SLE - Systemic lupus erythematosus a BLySful, yet BAFFling, disorder 英文参考文献.docVIP

Arthritis Research Therapy Vol 5 No 3 Stohl Commentary SLE Systemic lupus erythematosus: a BLySful, yet BAFFling, disorder William Stohl Division of Rheumatology, University of Southern California Keck School of Medicine, Los Angeles, CA, USA Corresponding author: Williams Stohl (e-mail: stohl@) Received: 13 Feb 2003 Accepted: 5 Mar 2003 Published: 27 Mar 2003 Arthritis Res Ther 2003, 5:136-138 (DOI 10.1186/ar755) ? 2003 BioMed Central Ltd (Print ISSN 1478-6354; Online ISSN 1478-6362) Abstract BLyS/BAFF (B-lymphocyte stimulator/B-cell activating factor) is a vital B-cell survival factor. Overexpression of BLyS in mice may lead to systemic-lupus-erythematosus-like (SLE-like) disease, and BLyS overexpression is common in human SLE. Treatment of SLE-prone mice with a BLyS antagonist ameliorates disease progression and enhances survival, making BLyS an attractive therapeutic target in human disease. However, several unresolved issues remain, including what is the contributory role of APRIL (a tumor-necrosis-factor superfamily member related to BLyS) in the ‘autoimmunogenic’ effects of BLyS, identification of the ‘optimal’ BLyS antagonist, and identification of those SLE patients most likely to benefit from BLyS antagonist therapy. Keywords: APRIL, B cells, biologic antagonists, BLyS/BAFF, SLE Introduction SLE), constitutive overproduction of BAFF in mice not only leads to B-cell expansion and polyclonal hypergamma- globulinemia, but also often leads to elevated circulating titers of multiple autoantibodies (including anti-dsDNA), BLyS (B-lymphocyte stimulator), also commonly known as BAFF (B-cell activating factor), is a 285-amino-acid member of the TNF- (tumor necrosis factor)-ligand super- family [1–5]. (Because the two names are both so common, I use them interchangeably throughout this com- mentary.) It is a vital B-cell survival factor [6–8], and mice genetically defi