Successful treatment by systemic corticosteroids and intravenous cyclophosphamide in acquired pure red cell aplasia associated with systemic lupus erythematosus

Abstract Commonly acquired pure red cell aplasia (PRCA) can be associated with an underlying autoimmune disease. Reports of PRCA associated with systemic lupus erythematosus (SLE) are rare, and no treatment strategies have been documented. We report a case of PRCA associated with SLE that responded to intravenous cyclophosphamide (IVCY) and systemic corticosteroids. A 62-year-old Japanese female was diagnosed with PRCA and SLE. At the time of the initial visit, the patient simultaneously presented with severe proteinuria, massive pleural effusion, and interstitial changes. Notably, cyclosporine and corticosteroids did not improve PRCA and SLE status of the patient. After IVCY and corticosteroid administration, the patient’s reticulocyte count and anaemia improved. Various other symptoms associated with SLE also improved. Cyclophosphamide is typically administered in PRCA in small, continuous oral doses. However, in this case, we did not need to initiate this continuous, low-dose treatment after IVCY. Overall, this study highlights therapeutic strategies involving IVCY in treating PRCA associated with SLE.