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Copyright 2012. Nova Science Publishers, Inc. All rights reserved.

. May not be reproduced in any form without permission from the publisher, except fair uses permitted under Lupus Erythematosus 15

gene cluster [17]. Among the identified targets undergoing demethylation are genes involved
in autoreactivity (ITGAL), osmotic lysis and apoptosis (PRF1, MMP14 and LCN2), antigen
presentation (CSF3R), inflammation(MMP 14), B- and T-cell interaction (CD70 and
CD40LG) and cytokine pathways (CSF3R, IL-4, IL-6 and IFNGR2) [17]. DNA methylation
inhibitors are also known to induce autoreactivity in vitro and cause a lupus-like disease in
vivo. Further, altered patterns of histone modifications have been described in SLE. For
example, CD4 positive lymphocytes undergo global histone H3 and H4 deacetylation and
consequent skewing of gene expression [17]. Although multiple lines of evidence highlight
the contribution of epigenetic alterations to the pathogenesis of lupus in genetically
predisposed individuals, many questions remain to be answered. Attaining a deeper
understanding of this subject will create possibilities in the emerging area of epigenetic
treatments. It is difficult to estimate how many people in the United States have the disease,
because its symptoms vary widely and to date no obligatory governmental reporting system
exists. However, in other countries, extended studies are yielding some pertinent
epidemiologic data [18]. In one recent study in the Lugo region of northwestern Spain,
performed between January 1987 and December 2006, 150 residents were diagnosed as
having SLE according to the 1982 American College of Rheumatology (ACR) criteria for the
classification of SLE. Women outnumbered men (127 [84.7%] vs. 23 [15.3%]) [18]. The
mean age at the time of disease diagnosis was 46.1 19.6 years. The mean follow-up from
the time of disease diagnosis was 7.8 4.5 years. The age- and sex-adjusted annual incidence
rate over the 20-year study period was 3.6 (95% confidence interval [CI], 3.0-4.2) per
100,000 population aged 15 years and older [18]. The overall annual incidence rate over the
20-year study period in women (5.9/100,000 population aged 15 yr; 95% CI, 4.9-7.0) was
higher than in men (1.1/100,000 population aged 15 yr; 95% CI, 0.7-1.7) (p < 0.001) [18].
By December 31, 2006, the overall age-adjusted SLE prevalence in the Lugo region for
patients who fulfilled at least 4 of 1982 ACRC criteria was 17.5 per 100,000 population aged
15 years and older (95% CI, 12.6-24.1). Prevalence in women (29.2/100,000 population aged
15 yr; 95% CI, 20.0-40.7) was higher than in men (5.8/100,000 population aged 15 yr;
95% CI, 2.0-12.0).The most frequent clinical manifestation was arthritis [18]. As reported in
population-based studies on SLE patients of European descent, renal disease was observed in
only 27.3% of the patients. The rate of flares was 0.084/year. A younger age and the presence
of nephritis at the time of disease diagnosis were significantly associated with the
development of flares during the follow-up of Lugo patients. Compared with the general
population, the probability of survival in patients with SLE was significantly reduced (p =
0.04). In conclusion, the Lugo study establishes a baseline estimate of the incidence and
clinical spectrum of SLE in northwestern Spain. According to the results, the incidence of
SLE in northwestern Spain is slightly greater than that reported in most European regions.
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Patients with SLE from northwestern Spain have a later age of onset and a lower frequency of
nephritis than in the African-American population; however, the data show a reduced
probability of survival in Spanish patients with SLE [18]. Most studies have shown that about
1 in 20 people with lupus will have a close relative (mother, aunt, sister, brother; less often
father or uncle) with lupus. Occasionally the baby of a mother with lupus will be born with a
special form of lupus called neonatal lupus syndrome, due to the passage of certain antibodies
(anti-Ro and/or anti-La) from the mother to the baby during pregnancy. The neonatal form of
lupus only lasts a few months, as the baby destroys the antibodies from the mother and does

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