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Systemic lupus erythematosus : Still a challenge for physicians

Bengtsson, Anders LU and Rönnblom, L. (2017) In Journal of Internal Medicine 281(1). p.52-64
Abstract

Systemic lupus erythematosus (SLE) has a complex clinical picture, and a number of defects in the immune system have been described in patients with the disease. Most organs can be involved in SLE, and in addition to the typical major organ manifestations (e.g. from kidneys and the central nervous system), early cardiovascular disease is a major determinant of prognosis. Several important findings during the last decade have increased the understanding of the mechanisms behind the disease characteristics and the underlying autoimmune process. Amongst, these are defects in the handling of apoptotic cells, increased expression of type I interferon-regulated genes and activation of autoreactive B cells, with both the type I interferon... (More)

Systemic lupus erythematosus (SLE) has a complex clinical picture, and a number of defects in the immune system have been described in patients with the disease. Most organs can be involved in SLE, and in addition to the typical major organ manifestations (e.g. from kidneys and the central nervous system), early cardiovascular disease is a major determinant of prognosis. Several important findings during the last decade have increased the understanding of the mechanisms behind the disease characteristics and the underlying autoimmune process. Amongst, these are defects in the handling of apoptotic cells, increased expression of type I interferon-regulated genes and activation of autoreactive B cells, with both the type I interferon system and the B lymphocyte stimulator (BLyS) having key roles. In addition, a large number of genes have been identified that contribute to these abnormalities. It has also become clear that certain SLE risk genes are associated with some organ manifestations, such as STAT4 with nephritis and IRF8 with myocardial infarction. Furthermore, environmental factors that can induce SLE or trigger a disease flare have been identified. As a consequence of this increased knowledge, new treatments for SLE have been developed. The most recently approved drug for SLE is belimumab, which blocks BLyS, and several new therapies and therapeutic strategies are in the pipeline for clinical application.

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author
and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Genes, Interferon, Pathogenesis, Systemic lupus erythematosus, Treatment
in
Journal of Internal Medicine
volume
281
issue
1
pages
52 - 64
publisher
Wiley-Blackwell
external identifiers
  • pmid:27307107
  • wos:000393950000005
  • scopus:84995792547
ISSN
0954-6820
DOI
10.1111/joim.12529
language
English
LU publication?
yes
id
46a23083-7fc6-4727-83ec-b47ced561bde
date added to LUP
2016-12-05 07:51:24
date last changed
2024-06-15 21:52:24
@article{46a23083-7fc6-4727-83ec-b47ced561bde,
  abstract     = {{<p>Systemic lupus erythematosus (SLE) has a complex clinical picture, and a number of defects in the immune system have been described in patients with the disease. Most organs can be involved in SLE, and in addition to the typical major organ manifestations (e.g. from kidneys and the central nervous system), early cardiovascular disease is a major determinant of prognosis. Several important findings during the last decade have increased the understanding of the mechanisms behind the disease characteristics and the underlying autoimmune process. Amongst, these are defects in the handling of apoptotic cells, increased expression of type I interferon-regulated genes and activation of autoreactive B cells, with both the type I interferon system and the B lymphocyte stimulator (BLyS) having key roles. In addition, a large number of genes have been identified that contribute to these abnormalities. It has also become clear that certain SLE risk genes are associated with some organ manifestations, such as STAT4 with nephritis and IRF8 with myocardial infarction. Furthermore, environmental factors that can induce SLE or trigger a disease flare have been identified. As a consequence of this increased knowledge, new treatments for SLE have been developed. The most recently approved drug for SLE is belimumab, which blocks BLyS, and several new therapies and therapeutic strategies are in the pipeline for clinical application.</p>}},
  author       = {{Bengtsson, Anders and Rönnblom, L.}},
  issn         = {{0954-6820}},
  keywords     = {{Genes; Interferon; Pathogenesis; Systemic lupus erythematosus; Treatment}},
  language     = {{eng}},
  number       = {{1}},
  pages        = {{52--64}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{Journal of Internal Medicine}},
  title        = {{Systemic lupus erythematosus : Still a challenge for physicians}},
  url          = {{http://dx.doi.org/10.1111/joim.12529}},
  doi          = {{10.1111/joim.12529}},
  volume       = {{281}},
  year         = {{2017}},
}