated serum CPK myopathic changes on EMG muscle biopsy inflammation definitive with four, probable with three, possible
with two dermatomyositis skin rash with above
General◦ labs
anti-tRNA synthetases◦ up to 20% associated with malignancy
lung, ovary, breast, stomach parotid, tonsil reported dermatomyositis with nasopharyngeal carcinoma,
endemic areas
Head and Neck Manifestations◦ weakness neck muscles◦ difficulty phonation, deglutition, nasal
regurgitation◦ dysphagia from upper esophagus◦ may result in aspiration pneumonia
Enzyme levelsSerum creatine kinase (CK) levels are usually
elevated in persons with polymyositis, ranging from 5-50 times the reference range. A level greater than 100 times the reference level is rare and is a signal of other diagnoses.
Other muscle enzymes that may be elevated include the following:
Lactic dehydrogenase Aspartate aminotransferase Alanine aminotransferase Aldolase - In practice, usually only the CK
and aldolase levels are determined
ElectromyographyElectromyographic findings are abnormal in
almost all patients (90%) with polymyositis. Various abnormalities consistent with polymyositis may be found, depending on the stage of disease
Biopsy Muscle biopsy is crucial in helping to
diagnose polymyositis and in excluding other rare muscle diseases
Inflammatory changes are seen at muscle biopsy (eg, deltoid or quadriceps femoris).
Treatment◦ steroids if symptomatic◦ methotrexate, immunosuppresives nonresponders◦ H2 blockers◦ metoclopromide
Corticosteroids Prednisone is the first-line treatment of
choice for polymyositis. Typically, the dose is 1 mg/kg/day, either as a single or divided dose. This high dose is usually continued for 4-8 weeks, until the CK level returns to reference ranges. Taper prednisone by 5-10 mg on a monthly basis until the lowest dose that controls the disease is reached.
Monitor response to therapy based on improvement in muscle strength and muscle endurance and decrease in CK levels.
Immunosuppressants Immunosuppressive agents are indicated in
patients who do not improve with steroids within a reasonable period (ie, 4 wk) or in whom adverse effects from corticosteroids develop
Patients with poor prognostic indicators, such as dysphagia or dysphonia, are likely to require immunosuppressive agents.
Other agents Intravenous immunoglobulin (IVIG) has
been used for the short-term treatment of steroid-resistant cases of polymyositis.
Diet Patients with polymyositis may benefit from
a high-protein diet. Monitor patients to avoid excessive weight gain due to corticosteroid use.
Activity During the acute stage of polymyositis,
patients may benefit from heat therapy, passive range-of-motion exercises, and splints to avoid contractures.
Etiologi : Pemakaian kortiko steroid Gangguan metabolik (hipoglikemi kronik,
myiglobinuria miopatik, penyakit penimbunan glikogen)
Gangguan endokrin (tirotoksikosis, sindroma cushing, penyakit addison)