Systemic lupus erythematosus complicated with pure red cell aplasia: a case report and literature review

Abstract

This article reports a single case of a patient with systemic lupus erythematosus (SLE) combined with pure red cell aplasia (PRCA), and reviews 51 additional cases of patients reported by domestic and overseas papers from 1974 to 2021. These 52 (51+1) cases were analyzed to summarize the epidemiological features, clinical features, laboratory inspections, treatments and prognosis of the patients. The results indicated that among all the 52 cases, cases of SLE combined with PRCA were mostly seen in Asian childbearing age women. The median ages of patients diagnosed with SLE and diagnosed with PRCA were 31.5 years and 36.0 years, respectively. The time interval between the initial diagnosis of SLE and subsequent diagnosis of PRCA was significantly longer than the interval for the initial diagnosis of PRCA, suggesting a delayed onset of SLE in these patients (P=0.042). Various clinical features of the 52 patients were reported, including mostly fatigue, joint pains, Raynaud phenomena and rashes, and SLE maybe combined with autoimmune hemolytic anemia (AIHA), thymoma, hypothyroidism and myasthenia gravis (MG). In these reported cases, laboratory indicators showed higher proportions of antinuclear antibody (ANA), anti double stranded DNA antibody (anti-dsDNA antibody), positive urinary protein and low complement levels. Among the 52 patients, 51 cases (98.08%) were treated with glucocorticoids, followed by blood transfusion, cyclosporin A, cyclophosphamide and high-dose intravenous immunoglobulin. Of the 50 patients whose prognoses were reported, 44 showed improvement, while 3 treatments were not effective and 3 resulted in death. This article aims to enhance the understanding of SLE combined with PRCA among doctors.

Keywords

• systematic lupus erythrematosus (sle)
• pure red cell aplasic anemia (prca)
• epidemiological characteristic
• clinical characteristic