Interstitial Lung Disease in Dermatomyositis Complicated by Right Ventricular Thrombus Secondary to Macrophage Activation Syndrome D ermatomyositis (DM) is a rare connective tissue disor- der that results from auto immune antibodies by hu- moral immune system activation. Reported incidence of amyopathic DM (46.4 %; 21%) is less as compared to classic DM and has a female preponderance. [1, 2] Prior to the intro- duction of corticosteroids in the treatment of DM, progno- sis was extremely poor with a mortality rate as high as 50 to 61%. [3, 4] Muscle or skin biopsy is required to support the diagnosis, however is no t necessary if there is a typical clin- ical presentation suggestive of DM. Cytokine storm known as Macrophage activation syndrome (MAS) is fatal and un- common complication of DM. This case highlights MAS, which depicts a miscellaneous clinical scenario with multiple diferential diagnoses which is the real challenge and can be extremely tricky to manage if the pathogenesis is not fully understood. We report a fatal case of DM with rapidly progressive ILD which failed to respond to systemic steroids and conse- quently resulted in MAS leading to multi organ failure and thromboembolism (right ventricular thrombus) (RVT). To 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 fbros- ing alveolitis, interstitial pneumonia and desquamative interstitial pneumonia or difuse alveolar hemorrhage. Auto antibodies linked to ILD are anti Jo-1, PL12, PL7, EJ, and OJ and anti Mi 2. Our case describes a ffty 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 Namrata Paul, Rahul Vasudev, Chandra Chandran St. Joseph's Healthcare and Rehab Center Ringgold standard institution - Medicine paterson, New Jersey, United States Abstract DOI: 10.14744/ejmo.2019.53051 EJMO 2019;3(3):219-223 Case Report Cite This Article: Paul N, Vasudev R, Chandran C. Interstitial Lung Disease in Dermatomyositis Complicated by Right Ventric- ular Thrombus Secondary to Macrophage Activation Syndrome. EJMO 2019;3(3):219-223. Address for correspondence: Namrata Paul, MD. St. Joseph's Healthcare and Rehab Center Ringgold Standard institution - Medicine Paterson, New Jersey, United States Phone: +99737543285 E-mail: drnamratapaulmd@gmail.com Submitted Date: January 30, 2019 Accepted Date: April 14, 2019 Available Online Date: August 01, 2019 © Copyright 2019 by Eurasian Journal of Medicine and Oncology - Available online at www.ejmo.org OPEN ACCESS This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.