Refractory Pericardial Effusion in a Patient with Rosai-Dorfman Disease

Rosai-Dorfman disease (RDD) is an extremely rare, histiocytic proliferative disorder most commonly presenting as lymphadenopathy in young adults and children. Although it may present with a wide range of extranodal manifestations, involvement of the cardiovascular system is extremely rare. The etiopathogenesis of RDD is poorly understood. Sporadic RDD is mostly a self-limited disease and has a good clinical outcome. There is a lack of standardized, evidence-based recommendations to treat multifocal extranodal disease. Poor prognostic factors include autoimmune cytopenias as well as disseminated organ involvement, including kidneys, heart, lower respiratory tract, liver, etc. We report a case of a 54-year-old gentleman who presented with refractory pericardial effusion, orbital disease, and lymphadenopathy, with a good clinical response to immunosuppressive therapy.