Fulminant lupus myocarditis (LM) is an uncommon heart manifestation of systemic lupus erythematosus (SLE).1 Although extracorporeal membrane oxygenation (ECMO) is a standard therapy for patients with cardiogenic shock, its use in the context of SLE is anecdotal.2 We describe an SLE patient with fulminant LM requiring ECMO.
A 26-year-old Filipino woman with a history of SLE was admitted to our center in January 2015 with fever and dyspnea. SLE had been diagnosed 4 years previously based on perimyocarditis, serositis, leucopenia, Raynaud's phenomenon, hypocomplementemia, and positive antinuclear, anti-Sm, and anti-RNP antibodies. She was treated with hydroxychloroquine and pulses of methylprednisolone and cyclophosphamide and received azathioprine as maintenance treatment. She showed a good clinical response and remained in remission for the next 4 years.
At the current admission, the patient reported a 6-day history of fever and worsening dyspnea. Six months previously, she had abandoned treatment. Physical examination revealed increased heart (110 bpm) and respiratory (26 pm) rates and unremarkable breath and heart sounds. Laboratory tests found a high erythrocyte sedimentation rate (60mm/h; normal value, < 20) and normal creatinine kinase but elevated troponin I (0.103mg/L; normal value, < 0.05). Electrocardiogram showed sinus tachycardia, with no ST-segment changes, and transthoracic echocardiography found no dilated chambers and normal valve function and left ventricular ejection fraction (LVEF; 55%).
Treatment with prednisone (30mg/d) and meropenem was instituted, but the patient continued to have recurrent fever and a tendency to hypotension. Blood tests showed elevated troponin I (1 ng/dL) and pancytopenia (leucocytes, 2.3 × 109/L; hemoglobin, 6.7g/dL; platelet count, 75 × 109/L). A second echocardiogram showed biventricular global dysfunction (LVEF, 35%). Microbiological tests for bacteria, fungi, viruses, and mycobacteria were all negative. Subsequent investigations revealed a positive antinuclear titer (> 1:640), negative anti-DNA and antiphospholipid antibodies, and normal complement levels. Despite dobutamine infusion, diuretics, intravenous pulses of methylprednisolone (1g/d for 5 days) followed by prednisone (60mg/d), an intravenous pulse of cyclophosphamide (1g), and empiric broad-spectrum antibiotics (piperacillin/tazobactam and daptomycin), the patient developed respiratory distress with shock. Repeat echocardiography showed ventricular deterioration (LVEF, 20%) with a restrictive transmitral flow pattern, moderate mitral insufficiency, mild tricuspid insufficiency with mildly elevated pulmonary artery pressure, and a cardiac index of 2.0 L/m2. Due to the clinical suspicion of recurrent acute LM, propensity for reversibility with immunosuppression, and given the life-threatening situation, ECMO was instituted and rituximab (2 doses of 1g fortnightly) was added to the medical therapy.
After ECMO implementation and following high-intensity immunosuppression therapy, the patient showed progressive clinical improvement with hemodynamic stability. After a week, she was completely weaned from dobutamine and the ECMO was discontinued, with full recovery of ventricular function (LVEF, 50%). The patient was discharged with no signs of ECMO complications and was treated with hydroxychloroquine (200mg/d) and prednisone (15mg/d) as maintenance therapy. After 1-year follow-up, the patient remains asymptomatic.
LM is a rare manifestation of SLE with a prevalence ranging from 5% to 10%.1 Clinical features include fever and congestive heart failure.1 Fulminant LM presenting as cardiogenic shock was described in only 10% of the largest reported series on LM.3
In daily clinical practice, LM diagnosis represents a major challenge and is based on high clinical suspicion supported by elevated cardiac biomarkers and echocardiographic evidence of impaired LVEF. Endomyocardial biopsy is the gold standard, but the invasiveness of the procedure and its poor negative predictive value limit its use.4 Cardiac magnetic resonance is a promising technique but further studies are needed to support its usefulness in SLE patients.
Up to 70% of patients with LM have good prognosis with recovery of cardiac function within days or weeks. However, in the acute phase, 4%-10% of patients die of fulminant myocarditis with cardiogenic shock refractory to medical therapy due to severely reduced ventricular contractility or malignant arrhythmias. In these patients, mechanical circulatory support devices such as ECMO may play a major role as bridging therapy to keep the patient alive and allow time for the potential recovery of the myocardium in response to immunosuppressive treatment.5
ECMO has a notable value in fulminant myocarditis compared with left ventricular assist devices. When this severe clinical situation is associated with ventricular arrhythmias, left ventricular assist devices are unlikely to provide sufficient cardiac output when the right ventricle is not pumping effectively, whereas ECMO bypasses biventricular failure.
In the case of fulminant myocarditis where recovery of cardiac function is expected if the patient survives the acute phase of the disease, such as in the case of LM, data from a recent meta-analysis suggest a favorable short- and long-term survival, with more than two-thirds of patients requiring ECMO surviving to hospital discharge.6
Treatment of LM is based on high doses of glucocorticoids combined with immunosuppressant agents such as intravenous cyclophosphamide. Anecdotal reports describe good outcomes with intravenous immunoglobulin and plasma exchange and rituximab should be considered in refractory patients.1
Hence, we describe an SLE patient with fulminant LM, an uncommon but severe cardiac manifestation. The present case highlights the value of bridging therapy with ECMO and the usefulness of ECMO in boosting the long-term outcomes of severe SLE patients.