What is the cause and treatment for polymyositis?
One of your neurologists Dr. XXXXXXX gave me a scant answer which I would have known after different doctors have seen me. If you have something more novel, I would like to hear it.
Thanks for your question.
Myositis varies tremendously from patient to patient, and no one treatment works for everyone. Your physician may use a combination of drugs to treat you, or change medications over time. It is extremely important that you communicate well with your doctor about your treatment and any side effects.
An important part of your treatment will be the regular practice of an exercise program that works for you, no matter what your level of ability or weakness. Talk with your physician about supplements that are sometimes recommended for muscle strength.
While there is not yet a cure for myositis, for some patients, treatments can effectively control and improve symptoms.
Because there are many different symptoms and possible complications, few controlled patient trials and a wide range of reactions to medicines, each person's treatment for myositis is individual. There are commonly used treatment regimens based on experience and case reports:
Corticosteroids: Prednisone, a corticosteroid medicine, is commonly used as a first choice treatment with fairly fast results in polymyositis, juvenile myositis and dermatomyositis. Corticosteroids slow the body's immune system and stop the inflammatory attack on muscle, skin and other body systems. These medicines control the inflammation, ease pain, and increase muscle strength.
The specific dose varies from patient to patient, but we tend to prescribe relatively high doses and decrease the dose slowly as the symptoms improve. Doses depend your weight and disease severity. Prednisone can be given orally (as a pill) or intravenously (methylprednisolone given through a needle into a vein). Often, we begin patients on high-dose intravenous corticosteroids followed by regular oral or intravenous doses, although a recent study showed that oral prednisone is just as effective and has less complications than intravenous prednisone. We closely monitor patients for possible side effects. Some side effects are brittle bones (osteoporosis), cataracts, stomach upset, weight gain, mood swings, and changes in blood sugar. To reduce these side effects, we try to taper the dose as quickly as possible while still effectively combating the symptoms. We are increasingly introducing other medicines to allow for a more successful steroid taper.
We sometimes recommend taking corticosteroids every other day rather than every day to lessen the side effects.
Never discontinue or reduce your dose without checking with your physician. Tapering or slowly lowering your dose of corticosteroids is essential to allow your body to begin cortisol production on its own.
Immunosuppressants: Methotrexate and azathioprine are often introduced as secondline medicines or used in combination with prednisone. This allows patients to taper off prednisone more quickly and avoid some of the unwanted effects. Both methotrexate and azathioprine are well-tolerated, but methotrexate tends to work faster. Methotrexate is given orally (as a pill), intravenously or subcutaneously (injected under the skin). Azathioprine is taken orally (by mouth). More and more doctors now treat patients with a combination of methotrexate and prednisone right from the start.
Cyclophosphamide (oral or intravenous) and cyclosporine (oral) are more commonly used in cases where patients exhibit lung complications like interstitial lung disease (ILD) as these are more potent immunosuppressants.
Which immunosuppressants a particular doctor chooses depends on a number of factors, including the patient's response to corticosteroids, medical history and disease severity, as well as the doctor's past experience and personal preference.
Side effects include nausea, cough, fatigue, fever, back pain, abdominal pain, kidney problems, hair loss, and delayed-onset cancers.
Acthar is a preparation of ACTH in 16% gelatin formulation which is used either subcutaneously or intramuscularly in the treatment of polymyositis and dermatomyositis. Acthar is the only FDA approved drug for PM and DM other than corticosteroids. It is a naturally occurring hormone produced in the pituitary gland and has several potential mechanisms as to how it may benefit patients with PM or DM. ACTH causes the release of cortisol from a patient's adrenal glands, thus mimicking the effects of taking corticosteroids by mouth. ACTH also interacts at a number of receptors throughout the immune system and may reduce immune overactivity responsible for causing PM and DM. Although Acthar has FDA approval for treating PM and DM, there is very limited data on the effectiveness of the therapy. Ongoing studies are being performed to evaluate the role for Acthar in the treatment of PM and DM.
Intravenous immune globulin (IVIG): IVIG is a blood product derived from large pools of donated human plasma. IVIG boosts the body's immune system response, and doctors don't know the exact reasons IVIG works in some myositis patients. IVIG is usually reserved for cases resistant to other treatments, and people with inclusion-body myositis typically do not benefit from IVIG unless they have accompanying swallowing problems.
IVIG is given slowly, and the dose depends on the patient's weight. Side effects include backache, headache, fever, chills, general discomfort, and joint pain. Meningitis is possible. Side effects can be related to the rate of infusion, and often slowing this rate relieves unwanted effects. Premedication with Benadryl or Tylenol can also prevent some of these side effects.
IVIG is expensive and typically requires repeated doses.
Biologic agents: Biologic agents inhibit cytokines, key players in myositis inflammation. Most of these agents are monoclonal antibodies, or proteins targeting tumor necrosis factor-alpha, B cells and T cells. These medicines provide a more targeted therapy now being studied in clinical trials for myositis.
Anti-TNF agents suppress tumor necrosis factor proteins that are associated with inflammation and include etanercept (Enbrel), adalimumab (Humira) and infliximab (Remicade). Etanercept and adalimumab are subcutaneous injections; infliximab, is an intravenous injection.
Rituximab (Rituxan) targets B cells, or lymphocytes of the immune system that produce antibodies that attach to a specific antigen to help destroy it. B cells play a role in the inflammation of myositis. Rituximab is given intravenously. A large, mutli-center study of rituximab in myositis (RIM) was statistically inconclusive, but found that rituximab shows promise for the treatment of myositis.
Alemtuzumab (Campath 1H) is directed against T cells, another type of lymphocyte that attacks antigens directly. Alemtuzumab is given intravenously and is still restricted to research study participants.
Since these agents are fairly new in treating myositis, doctors monitor patients closely for any side effects. Some reported include abdominal pain, diarrhea, loss of energy, chills, dizziness, frequent urination, and headache.
These treatments are expensive and often are not covered by insurance.
Many doctors recommend a holistic approach to treatment,keeping in mind nutrition, appropriate exercise, physical therapy, sunscreen, and other complements to the conventional medicines listed above.
I hope this helps answer your question.
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