Brief CommunicationRelapsing Evans syndrome and systemic lupus erythematosus with antiphospholipid syndrome treated with Bortezomib in combination with plasma exchange
Introduction
The presence of autoimmune hemolytic anemia (AIHA) in conjunction with immune-mediated thrombocytopenia characterizes Evans syndrome (ES). Although anemia and thrombocytopenia are common features of systemic lupus erythematosus (SLE), ES is a rare manifestation in systemic lupus SLE. Coexistence of antiphospholipid syndrome (APS) with AIHA and APS with ES displays a frequency of 4% and 10%, respectively [1,2]. As the first-line therapy for ES glucocorticosteroids (GC) and intravenous immunoglobulin (IVIG) are typically used. The second-line therapy includes immunosuppressive agents, the monoclonal antibody Rituximab, or chemotherapy (vincristine). In 2008 Rückert et al. reported a case of APS with ES successfully treated with a combination of rituximab and cyclophosphamide [3]. Here, we present a clinical history of refractory ES and SLE with APS successfully treated with Bortezomib (BTZ) that led to steady clinical and serological improvement for both syndromes.
Section snippets
Clinical case
A 32-year-old man with history of recurrent deep vein thrombosis was admitted to our institution with fever, dyspnea and episode of nasal bleeding, life-threatening hemolytic anemia (Hb = 68 g/L) and pancytopenia (RBC = 2,29 ∗ 109/L, WBC = 1,75 ∗ 10^9/L, PP = 4800/μL). Laboratory signs of hemolysis were found (lactate dehydrogenase (LDH) was 655 U/L, reticulocytes were 5%, direct and indirect antiglobulin test were positive). The enzyme-linked immunosorbent assay (ELISA kits, Orgentec
Discussion
We present a case report of a stable remission of relapsing ES and SLE- APS treated with Bortezomib and plasma exchange after non responsiveness to a combination therapy with medium/high dose GC, Cyclosporine A, Rituximab and Cyclophosphamide.
Considering the presence of oral mucosal ulceration, no-erosive arthritis, serositis (pleuritis and pericarditis), hematologic syndrome (leucopenia, thrombocytopenia, hemolytic anemia), immunologic syndrome (aPL, low level of complement), CNS disorder
Conclusion
Unfortunately, we did not observe stable decrease of anti-beta-2-glycoprotein 1 and anti-cardiolipin during BTZ therapy. Still is not clear the pharmacological mechanism of action of PIs in these autoantibody-mediated disorders, since BTZ inhibited the production of the anti-erythrocyte antibodies but this was not the case for antiphospholipid antibodies. A combination of plasma exchange followed by BTZ, low GC doses and cyclosporine A may be a rescue therapy in resistant cases of SLE with APS
Declaration of conflicting interests
The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Acknowledgments
The authors would like to thank Professor P.L. Meroni for critical review of the manuscript.
Funding
This work is supported in part by the grant of the Government of the Russian Federation for the state support of scientific research carried out under the supervision of leading scientists, agreement 14.W03.31.0009, on the basis of SPbU projects 15.34.3.2017 and 15.64.785.2017.
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