Therapeutic plasma exchange for refractory SLE: A comparison of outcomes between different sub-phenotypes

Abstract

Objective: Therapeutic plasma exchange (TPE) offers an alternative therapeutic modality for patientswith systemic lupus erythematosus (SLE) and primary antiphospholipid syndrome (APS). However,there is conflicting evidence regarding its efficacy in different sub-phenotypes. This study aimed toinvestigate the main clinical characteristics and outcomes of patients with different phenotypes ofSLE and APS treated with TPE at a tertiary care center.Methods: The database of the Blood and Apheresis Unit between 2001 and 2013 was screened forpatients with SLE and primary APS. SLE disease activity index (SELENA-SLEDAI), the indications fortreatment, complications, and outcomes were obtained from a review of medical records and phonecalls. A total of 24 patients (SLE: 20, APS: 4) were recruited for the study.Results: Mean ages of SLE (M/F: 1/19) and primary APS (PAPS) patients (M/F: 2/2) were 32.4±12.89and 52.0±10.7 years, respectively. The main indications for TPE were hematologic, neurologic, andpulmonary involvement and APS-related symptoms. TPE was preferred in eight patients because ofleucopenia and co-infection. SLEDAI was significantly decreased after TPE (16.7±8.3 before vs. 8.8±3.1after, p=0.001). Both primary APS and SLE-related catastrophic APS (CAPS) patients had completelyresponded to TPE. The success rate of TPE in patients with thrombocytopenia was lower than patientswith hemolytic anemia. The median (IQR 25%-75%) number of TPE sessions was 6.5 (5-10.5). In to-tal, five patients experienced TPE-related major adverse events (catheter infections in three patients,bleeding in one patient, and hypotension in one patient). The median (IQR 25%-75%) follow-up timewas 33.5 (6.75-81.25) months. In total, four patients died during follow up, of which three died duringthe period of TPE administration.Conclusion: Our data suggest that CAPS and other APS-related problems respond well to the TPEtreatment. TPE should be kept in mind for the treatment of patients with other features of SLE, espe-cially those resistant to other agents and in the presence of leucopenia.