Background: Extracorporeal treatments such as plasmapheresis (PP) and immunoadsorption (IA) may be considered in patients with systemic lupus erythematosus (SLE) who do not respond to cyclophosphamide and rituximab for organ-threatening or life-threatening disease. In the treatment of catastrophic antiphospholipid syndrome (CAPS), plasmapheresis also has its place. In a randomized controlled trial involving 86 patients with lupus nephritis (LN), the addition of PP to standard therapy (prednisone and cyclophosphamide) was not shown to improve clinical outcome. Otherwise, the literature on PP/IA in patients with SLE and APS is limited to case reports and observational studies with small numbers of patients.

Objectives: Our objective was to evaluate efficacy and safety data of PP/IA treatments for various disease involvement in a single-center SLE/APS cohort.

Methods: This retrospective analysis included 25 patients (13 SLE, 7 SLE+APS, 5 primary APS) who underwent at least 7 sessions of PP/IA for various indications between 2010 and 2024. Baseline demographic, clinical, and serologic characteristics, indications, extracorporeal treatment modalities, complications attributed to PP/IA, concomitant treatments and laboratory data, SLE disease activity indices (SLEDAI-2K), SLICC damage index (SDI) at 0, 1, 3, 6, 12 months of PLEX were reviewed. Renal response in patients undergoing PP/IA for LN was defined as reduction in proteinuria ≥25% and 50% at 3 and 6 months, respectively, and below 700 mg/day at 12 months. In patients with thrombotic microangiopathy (TMA), response was defined as a platelet count >150×10 ⁹ /L for 2 consecutive days or normalization of the lactate dehydrogenase (LDH) level.

Results: The demographic, clinical, and serologic characteristics of the patients and prior treatments are summarized in Table 1. PP/IA was performed at the time of diagnosis in 10 patients and within 1 year of diagnosis in 12 patients. The indications were LN in 7 patients, TMA in 14 s (2 with thrombotic thrombocytopenic purpura and 3 with CAPS) and diffuse alveolar hemorrhage (DAH) in 6. Twenty-two patients underwent double filtration plasmapheresis (DFPP), while 3 patients underwent IA. Of 22 patients who underwent DFPP, fresh frozen plasma (FFP) was used in 17, albumin in 2, and FFP + albumin in 3. Eleven patients developed plasmapheresis-related coagulopathy (none of whom experienced bleeding) and 1 patient had fluid-electrolyte imbalance. The mean dosage of 1.4 ± 0.4 g methylprednisolone administered with PP/IA, while cyclophosphamide was used in 13 patients and rituximab in 9 patients. In 5 of the 7 patients who underwent PP/IA for LN, renal response was observed as of the 3rd month and persisted until the 12th month. In one of the remaining 2 patients, renal response was achieved in the 24th month, whilst end-stage renal failure developed in other as of the 1st month. All of these 7 patients with LN had also treated with cyclophosphamide. In patients with TMA, the response rate was 5/14 immediately after PP/IA, 11/14 at 1 month, 11/14 at 3 months, and 14/14 at 6 and 12 months. Patients who underwent plasmapheresis for DAH did not require ventilation during follow-up. The SLEDAI-2K and SDI scores and mean daily prednisolone doses of the patients at follow-up are demonstrated in Table 2.

Conclusion: PP/IA administered concomitantly with immunosuppressives for TMA, LN, CAPS, and DAH that emerged in the course of SLE may show efficacy in these indications. This treatment modality was associated with a reduction in disease activity and daily glucocorticoid doses, and no increase in damage score during follow-up. Notably, no significant adverse event other than mild coagulopathy was observed.

REFERENCES: NIL.

Acknowledgements: NIL.

Disclosure of Interests: None declared.

© The Authors 2025. This abstract is an open access article published in Annals of Rheumatic Diseases under the CC BY-NC-ND license ( http://creativecommons.org/licenses/by-nc-nd/4.0/ ). Neither EULAR nor the publisher make any representation as to the accuracy of the content. The authors are solely responsible for the content in their abstract including accuracy of the facts, statements, results, conclusion, citing resources etc.