Is There a Role for B-cell Depletion as Therapy for Scleroderma? A Case Report and Review of the Literature

被引:45
作者
Daoussis, Dimitrios [1 ]
Liossis, Stamatis-Nick C. [1 ]
Tsamandas, Athanassios C. [2 ]
Kalogeropoulou, Christina [3 ]
Kazantzi, Alexandra [3 ]
Korfiatis, Panagiotis [4 ]
Yiannopoulos, Georgios [1 ]
Andonopoulos, Andrew P. [1 ]
机构
[1] Univ Patras Med Sch, Patras Univ Hosp, Div Rheumatol, Dept Internal Med, Patras 26504, Greece
[2] Univ Patras Med Sch, Patras Univ Hosp, Dept Pathol, Patras 26504, Greece
[3] Univ Patras Med Sch, Patras Univ Hosp, Dept Radiol, Patras 26504, Greece
[4] Univ Patras Med Sch, Dept Med Phys, Patras 26504, Greece
关键词
systemic sclerosis; scleroderma; rituximab; B-cells; fibrosis; ILD; therapy; INTERSTITIAL LUNG-DISEASE; GROWTH-FACTOR RECEPTOR; VERSUS-HOST-DISEASE; SYSTEMIC-SCLEROSIS; STIMULATORY AUTOANTIBODIES; SKIN FIBROSIS; PDGF RECEPTOR; RITUXIMAB; AUTOIMMUNITY; EXPRESSION;
D O I
10.1016/j.semarthrit.2009.09.003
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Objectives: Rituximab (RTX) has been successfully used in the treatment of several rheumatic diseases with an acceptable safety profile. We present herein a patient with systemic sclerosis (SSc) who exhibited significant improvement of his lung function and skin fibrosis following RTX administration, and review the literature regarding the role of B-cells in SSc and the potential efficacy of RTX in its treatment. Methods: We performed an internet search using the keywords systemic sclerosis, scleroderma, rituximab, B-cells, fibrosis, interstitial lung disease (ILD), and therapy. Results: Our patient, a 40-year old man with severe SSc-associated ILD, received 4 courses of RTX. The patient's lung function improved; forced vital capacity and diffusing capacity of carbon monoxide reached values of 35% and 33%, respectively, compared with 30% and 14% of pretreatment values. Skin thickening assessed clinically and histologically improved as well. Several lines of evidence suggest that B-cells may have a pathogenic role in SSc. B-cells from tight skin mice-an animal model of SSc-exhibit chronic hyperactivity; likewise, B-cells from patients with SSc overexpress CD19 and are chronically activated. Furthermore, studies have revealed that B-cell genes were specifically transcribed in SSc skin and that B-cell infiltration was a prominent feature of SSc-associated ILD. The potential clinical efficacy of RTX in SSc has been explored in a limited number of patients with encouraging results. Preliminary data suggest that RTX may favorably affect skin as well as lung disease in SSc. Conclusions: Several basic research data underscore the potential pathogenic role of B-cells in SSc and clinical evidence suggests that RTX might be a therapeutic option in SSc. Large-scale multi-center studies are needed to evaluate the potential clinical efficacy of RTX in SSc. (C) 2010 Elsevier Inc. All rights reserved. Semin Arthritis Rheum 40:127-136
引用
收藏
页码:127 / 136
页数:10
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