Prevalence, patterns of disease and outcome in patients with systemic lupus erythematosus who develop severe haematological problems

Objective. To evaluate the prevalence of major haemolytic disease-severe autoimmune haemolytic anaemia and severe thrombocytopenia-and to assess when these features develop. We also sought to analyse the clinical and serological outcomes of patients with haemolytic anaemia and thrombocytopenia with systemic lupus erythematosus (SLE) as compared with patients without these cytopenias. Methods. We reviewed retrospectively all the available case notes from our lupus cohort of 305 patients followed up between 1978 and 2000 (mean follow-up 7 yr). We identified 30 patients with SLE (9.8%), of whom 20 (6.6%) had severe haemolytic anaemia and 10 (3.3%) had severe thrombocytopenia. Each patient was matched for age, sex and ethnicity with two control patients. Results. We recorded a total of 42 episodes of severe haematological events: four patients had a second haemolytic episode and eight patients had a second thrombocytopenic episode. Five patients had both thrombocytopenia and haemolytic anaemia. One per cent of patients had severe haemolytic anaemia prior to the diagnosis of SLE and 2.5% of patients presented with these haematological disorders. Haemolytic anaemia and thrombocytopenia were associated with renal involvement (0.01 > P > 0.001) and anticardiolipin antibodies (ACL) (0.01 > P > 0.001), but not anti-dsDNA antibodies. Calculation of the BILAG index at the time of severe haematological crisis demonstrated that renal, central nervous system involvement and general symptoms are more frequently present. Forty-one per cent of patients were already on either prednisolone (<10 mg) or an immunosuppressive agent at the onset of the event. Conclusion. Our data demonstrate that both haemolytic anaemia and thrombocytopenia are associated with ACL but not anti-dsDNA antibodies. When faced with a patient with a severe haematological manifestation of lupus, active disease in other organs is likely to be present.