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Giant cell myocarditis: a rare cardiovascular manifestation in a patient with systemic lupus erythematosus

Division of Immunology and Rheumatology, Department of Medicine, Stanford University School of Medicine, Palo Alto, CA, USA

G J Berry

Department of Pathology, Stanford University School of Medicine, Palo Alto, CA, USA

E F Chakravarty

Division of Immunology and Rheumatology, Department of Medicine, Stanford University School of Medicine, Palo Alto, CA, USA, echakravarty{at}stanford.edu

Giant cell myocarditis (GCM) is a rare form of myocarditis with a median survival of less than one year. It has been reported to occur in patients with various underlying autoimmune diseases; however, no cases of GCM have been described in patients with clear evidence of underlying systemic lupus erythematosus (SLE). The presentation of GCM may mimic that of lupus myocarditis, including an initial response to immunosuppression. Despite initial clinical similarities, lupus myocarditis and GCM are histologically distinct entities with dramatic differences in prognosis. We report herein a patient with a longstanding history of SLE, who presented acutely with myocarditis, responded well to initial immunosuppression and then subsequently died of progressive heart failure that was found to be due to GCM. Endomyocardial biopsy can help define diagnosis and prognosis of lupus patients presenting with myocarditis, and early referral for cardiac transplantation should be considered in patients diagnosed with GCM.

Key Words: congestive heart failure • giant cell myocarditis • immunosuppression • lupus myocarditis • systemic lupus erythematosus

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