systemic lupus erythematosus and the type i interferon system系统性红斑狼疮和i型干扰素系统.pdf

Arthritis Research Therapy Vol 5 No 2 Rönnblom and Alm Review Systemic lupus erythematosus and the type I interferon system Lars Rönnblom1 and Gunnar V Alm2 1Department of Medical Sciences, Section of Rheumatology, University Hospital, Uppsala, Sweden 2Department of Veterinary Microbiology, Biomedical Center, Uppsala, Sweden Corresponding author: Lars Rönnblom (e-mail: Lars.Ronnblom@medsci.uu.se) Received: 19 November 2002 Accepted: 20 December 2002 Published: 20 January 2003 Arthritis Res Ther 2003, 5:68-75 (DOI 10.1186/ar625) © 2003 BioMed Central Ltd (Print ISSN 1478-6354; Online ISSN 1478-6362) Abstract Patients with systemic lupus erythematosus (SLE) have ongoing interferon-α (IFN-α) production and serum IFN-α levels are correlated with both disease activity and severity. Recent studies of patients with SLE have demonstrated the presence of endogenous IFN-α inducers in such individuals, consisting of small immune complexes (ICs) containing IgG and DNA. These ICs act specifically on natural IFN-α-producing cells (NIPCs), often termed plasmacytoid dendritic cells (PDCs). Given the fact that the NIPC/PDC has a key role in both the innate and adaptive immune response, as well as the many immunoregulatory effects of IFN-α, these observations might be important for the understanding of the etiopathogenesis of SLE. In this review we briefly describe the biology of the type I IFN system, with emphasis on inducers, producing cells (especially NIPCs/PDCs), IFN-α actions and target immune cells that might be relevant in SLE. O