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

Therapeutic plasma exchange (TPE) offers an alternative therapeutic modality for patients with systemic lupus erythematosus (SLE) and primary antiphospholipid syndrome (APS). However, there is conflicting evidence regarding its efficacy in different sub-phenotypes. This study aimed to investigate the main clinical characteristics and outcomes of patients with different phenotypes of SLE and APS treated with TPE at a tertiary care center.

The database of the Blood and Apheresis Unit between 2001 and 2013 was screened for patients with SLE and primary APS. SLE disease activity index (SELENA-SLEDAI), the indications for treatment, complications, and outcomes were obtained from a review of medical records and phone calls. A total of 24 patients (SLE: 20, APS: 4) were recruited for the study.

Mean ages of SLE (M/F: 1/19) and primary APS (PAPS) patients (M/F: 2/2) were 32.4±12.89 and 52.0±10.7 years, respectively. The main indications for TPE were hematologic, neurologic, and pulmonary involvement and APS-related symptoms. TPE was preferred in eight patients because of leucopenia and co-infection. SLEDAI was significantly decreased after TPE (16.7±8.3 before vs. 8.8±3.1 after, p=0.001). Both primary APS and SLE-related catastrophic APS (CAPS) patients had completely responded to TPE. The success rate of TPE in patients with thrombocytopenia was lower than patients with hemolytic anemia. The median (IQR 25%-75%) number of TPE sessions was 6.5 (5-10.5). In total, 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 time was 33.5 (6.75-81.25) months. In total, four patients died during follow up, of which three died during the period of TPE administration.

Our data suggest that CAPS and other APS-related problems respond well to the TPE treatment. TPE should be kept in mind for the treatment of patients with other features of SLE, especially those resistant to other agents and in the presence of leucopenia.