Course of dermatomyositis (DM) can be complicated by multi organ failure, by complex pathophysiological mech-anisms 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 dermato-myositis (CADM) has stronger association with ILD as compared to classic form of DM. ILD can be in the form of fibros-ing 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, di-agnosed 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 multisys-tem 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. Keywords: Amyopathic dermatomyositis, myositis, macrophage activation syndrome, right ventricular thrombus, thromboembolism in dermatomyositis
Corresponding Author: Paul N.