Description Systemic lupus erythematosus (SLE) is an immune system disease characterized by immune system activation, which leads to exaggerated B cell and T cell responses and loss of immune tolerance against self-antigens. We presented a case of SLE presented with autoimmune hemolytic anemia occurring in a 49-year-old Chinese female patient. She was failed in the first and sceond line treatment, including methylprednisolone pulse, immunosuppressants (monotherapy or combination therapy), intravenous gammaglobulin, two courses of rituximab with each course consisting of four 200mg intravenously, and plasmapheresis. However, the patient’s condition was always clinically unstable. Surprisingly, after being treated with rituximab (500mg d 0) plus bortezomib (2.5mg d 1, 4, 8, 11), the immune status of the patient was improved gradually and all indexes returned to normal ranges. Half a year later, rituximab (100mg d 0) plus bortezomib (2.5mg d 1, 4, 8, 11) was applied. Ultimately, the patient was in remission without recurrence until now. Additionally, we summarized the available literature and explained the possible mechanism. Rituximab combined with bortezomib was more effective for depleting B cells and plasmocytes, which was accompanied by decreases in antibodies. Finally, although there were no adverse effects in this patient, we pointed out that rituximab plus bortezomib might cause infection and peripheral neuropathy. Therefore, monitoring safety is required.

Conclusions Our case shows that rituximab combined with bortezomib is effective in treating SLE patients with refractory hemolytic anemia, but safety should be monitored.