Polymyositis/dermatomyositis - clinical picture and treatment

Klinička slika miozitisa varira od bezbolne slabosti mišića do izraženih mialgija sa slabostima mišića i konstitucijskim simptomima. Uz afekciju mišića i kože te konstitucijske simptome bolest se može prezentirati afekcijom pluća, zglobova, srca, gastrointestinalnog sustava. Bitno je napomenuti da se klinička slika sindroma miozitisa može preklapati sa simptomima drugih definiranih bolesti vezivnog tkiva u sindromima preklapanja (SLE, SSCl, RA, SSjö). Najčešće manifestacije bolesti su slabost i zamor muskulature koji su posljedica upale skeletne muskulature (najčešće proksimalne skupine, simetrično i bilateralno). Teški oblici bolesti s afekcijom muskulature ždrijela ili respiratorne muskulature vitalno ugrožavaju bolesnika. Od općih, konstitucijskih simptoma najčešće su izraženi febrilitet, opća slabost i gubitak na težini. Kožne promjene dermatomiozitisa mogu biti lokalizirane ili generalizirane poput vezikobulozne eritrodermije. Patognomonične kožne manifestacije u dermatomiozitisu Gottronove papule i heliotropni eritem. Pluća su najčešće zahvaćen nemišićni organ u polimiozitisu i dermatomiozitisu afekcija kojeg može rezultirati i letalnim ishodom (intersticijska bolest pluća, sekundarna plućna hipertenzija). Srčane manifestacije najčešće su subkliničke, no mogu biti i izražene poput srčanog popuštanja, akutnog koronarnog sindroma ili pak smetnji provođenja. Rjeđa očitovanja bolesti su gastroezofagealni refluks, malapsorpcija, ulceracije sluznice probavnog sustava, kalcifikacije mekih tkiva, Raynaudov sindrom, artralgije/artritis i ostale rjeđe kliničke prezentacije bolesti. Liječenje polimiozitisa/dermatomiozitisa uključuje osnovnu, imunosupresivnu/imunomodulatornu terapiju i simptomatsko potporno liječenje. Temelj liječenja miozitisa predstavljaju glukokortikoidi koji se primjenjuju peroralno u dnevnom režimu u dozama 0,75-1 mg/kg/dan ili u teškim oblicima bolesti parenteralno u pulsnim dozama od 1 g/dan. Imunosupresivi/imunomodulatori dodaju se glukokortikoidima radi bolje kontrole bolesti te smanjenja potrebne doze glukokortikoida. Najčešće se primjenjuje metotreksat u dozi do 25 mg/tjedan. Hidroksiklorokin u dozi ima dobar učinak na kožne manifestacije bolesti. Od ostalih imunosupresiva primjenjuju se azatioprin, ciklosporin (u bolesnika s plućnom afekcijom), mofetil mikofenolat, takrolimus. Intravenski imunoglobulini primjenjeni parenteralno u dozi od 2 g/kg razdjeljeno u više doza pokazuju odličan učinak u bolesnika s afekcijom muskulature jednjaka i ždrijela, u bolesnika s plućnom afekcijom te onih s rezistentnom bolesti. Za sada su iskustva s biološkom terapijom ograničena na mali broj bolesnika. Fizikalna terapija u fazi remisije bolesti nužan je oblik liječenja u oporavku snage zahvaćene muskulature. Pravovremeno suzbijanje infekcija kao i liječenje srčanog popuštanja ponekad su od vitalne važnosti u bolesnika oboljelih od miozitisa. Simptomatsko liječenje boli analgeticima i nesteroidnim antireumaticima umanjuju tegobe, ubrzavaju oporavak i poboljšavaju kvalitetu života u ovih bolesnika.The clinical presentation of myositis 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 of myositis 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 endanger 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