Abstract

Abstract Background and Aims Lupus nephritis is a common part of systemic lupus erythematosus (SLE), severe autoimmune disease that affects multiple organ systems and associated with pure prognosis. Patients with lupus nephritis who experience persisted disease activity despite conventional immunosuppression are at high risk of early death. Re-setting of the immune system and self-tolerance by high-dose immunosuppressive therapy with autologous stem cell transplantation (ASCT) is a new approach in the treatment of refractory SLE. Remote outcomes of this method and effectiveness relapse treatment are still unclear and were the aims of this study. Method We report a sick woman 39 years old, with refractory severe lupus nephritis. Standard therapy was ineffective, SLEDAI score remains 22 so patient was underwent high dose immunosuppressive therapy with ASCT and included in European Group for Blood and Marrow Transplantation European League against Rheumatism (EBMT/EULAR) registry on 53 pts who received ASCT for SLE between 1996 and 2005. Ethical approval was obtained for this study from Cambridge University Hospital Ethics Committee. Results The ASCT induced complete clinical and serological remission (SLEDAI score was 0) for 3 years. Than relapse with nephritic syndrome and anti-dsDNA positivity occurred and caused renal failure, creatinine clearance decreased to 21. Despite therapy including prednisolone, 45 mg daily, and mycophenolate mofetil, 2000 mg daily, disease activity persisted, creatinine clearance remained decreasing and 3 years later became 15 ml/min. During 1 year patient was on regular hemodialysis, then renal transplantation performed. Now, follow up is 15 years after ASCT and 8 years after kidney transplantation. Patient received a standard post-transplant immunosuppression with prednisolone, 5 mg daily, tacrolimus, 2.5 mg daily, and azathioprine, 100 mg daily, and her conditions remains stable, she has functioning renal allograft, SLEDAI score is 2 (anti-dsDNA positivity in low titer). Conclusion We present the successful remote outcomes of first case of immunoablation and double transplantation of autologous stem cells and allogeneic kidney in severe refractory to conventional immunosuppression SLE with lupus nephritis.

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