LUPUS NEPHRITIS: CLINICAL CASE WITH DOUBLE TRANSPLANTATION. Ar.A. DEMIN’S CONTRIBUTION TO STUDYING THE DISEASE

Abstract

Introduction. Lupus nephritis (LN) is common in systemic lupus erythematosus (SLE), a severe autoimmune disease with a poor prognosis. Restoring the immune system and self-tolerance using high-dose immunosuppressive therapy with autologous hematopoietic stem cell transplantation (AHSCT) is a new approach in the treatment of refractory SLE. The effectiveness of SLE with LN treatment in the long-term follow-up should be the subject for study. Methods. Patient I., 25 years old, with refractory SLE and LN is described. Standard therapy was ineffective, the SLEDAI score remained 22 points, so the patient underwent high-dose immunosuppressive therapy (HIT) with ASCT, and she was included in the registry of the European Group for Blood and Marrow Transplantation of the European League Against Rheumatism (EBMT/EULAR). Results. As a result of ASCT, complete clinical and serological remission was achieved (score on the SLEDAI scale – 0 points) for 3 years. Then there was a relapse with nephrotic syndrome and anti-DNA positivity which led to the end-stage renal failure. Despite therapy, the disease activity persisted, and the patient was on regular hemodialysis for 1 year, then underwent allogeneic kidney transplantation. Currently, 18 years have passed since HIT and AHSCT and 11 years since renal transplantation. The patient receives standard post-transplant immunosuppression, and her condition remains stable, the renal allograft is functioning, the SLEDAI score is 2 points (low titer anti-DNA positivity). Discussion. Modern standards of chemotherapy (hydroxychloroquine, mycophenolate mofetil, cyclophosphamide, corticosteroids), bioactive immunotherapy (rituximab, belimumab, obinituzumab, anifrolumab), “multi-target therapy” (standard + tacrolimus, voclosporin, mycophenolate mofetil), and new approaches (immuno- and nephrotransplantation) for lupus nephritis. Conclusion. The successful long-term results of the first case of immunoablation with high-dose immunosuppressive therapy and double autologous stem cell and allogeneic kidney transplantation for SLE with refractory lupus nephritis and an analysis of the effectiveness of modern treatment approaches are presented.