(251) The Constricting Heart: A Rare Complication of Systemic Sclerosis

Introduction: Systemic sclerosis (scleroderma) is an inflammatory disease process causing progressive tissue fibrosis via pathologic deposition of type I and III collagen. More common in females, this disease universally affects the skin but can expand into the pulmonary, gastrointestinal, and cardiovascular systems. Cardiac involvement carries a poor prognosis with a mortality rate up to 70% over 5 years. Pericardial effusions and tamponade are rare. Here, we present the case of a 45-year-old male with an unstable cardiac tamponade secondary to scleroderma.

Description: The patient presented to the emergency department with shortness of breath and sharp chest pain. He had several prior hospitalizations for similar symptoms and was aggressively treated for heart failure. Vital signs were notable for hypoxia and severe hypertension, requiring ICU admission, noninvasive ventilation, and a nicardipine drip. Chest CTA noted bilateral pleural effusions and pulmonary edema. An echocardiogram demonstrated a large pericardial effusion with right atrial free wall collapse, physiology concerning for tamponade. The patient underwent emergent pericardial window placement. Given his history of recurrent pericardial effusions, Raynaud's phenomenon, joint pain, and a positive ANA (1:640), there was concern for an underlying rheumatologic process. Per rheumatology recommendations, he was treated empirically with colchicine and prednisone, and subsequently diagnosed with diffuse limited systemic sclerosis.

Discussion: Scleroderma is a debilitating disease with a poor prognosis when untreated. Literature suggests that the incidence of pericardial involvement in scleroderma is 50%, but only 16% of patients are symptomatic, contributing to decreased awareness and late identification. This case demonstrates multiple ICU admissions and ultimately surgical intervention because of delayed treatment, emphasizing the potential consequences of late diagnosis and the need for early POCUS exams. Providers must identify recurrent pericardial effusions, especially in a patient with underlying rheumatological processes, as an indication to screen for systemic sclerosis to ensure timely diagnosis, earlier intervention, and better outcomes in this population.