Successful use of plasma exchange in fulminant lupus myocarditis coexisting with pneumonia: A case report

BACKGROUND Fulminant lupus myocarditis is a rare but fatal manifestation of systemic lupus erythematosus.Aggressive immunosuppressive treatments are important in its successful management.However,they can significantly damage the immunity and are associated with a considerable risk of infection development and spread.We present a rare and complicated case of a 20-year-old female diagnosed with fulminant lupus myocarditis accompanied by pneumonia.The patient was successfully treated with plasma exchange(PE)for fulminant lupus myocarditis.CASE SUMMARY A 20-year-old Chinese woman presented to the Hematology Department complaining of fatigue and knee pain.Blood test showed anemia and thrombocytopenia.On the second day of hospitalization,she was transferred to the ICU due to dyspnea and hypotension.Autoimmune profiles showed hypocomplementemia and positive antinuclear antibodies.Computer tomography showed an enlarged heart and pneumonia.Ultrasound revealed an enlarged heart with a low left ventricular ejection fraction.Fulminant lupus myocarditis with cardiogenic shock was initially considered.Due to the accompanying pneumonia,aggressive immunosuppression was contraindicated.Her cardiac function remained critical after the initial therapy of intravenous immunoglobulin and corticosteroids at a conventional dose,but she responded well to later PE therapy plus corticosteroids administration.The patient fully recovered with normal cardiac function.CONCLUSION This case indicates that PE is a valuable treatment choice without adverse effects of immunosuppression in patients with fulminant lupus myocarditis and coexisting infection.

Lupus Myocarditis： A Case-Control Study from China

Background：Myocarditis is an uncommon but serious manifestation of systemic lupus erythematosus （SLE）.This study aimed to investigate clinical characteristics and outcomes of lupus myocarditis （LM） and to determine risk factors of LM in hospitalized Chinese patients with SLE.Methods：We conducted a retrospective case-control study.A total of 25 patients with LM from 2001 to 2012 were enrolled as the study group,and 1 O0 patients with SLE but without LM were randomly pooled as the control group.Univariable analysis was performed using Chi-square tests for categorical variables,and the Student＇s t-test or Mann-Whitney U-test was performed for continuous variables according to the normality.Results：LM presented as the initial manifestation of SLE in 7 patients （28％） and occurred mostly at earlier stages compared to the controls （20.88 ± 35.73 vs.44.08 ± 61.56 months,P =0.008）.Twenty-one patients （84％） experienced episodes of symptomatic heart failure.Echocardiography showed that 23 patients （92％） had decreased left ventricular ejection fraction （＜50％） and all patients had wall motion abnormalities.A high SLE Disease Activity Index was the independent risk factor in the development of LM （odds ratio =1.322,P ＜ 0.001）.With aggressive immunosuppressive therapies,most patients achieved satisfactory outcome.The in-hospital mortality was not significantly higher in the LM group than in the controls （4％ vs.2％,P =0.491）.Conclusions：LM could result in cardiac dysfunction and even sudden death.High SLE disease activity might potentially predict the occurrence of LM at the early stage of SLE.Characteristic echocardiographic findings could confirm the diagnosis of LM.Early aggressive immunosuppressive therapy could improve the cardiac outcome of LM.