Recurrent thrombotic events occur in 20% of patients with APS despite of adequate antithrombotic therapy. As phospholipid-cofactor antibodies are key elements of pathophysiology, B cell depleting rituximab is a rational therapeutic approach.
Aims were to identify and characterise APS patients receiving rituximab. All together 179 (60 primary, 119 lupus-associated) APS patients were found in our database of whom 15 (8.4%) were treated with RTX. As younger and male patients dominated in RTX +group, 15 from RTX naive patients were matched regarding age and gender for a case control study. First symptoms appeared at the age of 30 in both groups. Patients were followed for 4 and 7 years, the diagnosis delayed 4 and 1 year in RTX +and RTX- groups, respectively. This 3 year difference might contribute to higher disease severity. Primary APS (47% vs 32%), cerebrovascular events (13 vs 2), valvular heart disease (5 vs 2), and high risk triple aPL positivity (13 vs 5) were more prevalent in RTX +group. All 4 deep venous thrombosis cases in the RTX +group were complicated with pulmonary embolism in comparison with 1 PE within 6 RTX- DVT patients. Raynaud syndrome resulted in digital ulcer more frequently in RTX +than in RTX- group (4/4 vs 1/6). Antimalarials were given to 3 and 10, cumarin or NOAC were iniciated in 15 and 5 patients in the RTX +and RTX- groups, respectively. Rituximab was started 2 (0.5–6) years after diagnosis. Within 10 (3–60) moths follow-up on RTX there were no incident thrombotic events. Two thirds of patients could stop steroid. RTX was stopped in 6 cases: 3 due to remission (of whom 2 relapsed), 1 LFU, and 2 adverse events (1 longstanding B cell depletion, 1 peroneus paresis). Other AEs were 1 mild infusion reaction, 1 leukopenia, 1 UTI. As summarised, delay in APS diagnosis and male gender were associated with more severe disease and the need for rituximab. Antimalarials and associating lupus were identified as markers of more favourable disease outcome. In patients with recurrent thrombotic events despite of adequate anticoagulant therapy, RTX can be a rational and effective choice with favourable safety profile.
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