Polymyositis ( PM ) is a type of chronic inflammation of the muscles (myopathic inflammation) associated with dermatomyositis and myositis inclusion of the body. Its name means "inflammation of many muscles" ( poly - myos - <-> ). Inflammation is dominated from endomisium in polymyositis, whereas dermatomyositis is characterized by perimysial inflammation.
Video Polymyositis
Signs and symptoms
Characteristic of polymyositis is weakness and/or loss of muscle mass in proximal muscle, and flexion of neck and torso. These symptoms can be attributed to marked pain in these areas as well. The hip extensors are often severely affected, leading to certain difficulties in climbing stairs and up from a sitting position. Dermatomyositis skin involvement is absent in polymyositis. Dysphagia (difficulty swallowing) or other problems with esophageal motility occur in as many as 1/3 patients. Low grade fever and peripheral adenopathy may be present. Foot drop on one or both legs can be a symptom of advanced polymyositis and myositis body inclusions. The systemic involvement of polymyositis includes interstitial lung disease (ILD) and heart disease, such as heart failure and conduction abnormalities.
Polymyositis tends to become apparent in adulthood, with bilateral proximal muscle weakness often noted in the upper leg due to premature fatigue while walking. Sometimes weakness arises as an inability to rise from a sitting position without the help or inability to raise a person's hand over a person's head. This weakness is generally progressive, accompanied by lymphocytic inflammation (especially cytotoxic T cells).
Related diseases
Polymyositis and associated inflammatory myopathy have an increased risk of malignancy. The features they found associated with an increased risk of cancer were older age, age over 45 years, male gender, dysphagia, skin necrosis, skin vasculitis, rapid onset myositis (& lt; 4 weeks), increased creatine kinase, rate higher blood sediment. and higher levels of C-reactive protein. Several factors were associated with a lower-than-average risk, including the presence of ILD, arthritis/arthralgia, Raynaud's syndrome, or anti-Jo-1 antibodies. Associated malignancies are nasopharyngeal cancer, lung cancer, non-Hodgkin's lymphoma and bladder cancer, among others.
Cardiac involvement usually manifests itself as heart failure, and is present in up to 77% of patients. Interstitial lung disease is present in up to 65% of patients with polymyositis, as defined by HRCT or ILS-compatible restrictive vent defective defects.
Maps Polymyositis
Cause
Polymyositis is an inflammatory myopathy mediated by cytotoxic T cells with an unknown autoantigen, while dermatomyositis is a humor-mediated angiopathy that produces typical myositis and dermatitis.
The cause of polymyositis is unknown and may involve viruses and autoimmune factors. Cancer can trigger polymyositis and dermatomyositis, possibly through an immune response to cancer that also attacks the muscle component. There is tentative evidence of association with celiac disease.
Diagnosis
The diagnosis is fourfold: History and physical examination, increased creatine kinase, electromyographic changes (EMG), and positive muscle biopsy.
The clinical characteristic of polymyositis is proximal muscle weakness, with less important findings being muscle pain and dysphagia. Heart and lung findings will be present in approximately 25% of cases of patients with polymyositis.
Myositis sporadic inclusion bodies (sIBM): IBM is often confused with (incorrectly diagnosed as) polymyositis or dermatomyositis that does not respond to treatments likely to be IBM. sIBM comes in several months to years; polymyositis comes in weeks to months. Polymyositis tends to respond well to treatment, at least initially; IBM does not.
Treatment
First-line treatment for polymyositis is corticosteroids. Specialized exercise therapy can complement treatments to improve quality of life.
Epidemiology
Polymyositis, like dermatomyositis, attacks women with a greater frequency than men.
Famous cases
- And Christensen, an abstract art painter. Died of heart failure caused by polymyositis.
- Robert Erickson, American composer and teacher who is a prominent modernist exponent of the "12-tone" composition. Died due to polymyositis effect.
- David Lean, film director.
See also
- limb girdle syndrome
References
External links
- Asosiasi Myositis
Source of the article : Wikipedia
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