Treatment of Systemic Lupus Erythematosus (SLE) remains challenging. The aim of this real-world evidence study of patients with refractory SLE treated with rituximab (RTX) was to explore for any potential long-term effect(s) of this particular B cell depletion approach. This study included patients with SLE who had i) received at least 1 cycle of RTX and had ii) an at least 10 yr of follow-up after their first RTX infusion. Outcomes were assessed at 1 year and at their latest evaluation that was ≥ 10 yr after RTX treatment initiation. In cases where the SLE Disease Activity Index 2000 (cSLEDAI-2 k) was employed, a response was defined as a cSLEDAI-2 k of less than 4 in cases where the cSLEDAI-2 k was ≥ 4. In cases where the cSLEDAI-2 k was 2–4 at baseline, a response was defined as a cSLEDAI-2 k of 0. RTX was administered in 62 patients with SLE. For this real-world evidence study 23 patients (25 cases) with SLE (all Caucasian female, age range: 14–72 yr, mean: 31 yr) with active or relapsing disease, fulfilling inclusion criteria were enrolled. RTX treatment was associated with a response rate of 68.75% after 1 yr and 75% after ≥ 10 yr. The median cSLEDAI-2 K score decreased from 5.83 ± 3.70 at baseline to 1.95 ± 2.40 (p < 0.001) at 1 yr and to 2.37 ± 3.00 (p < 0.001) at the ≥ 10 yr time-point of follow-up. Our data suggest that RTX may indeed represent an alternative therapeutic option in patients with SLE refractory to standard treatment with an acceptable safety profile.

Lupus myocarditis (LM) is a rare but potentially life threatening manifestation of systemic lupus erythematous (SLE). LM may be rarely the initial presentation of SLE or ma y be presented during the course of the disease. Herein, we report a case of an adult female patient who presented with a 3-day history of abdominal pain, fever and recent onset dyspnea on exertion. Twenty-four hours later the patient developed acute heart failure went into cardiac arrest during her transfer from the internal medicine department to the CT scanner. She was intubated, an intra-aortic balloon pump was inserted, and was transferred to the intensive care unit (ICU). Based on clinical signs, laboratory parameters and cardiac imaging and after the exclusion of other causes of acute heart failure LM was diagnosed. The patient received high doses of corticosteroids and cyclophosphamide and she was improved. The vasopressors were tapered off and the intra-aortic balloon pump was removed. The patient improved and she shifted out of the ICU. The initially increased t roponin levels were decreased, while the ejection fraction increased to 60% five days later. The patient was followed as an outpatient after hospital discharge. At 6 months of follow-up the she was on treatment with low doses of oral steroids, hydroxychloroquine and azathioprine and her ejection fraction is 73% without signs of active disease. No recurrences of her LM have been observed during her 32 month follow-up. Prompt recognition and aggressive immunosuppressive treatment may lead to favourable prognosis.