Affiliation:
1. Rheumatology and Clinical Immunology Unit, 4th Department of Internal Medicine, Attikon University Hospital, Athens, Greece
2. Faculty of Medicine, National and Kapodistrian University of Athens, Inflammation & Autoimmunity Lab, Biomedical Research Foundation of the Academy of Athens (BRFAA), Athens, Greece
3. 1st Department of Propaedeutic Internal Medicine, “Laiko” General Hospital, Athens, Greece
4. Joint Academic Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece
Abstract
Background Hematologic manifestations are common in systemic lupus erythematosus (SLE), either at initial presentation or during the course of the disease, but data regarding their natural history are scarce. Objective To describe the characteristics, treatments, and outcomes of severe hematological manifestations in a large cohort of lupus patients. Methods Retrospective cohort study of patients in the “Attikon” lupus cohort who had a history of a severe hematologic manifestation, defined as autoimmune hemolytic anemia (AIHA) with hemoglobin < 8 g/dL, thrombocytopenia with platelet count < 30,000/mm3, Evans syndrome with hemoglobin < 8 g/dL, and/or platelet count < 30,000/mm3, neutropenia with < 500 neutrophils/mm3, thrombotic microangiopathy (TMA)/thrombotic thrombocytopenic purpura (TTP)-like syndrome, or macrophage activation syndrome (MAS). Demographic and clinical characteristics, treatments, and outcomes were recorded. Results From over 300 patients with hematologic manifestations, 41 qualified as severe (70.7% women, mean [SD] age at SLE diagnosis 42.6 [18.0] years). Hematologic manifestations preceded SLE diagnosis in 13 patients (31.7%), was concomitant to SLE diagnosis in 16 patients (39%), and occurred during the course of the disease in 12 (29.3%) patients, with a mean (SD) disease duration of 8.7 (5.5) years. Thrombocytopenia was the most common severe hematological manifestation (56.1%), followed by AIHA (17.1%) and TTP-like syndrome (12.2%). For initial treatment, all patients were treated with glucocorticoids (GC), while rituximab and cyclophosphamide were the most frequently used immunosuppressive agents. Following initial treatment, relapse occurred in 22 patients (53.7%). Compared to patients that did not relapse, those that relapsed had less often received concomitant immunosuppressive agents following treatment of initial episode ( n = 17/23, 73.9% vs 5/17, 29.4%, p = 0.005). Conclusion Severe hematologic disease in SLE has a high risk of relapse, which may be mitigated by the early institution of GC-sparing agents.