Pure red cell aplasia in systemic lupus erythematosus, a nationwide retrospective cohort and review of the literature

Objectives To characterize the clinical and biological course, management and response to treatment in Systemic Lupus Erythematosus (SLE)-associated Pure Red Cell Aplasia (PRCA). Methods Nationwide multicentre retrospective cohort study. From 2006 to 2018, we included adults with a diagnosis of PRCA supported by bone-marrow examination and SLE or biologic manifestations of SLE after ruling out parvovirus B19 infection. Results We enrolled 24 patients (20 women). SLE was diagnosed before PRCA for 14 patients (median delay 81 months). At PRCA diagnosis, mean age, haemoglobin level and reticulocyte and differential erythroblast count were 39.2 ± 13.2 years, 62 ± 20 g/L, 9.1 ± 7.6 x 109/L and 2.8 ± 2.5%, respectively. Eleven (45%) patients experienced multiple PRCA flares (median 6, range: 2-11). Corticosteroid therapy resulted in only three complete sustained responses, and 19 (79%) patients required immunosuppressive agents with highly variable regimens. After a median follow-up of 76 months (range 13-173), 17 (71%) patients showed complete response for PRCA, five (21%) partial response and two (8%) treatment failure. In total, 21 (87%) patients required red-blood-cell transfusion; five had a diagnosis of transfusion-related iron overload. Eighteen (75%) patients experienced severe infectious events requiring hospitalization. Conclusion SLE-associated PRCA is a severe condition. Repeated red-blood-cell transfusions and several lines of immunosuppressant therapy are mostly required with high risk of severe infectious events and iron overload. Despite sustained response for PRCA and SLE obtained in most patients, the best therapeutic strategy remains to be determined.