[Polymyositis/dermatomyositis--clinical picture and treatment]. 2012

Branimir Anić, and Mislav Cerovec
Zavod za klinicku imunologiju i reumatologiju, Klinika za unutarnje bolesti, Klinicki bolnicki centar Zagreb, Kispatićeva 12, 10000 Zagreb.

The clinical presentation ofmyositis ranges from a painless muscle weakness to significant myalgia with muscle weakness and constitutional symptoms. Along with muscle and skin affection and constitutional symptoms, the disease can affect lungs, joints, heart and gastrointestinal system. It is important to note that the clinical presentation ofmyositis syndrome may overlap with symptoms of other connective tissue disease in overlap syndromes (SLE, SSCL, RA, SSjö). Common manifestations of the disease are weakness and muscle fatigue, which is the result of skeletal muscles inflammation (usually the proximal group of muscles, bilaterally and symmetrical). Severe forms of the disease with affection of the throat and respiratory muscles can vitally endan- ger patients. Among constitutional (general) symptoms, fever, malaise and weight loss are usually expressed. Skin affection in dermatomyositis can be localized or generalized like vesiculobullous erythroderma. Pathognomonic cutaneous manifestations of dermatomyositis are Gottron's papules and heliotrope erythema. Lungs are most commonly affected organs (with exception of muscles and skin) in polymyositis and dermatomyositis. The affection of lung can sometimes result in fatal outcome (interstitial lung disease, secondary pulmonary hypertension). Cardiac affection is usually subclinical, but can also be expressed as heart failure, acute coronary syndrome or conduction disturbances. Infrequent manifestations of the disease are gastroesophageal reflux, malabsorption, gastrointestinal mucosal ulceration, soft tissue calcification, Raynaud's syndrome, arthralgia/arthritis and some other less common clinical manifestations of the disease. Treatment of polymyositis/dermatomyositis includes immunosuppressive/immunomodulatory therapy and supportive, symptomatic treatment. The basis for myositis treatment are glucocorticoids, which are applied orally in a daily dosage regimen of 0.75 to 1 mg/kg/day, and in severe forms of the disease in the i.v. pulse doses of 1 g/day. Immunosuppressants/immunomodulators are added in the therapy along with glucocorticoids for better control of the disease and to reduce the required dose of glucocorticoids (side effects of longterm high doses glucocorticoide use). The most commonly used immunosuppressive drug is methotrexate at a dose of up to 25 mg/week. Hydroxychloroquine has a good effect on the cutaneous manifestations of the disease. Among other immunosuppressants which are used in the treatment of myositis are azathioprine, cyclosporine (in patients with pulmonary affection), mycophenolate mofetil and tacrolimus. Intravenous immunoglobulins applied parenterally in a dose of 2 g/kg divided into multiple doses showed an excellent clinical effect in patients with affection of the esophagus and throat muscles, in patients with pulmonary affection and in patients with resistant disease. The experience with the biologics is limited to a small number of patients. Physiotherapy is a necessary form of treatment for the recovery of muscle strength in the remission phase of the disease. A prompt treatment of infections and heart failure is sometimes life-saving in patients with myositis. Symptomatic treatment of pain with analgesics and NSAIDs reduces pain, speeds up recovery and improves the quality of life in patients with myositis.

UI MeSH Term Description Entries
D003882 Dermatomyositis A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. The illness occurs with approximately equal frequency in children and adults. The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. The childhood form of this disease tends to evolve into a systemic vasculitis. Dermatomyositis may occur in association with malignant neoplasms. (From Adams et al., Principles of Neurology, 6th ed, pp1405-6) Polymyositis-Dermatomyositis,Dermatomyositis, Adult Type,Dermatomyositis, Childhood Type,Dermatopolymyositis,Juvenile Dermatomyositis,Juvenile Myositis,Adult Type Dermatomyositis,Childhood Type Dermatomyositis,Dermatomyositis, Juvenile,Myositis, Juvenile,Polymyositis Dermatomyositis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D017285 Polymyositis Diseases characterized by inflammation involving multiple muscles. This may occur as an acute or chronic condition associated with medication toxicity (DRUG TOXICITY); CONNECTIVE TISSUE DISEASES; infections; malignant NEOPLASMS; and other disorders. The term polymyositis is frequently used to refer to a specific clinical entity characterized by subacute or slowly progressing symmetrical weakness primarily affecting the proximal limb and trunk muscles. The illness may occur at any age, but is most frequent in the fourth to sixth decade of life. Weakness of pharyngeal and laryngeal muscles, interstitial lung disease, and inflammation of the myocardium may also occur. Muscle biopsy reveals widespread destruction of segments of muscle fibers and an inflammatory cellular response. (Adams et al., Principles of Neurology, 6th ed, pp1404-9) Myositis, Multiple,Polymyositis Ossificans,Polymyositis, Idiopathic,Idiopathic Polymyositides,Idiopathic Polymyositis,Multiple Myositis,Myositides, Multiple,Ossificans, Polymyositis,Polymyositides,Polymyositides, Idiopathic

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