Background/Purpose: To analyze the effect of antimalarials (AM) as preventive factor for the development of severe cytopenias and in their outcome after treatment in a large series of patients with systemic lupus erythematosus (SLE)

Methods: 253 SLE patients followed in rheumatology department (12 de Octubre hospital) between 1976-2014 were included. Demographic, clinical data and patients outcome were obtained from pre-existing databases and from the charts. SLE related cytopenias were defined as: autoimmune haemolytic anemia (AHA): hematocryt<35%, reticulocytes>5% or spherocytosis in peripheral blood smear; leukopenia:WBC<4000x106/l; neutropenia: neutrophils<1000x106/l; thrombocytopenia (TP):moderate 50-100x109/l and severe <50x109/l. The associations between categorical variables were tested using the chi-square or Fisher’s exact test, where appropriate. The odds ratios with the corresponding 95% CIs were calculated. For continuous variables, the comparisons were carried out using the t-test for two independent samples. P-values<0.05 were considered significant. The analysis was performed using advanced SPSS software version 11.

Results: 253 patients were included (91.3% women), with a mean age at SLE onset of 30 ± 13 years. Globally, 74.3% of patients developed hematological involvement (HI): 37.2% TP(21% moderate, 21% severe); 26.1% AHA, 75% leukopenia, 68% lymphopenia and 11.2% neutropenia. Evans syndrome was present in 13 patients and secondary antiphospholipid syndrome (APS) was diagnosed in 19.4%. Among patients with severe haematological disease, aggressive treatments (bolus of methylprednisolone, cyclophosphamide, rituximab, intravenous immunoglobulin or esplenectomy) were needed in 9% of patients with TP and in 18% of AHA. Only 20% of the patients had received AM prior to the developement of HI. In 5.5% of cases life-threatening complications appeared because of HI. Relapses occurred in TP (9,1%) and in AHA (2.8%). In 3 cases of neutropenia colony stimulating factor was needed, all relapsed cases. TP was significantly associated with having positive aDNA (p0.035), antiB2GP1IgG, lupus anticoagulant AL +, with APS and venous thrombosis secondary to APS (all p<0.01). AHA was associated with malar rash and discoid lupus, and AL+, ACL IgG +, antiB2GP1IgG and IgM (all p<0,05)  but not with APS. AHA was associated with exitus as a result of hematologic involvement (p0,039). Neutropenia was associated with arthritis (p 0.024). Hypocomplementemia (C3, C4) was associated with TP, AHA (all p<0.05) Treatment with hydroxychloroquine was associated with a tendency of complete response to therapy in AHA and a partial response in TP.

Conclusion: Up to 41% of patients had some kind of severe cytopenia. Only AHA was associated with increased mortality from this cause. All cytopenias were associated with hypocomplementemia except neutropenia. AM treatment tended with favorable therapeutic response in AHA and thrombocytopenia. In our series, treatment with antimalarial drugs not proved to be a protective factor for developing severe cytopenias LES related neither for prevent severe complications or to prevent recurrences, although further studies are needed.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/influence-of-antimalarial-doesnt-modify-the-outcome-of-cytopenias-in-systemic-lupus-erythematosus/