Abstract
BackgroundHematological manifestations are common in systemic lupus erythematosus (SLE), and are thought to result from immune-mediated peripheral cell destruction or bone marrow failure(1,2).ObjectivesTo assess the clinical characteristics and outcomes of severe hematological disease in a large cohort of lupus patients.MethodsRetrospective analysis of the “Attikon” lupus cohort (over 800 patients total) (3) for the identification of patients with a history of severe hematological manifestation. The latter were defined as: thrombocytopenia with a platelet count <30.000/mm3, hemolytic anemia with an hemoglobin <8 g/dL, neutropenia with less than 500 neutrophils/mm3, history of thrombotic microangiopathy (TMA) or macrophage activation syndrome (MAS). Treatments and long-term outcomes (relapses, mortality) were recorded.ResultsAmong 300 patients with hematologic manifestations, 40 patients had severe disease. Most of them were women (75%). Mean age at SLE diagnosis was 41,1 years and mean disease duration at diagnosis of cytopenia was 3.8 years. Hematologic manifestation preceded SLE diagnosis in 13 patients (32,5%).Autoimmune thrombocytopenia was the most common (57,5%), followed by hemolytic anemia (17.5%), TMA (12.5%) and Evans syndrome (7,5%). All patients received glucocorticoids (GC). Rituximab (15%) and cyclophosphamide (12,5%) were the most frequently used GC-sparing agents during the first episode. Nine patients (22,5%) received intravenous immunoglobulin (IVIg). Relapse occurred in 23 patients (57,5%), most of which (N=17, 73,9%) were treated with GC alone or in combination with IVIg or plasma exchange at initial presentation, without the use of GC-sparing agents. Most of the flares (60,8%) were severe. No deaths were observed.ConclusionHematological disease in SLE is treatment-responsive. GC remain the mainstay of treatment, but the high relapse rates underscore the need for more efficient GC-sparing agents.
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