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Introduction Systemic sclerosis (SSc) is a heterogeneous autoimmune disease characterized by vasculopathy, autoimmunity, and progressive fibrosis. Cardiac involvement is rare and under-recognized, but serious complications are associated with increased morbidity and mortality. Overlap syndromes with idiopathic inflammatory myopathies (IIM) further complicate disease course and therapeutic decision-making. Herein, we report a rare case of diffuse cutaneous systemic sclerosis (dcSSc) overlapping necrotizing autoimmune myopathy (NAM) with acute myopericarditis and impending cardiac tamponade. This case emphasizes the diagnostic complexity, the need for tailored immunosuppressive strategies, and the critical importance of multidisciplinary collaboration.Material and Methods A 60-year-old female presented with generalized edema, puffy hands, and progressive skin thickening above the elbows and knees over 4 months. Physical exam showed a tendon friction rub, and the Modified Rodnan Skin Score (mRSS) was 28. Workup revealed positive anti-topoisomerase I with organ involvement including esophageal dysmotility, myositis, and suspected peri-myocarditis. The initial echocardiogram showed a preserved ejection fraction (EF) of 84% and a moderate pericardial effusion without tamponade physiology. The patient developed proximal muscle weakness and elevated Creatine kinase. Electromyography was consistent with inflammatory myopathy, and biopsy confirmed necrotizing myositis. Despite intravenous cyclophosphamide for suspected acute peri-myocarditis, the cardiac biomarker continued to rise. Prednisolone 10 mg per day and rituximab 1,000 mg intravenous, two doses were initiated with subsequent biomarker improvement. However, cardiac MRI later revealed a large pericardial effusion with RA/RV diastolic collapse, indicating impending tamponade. An emergency pleuro-pericardial window was performed. Histopathology showed fibrotic pericardial tissue with eosinophils and fibrin, but no active infection or granuloma.Results Following Rituximab and subsequent initiation of mycophenolate mofetil (MMF), there was marked clinical improvement. Troponin T levels declined, creatine kinase and inflammatory markers gradually normalized, and pleural as well as pericardial effusions decreased after surgical drainage and immunosuppressive therapy, with no evidence of scleroderma renal crisis.Conclusions A rare case of dcSSc with overlap of NAM and peri-myocarditis progressing to impending tamponade highlights the need for early recognition, steroid-sparing immunosuppressive therapy, and timely surgery. Multidisciplinary care was crucial for patient survival.Abstract P.151 Figure 1EKG showed sinus tachycardia and low voltageAbstract P.151 Figure 2A) abnormally high SI on T2WI of all segments of LV myocardium; B) diffuse patchy subepicardial and mid-wall abnormal SI in T1WI suggestive of myocardial hyperemia; C and D) the T2 SI on quantitative T2 mapping images is increased, suggestive of myocardial edema; E) diffuse patchy subepicardial and mid-wall myocardial enhancement on late gadolinium enhancement sequence (LGE) involving all segments of LV myocardium with a large amount of pericardial effusion