Abstract
Objectives: To describe the characteristics, treatment, and outcome of patients with immune thrombocytopenia with clinical significance (ITPCS) associated with systemic lupus erythematosus (SLE).Methods: This retrospective multicentre study included SLE patients who experienced ≥1 ITPCS (defined as ITP with attributable bleeding disorders and/or a platelet count <30 x109/L). Other causes of secondary thrombocytopenia were excluded. Major bleeding event (MBG) was defined as Khellaf score>8 and/or WHO score>2.Results: A total of 90 patients were included, the median (range) follow-up duration was 80 (6-446) months. ITP was diagnosed before SLE in 25 patients. They presented high rate of autoimmune haemolytic anaemia (15%), antiphospholipid antibody (62%), and antiphospholipid syndrome (19%). The 25 (28%) patients who experienced MBG had significantly more bleedings at ITP diagnosis and higher bleeding scores, and serositis and thrombosis during follow-up. They required significantly more treatment lines, transfusions, and hospitalizations. The 11 (12%) patients who experienced no bleeding event presented a significantly more restricted SLE phenotype (cutaneous and/or articular). Patients received a mean (range) of 4.2 (1-11) treatment lines. Corticosteroids and hydroxychloroquine allowed ITPCS overall response in one third of patients. The median relapse-free survival of rituximab (n = 34), azathioprine (n = 19), mycophenolate mofetil (n = 8), thrombopoietin-receptor agonists (n = 16), and splenectomy (n = 19) were 53, 31.5, 61, 24.5, and 78 months, respectively. Four patients experienced thrombotic events after splenectomy and one occurred under thrombopoietin-receptor agonist treatment.Conclusion: SLE-ITCS patients displayed a high rate of haematological abnormalities and MBG patients exhibited higher morbidity. Management of thrombocytopenia was highly heterogeneous and many options seem viable.