Interstitial Lung Disease in Dermatomyositis Complicated by Right Ventricular Thrombus Secondary to Macrophage Activation Syndrome

Özet

Course of dermatomyositis (DM) can be complicated by multi organ failure, by complex pathophysiological mechanisms involving auto antibodies. Pulmonary complications are the leading cause of mortality, accounting for 30% deaths. There is a strong association between DM and interstitial lung disease (ILD); clinically amyopathic dermatomyositis (CADM) has stronger association with ILD as compared to classic form of DM. ILD can be in the form of fibrosing alveolitis, interstitial pneumonia and desquamative interstitial pneumonia or diffuse alveolar hemorrhage. Auto antibodies linked to ILD are anti Jo-1, PL12, PL7, EJ, and OJ and anti Mi 2. Our case describes a fifty three years old woman who presented with symptoms of lower respiratory tract infection, diagnosed with CADM on the basis of typical skin rash and polyarthritis and anti-CADM 140 antibodies. Systemic steroid therapy (initiated after ruling out sepsis) failed to provide improvement. Medical course was complicated by multisystem involvement (respiratory failure, cerebral edema, renal failure, coagulopathy, hepatic failure and thromboembolism). Transthoracic echocardiogram revealed thrombus in right ventricle which was the result of prothrombotic state.