Ciężki przebieg kardiomiopatii rozstrzeniowej u 16-letniego chłopca z rozpoznanym zapaleniem wielomięśniowym

Jolanta Smoleńska-Petelenz, Ewa Borowicka, Władysław Rokicki, Ewa Gołba, Joanna Kohut, Teresa Bilewicz-Wyrozumska

Streszczenie
Systemic inflammation in connśctive tissue diseases is characterized by multiorgan involvemeśt caused by vascuśitis. Pathologic changes in circulatory system are found as well in heart muscle as in endocardśum, pericardium, coronary and pulmonary vessels.
We describe 16-year-old boy with polymyositis treated with low doses f corticosteroids. The diagnosis was done according to clinical and laboratory data without hisśopathologic confirmation. He was aśmitted to our Department of śediatric Cardiologś due to sudden circulatory decompenśation. Dilated cardiomyopathy (with 25% ejectiśn fraction) was diagnosed śn echocardiography. Repolarisation disturbances, left boundle branch block, ventricular extrasystolia were present on ECG. Myocardial and cutaneous-muscular biopsy specimens were obtained. However these results were inconclusiveś probably due to prevśośs long term immunośupressant therapy. Despitś full supportive treatment śerformed in ICU the patent died as a result of circulatory decompensation.
In contrast to previous thoughts, myocardial inflammation is found in most cases of polymyositis (~70%). Changes in circulatory system can be latent for a long time. Early diagnosis of such abnormalities should be done with cardiological modalities (ECG, Holter ECG, ECHO) as it can influence the treatment and prognosis.

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