Faculty Bibliography

Systemic sclerosis is a connective tissue disorder that frequently involves the heart. All cardiac structures can be involved but aortic valve involvement is rare. We report a case of an 83-year-old man with a history of systemic sclerosis presenting with shortness of breath. A transthoracic echocardiogram revealed severe aortic stenosis that could not be explained by other causes. These patients often pose challenges in management as they are not surgical candidates. Transaortic valvular implantation is a viable option for these patients.

Hemophagocytic syndrome (HS) or hemophagocytic lymphohistiocytosis (HLH) is an immune mediated phenomenon that can occur in the setting of an autoimmune disease, chronic immunosuppression, malignancy, or infection. It has been more commonly described in the pediatric population and less commonly in adults. We describe a case of a 52-year-old male who presented with a rash. He simultaneously met the Systemic Lupus International Collaborating Clinics (SLICC) criteria for the diagnosis of systemic lupus erythematosus (SLE) and the diagnostic criteria of HS as described in the hemophagocytic lymphohistiocytosis (HLH) 2004 trial. The bone marrow on autopsy showed the presence of abundant hemosiderophages with focal hemophagocytosis. SLE-associated HS might be underdiagnosed due to the overlap in clinical findings. This case represents the importance of prompt diagnosis and treatment of such a potentially fatal clinical syndrome.