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What is the treatment for polymyositis?

Thursday, 24 February 2005
Answered by: Dr. Shirish Kumar
Consultant Haematologist,
Sir Ganga Ram Hospital,
New Delhi
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Q. My father is suffering from polymyositis from the past 5 years and the treatment given to him is pills which contains steroids which is not curing the case but trying to stop it from getting worse. Although we know that this is the standard treatment given but it is having side effects on him and also we fear that after some time it will not be effective. As of now, he takes 10 mg daily but it has been increased to 60 mg. One major problem is that he is not able to breathe normally as some parts of lungs is not functioning since the time he was diagnosed and he coughs up regularly. My question is whether there is any alternative treatment for polymyositis which is having less side effects? If not in allopathic than in other branches of medical (homeopathic or ayurvedic). I am open to any idea which can be beneficial in treatment to this fatal disease. I would be really grateful for that.

A.  The goal of therapy is to improve muscle strength to improve function in activities of daily living. Improvement in strength is usually accompanied by a fall in serum creatine kinase level, but this must be interpreted with caution because most immunosuppressive therapies result in a decrease in the levels of serum muscle enzymes without necessarily improving muscle strength. Consultation with an occupational and rehabilitation therapist may help patients with ambulation by providing necessary equipment. -A swallowing evaluation for dysphagia is recommended. -Proper emotional support is also very important. Of all the treatments that are available, prednisone remains the drug of choice. If treatment with steroids is not successful, other lines of treatment are considered, such as intravenous immunoglobulins (IVIG), antineoplastic agents, and antimetabolites. Corticosteroids: Act as anti-inflammatory and immunosuppressive agents and are the first-line drug for both polymyositis and dermatomyositis. The use of a nonsteroidal immunosuppressive drug is determined by the need for a steroid-sparing effect when: (1) serious complications have developed with steroid use (2) repeated relapses have occurred each time an attempt was made to lower a high steroid dosage (3) prednisone did not improve strength or (4) the patient has a rapidly progressive disease accompanied by severe weakness and respiratory failure. Antineoplastic agents: Inhibit cell growth and proliferation. Cyclophosphamide has shown promising results. The drug may be helpful in a subset of patients with interstitial lung disease. Methotrexate, an antagonist of folate metabolism, has been used frequently despite disappointing results. Immunoglobulins: Improve clinical and immunologic aspects of the disease. May decrease autoantibody production and increase solubilization and removal of immune complexes. IVIG has been shown to be effective in dermatomyositis. Improvement is observed after the first infusion and is evident clearly by the second monthly infusion. If no improvement is observed by second or third dose, then treatment is unlikely to be successful. Diet: Patients on steroid therapy should follow a strict low-salt, low-carbohydrate, and high-protein diet to avoid weight gain and hypertension. Activity: Physical therapy helps to preserve muscle function and prevents disuse atrophy of the weak muscles or joint contractures. The natural history of polymyositis and dermatomyositis is unknown because patients now are almost always treated with steroids. Patients with interstitial lung disease may have a high mortality rate. A number of patients still do not respond adequately to therapies and remain disabled. Acute fulminating disease seems to be more difficult to treat and more resistant to therapies. Patients in whom treatment is initiated soon after the onset of the symptoms have the best prognosis. When the treatment is unsuccessful, the patient should be reevaluated and the muscle biopsy specimen reexamined. A second biopsy may be considered to make sure that the diagnosis is correct. The disorders most commonly mistaken for polymyositis are inclusion-body myositis and sporadic limb-girdle muscular dystrophy, which is suspected when the disease has a slow onset and progression and the muscle biopsy specimen does not show primary inflammatory features. As we do not deal with alternative forms of medicine, it is difficult to comment on their role or efficacy. Yoga always helps by improving the general health of an individual.

A.  The goal of therapy is to improve muscle strength to improve function in activities of daily living. Improvement in strength is usually accompanied by a fall in serum creatine kinase level, but this must be interpreted with caution because most immunosuppressive therapies result in a decrease in the levels of serum muscle enzymes without necessarily improving muscle strength. Consultation with an occupational and rehabilitation therapist may help patients with ambulation by providing necessary equipment. -A swallowing evaluation for dysphagia is recommended. -Proper emotional support is also very important. Of all the treatments that are available, prednisone remains the drug of choice. If treatment with steroids is not successful, other lines of treatment are considered, such as intravenous immunoglobulins (IVIG), antineoplastic agents, and antimetabolites. Corticosteroids: Act as anti-inflammatory and immunosuppressive agents and are the first-line drug for both polymyositis and dermatomyositis. The use of a nonsteroidal immunosuppressive drug is determined by the need for a steroid-sparing effect when: (1) serious complications have developed with steroid use (2) repeated relapses have occurred each time an attempt was made to lower a high steroid dosage (3) prednisone did not improve strength or (4) the patient has a rapidly progressive disease accompanied by severe weakness and respiratory failure. Antineoplastic agents: Inhibit cell growth and proliferation. Cyclophosphamide has shown promising results. The drug may be helpful in a subset of patients with interstitial lung disease. Methotrexate, an antagonist of folate metabolism, has been used frequently despite disappointing results. Immunoglobulins: Improve clinical and immunologic aspects of the disease. May decrease autoantibody production and increase solubilization and removal of immune complexes. IVIG has been shown to be effective in dermatomyositis. Improvement is observed after the first infusion and is evident clearly by the second monthly infusion. If no improvement is observed by second or third dose, then treatment is unlikely to be successful. Diet: Patients on steroid therapy should follow a strict low-salt, low-carbohydrate, and high-protein diet to avoid weight gain and hypertension. Activity: Physical therapy helps to preserve muscle function and prevents disuse atrophy of the weak muscles or joint contractures. The natural history of polymyositis and dermatomyositis is unknown because patients now are almost always treated with steroids. Patients with interstitial lung disease may have a high mortality rate. A number of patients still do not respond adequately to therapies and remain disabled. Acute fulminating disease seems to be more difficult to treat and more resistant to therapies. Patients in whom treatment is initiated soon after the onset of the symptoms have the best prognosis. When the treatment is unsuccessful, the patient should be reevaluated and the muscle biopsy specimen reexamined. A second biopsy may be considered to make sure that the diagnosis is correct. The disorders most commonly mistaken for polymyositis are inclusion-body myositis and sporadic limb-girdle muscular dystrophy, which is suspected when the disease has a slow onset and progression and the muscle biopsy specimen does not show primary inflammatory features. As we do not deal with alternative forms of medicine, it is difficult to comment on their role or efficacy. Yoga always helps by improving the general health of an individual.

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