Key result
A patient with longstanding systemic lupus erythematosus developed giant cell myocarditis, initially responding to immunosuppression but ultimately dying of progressive heart failure.
Case Report (n=1)
Giant cell myocarditis can occur in patients with SLE and may mimic lupus myocarditis initially, highlighting the importance of endomyocardial biopsy for accurate diagnosis and prognosis.
May warrant biopsy in refractory SLE myocarditis; leaves open incidence, prognosis, and optimal therapy.
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.
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Chung et al. (2005) conducted a case report in Giant cell myocarditis and systemic lupus erythematosus (n=1). Immunosuppression was evaluated on Clinical outcome. A patient with longstanding systemic lupus erythematosus developed giant cell myocarditis, initially responding to immunosuppression but ultimately dying of progressive heart failure.