Polymyositis or proximal muscle weakness |
- Polymyositis is the inflammation (i.e., irritation, pain, swelling, and damage) of the muscles closest or proximal to the trunk of the body (i.e., limb girdles, neck, and pharynx). It is a progressive condition, with periods of relapse (gets worse) and remissions (gets better.)
- There is an adult form of Polymyositis in those between 40-60 years old, and a childhood form seen in those between ages 5-15. Polymyositis can also occur with other inflammatory type diseases (e.g., Rheumatoid Arthritis, mixed connective tissue diseases, and dermatomyositis) as well with some malignancies.
- First, may notice muscle aches and pain
- Weakness and fatigue
- Often affects arms and legs
- Heliotrope rash may be seen around the eyes, knuckles, and nail beds
- Swelling around the eyes
- Progressive difficulty getting up from the chair or bed
- Difficulty with climbing stairs
- Difficulty raising arms -- e.g., combing hair, brushing teeth.
- Difficulty kneeling
- Muscle and joint pain, stiffness, and tenderness
- Trouble swallowing
- Breathing difficulties
- Heart problems -- swollen ankles,
shortness of breath, fatigue, irregular heart rate
- Unknown (not certain)
- Possible causes:
- Viral (virus) Infections such as HTLV1
- Autoimmune -- body's natural defenses (white blood cells and antibody Proteins) attack the muscles for unknown reasons
- There may be an abnormal inherited gene involved.
- Family history
- Medical exam will demonstrate the presence of heliotrope rash and Proximal Muscle Weakness (arms and legs).
- The doctor may refer patient to a neurologist (muscle and nerve doctor) or a rheumatologist (muscle and joints doctor).
- Often tests are needed for diagnosis:
- Blood tests for evidence of muscle inflammation and damage (myositis) -- Creatinine kinase levels (a muscle chemical) are elevated
- Blood levels of other chemicals (enzymes) such as SGOT, LDH, Aldolase, and ESR (elevated in inflammation) may also be elevated.
- Blood may test positive for RF (rheumatoid antibody), ANA (lupus antibody), myositis, and other antibodies (body Proteins that fight off foreign invaders).
- Blood tests may show anemia (low blood) and increased number of white blood cells.
- A urine sample may show Myoglobin, a muscle pigment released when the muscle is damaged.
- Chest X-Ray may show changes in the lungs known as interstitial fibrosis.
- EKG -- measures electrical activity of the heart -- done if Arrhythmias (irregular heart rhythm) is suspected.
- EMG -- measures muscle electrical activity -- abnormal in Polymyositis.
- Muscle biopsy may show characteristic changes consistent with Polymyositis.
- Muscle biopsy -- surgically taking a tiny piece of the affected muscle and looking at it under a regular or even an electron (very powerful) microscope.
- Female > Male (2:1 ratio)
- Family history of Polymyositis, vasculitis, or other autoimmune disorders
- Dermatomyositis --Polymyositis with a purplish skin rash (heliotrope)
- Bacterial, viral, and parasitic infections
- Genetic -- association with HLA - DR3, HLA - DRw52 genes
- Certain drugs and medications:
- Alcohol -- ethanol
- Vaccine injury
- Usually outpatient
- Involves reducing inflammation with drugs, such as:
- Cyclosporin A.
- Other medications or combinations can be added.
- Intravenous (via blood) immunoglobulins (antibodies) are added to Prednisone therapy in some instances to enhance the effect in resistant cases.
- A search for cancer is recommended in all adults with Polymyositis.
- Those with thin bones, as demonstrated by a bone mineral density test, need to be closely observed while taking steroids (Prednisone, etc.), and supplemented with bone-building medications, Calcium, and vitamins.
- Exercise and physical therapy is recommended to prevent joint damage and keep up strength.
- Psychiatric counseling may be needed if the patient is depressed.
- Cardiologist (heart doctor) or a pulmonologist (lung doctor) may be consulted as needed.
Contact your physician. Ask about new treatments, ongoing research protocols, or clinical trials.
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