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1.1. Background
Systemic lupus erythematous (SLE) is a multisystem autoimmune disorder
that is characterized by widespread immune dysregulation, formation of
autoantibodies and immune complexes, resulting in inflammation and potential
damage to a variety of organs. It is not uncommon for children to present with
non-specific symptoms, and little else, and be treated for a presumed infection,
including tuberculosis with subsequent evaluation revealing the diagnosis of
lupus, requiring aggressive management. A high index of suspicion must be
maintained for the diagnosis of SLE in adolescent children, particularly girls.
Systemic lupus erythematosus (SLE) derives its name from the typical facial rash,
which resembles the malar erythema of a wolf. It is a multi system involving
autoimmune connective tissue disease of unknown etiology. It is characterized by
the marked diversity and heterogeneity in clinical course as well as presentation.
The clinical pattern of lupus has been changed dramatically in recent decades
from originally an acute fatal disease to the chronic inflammatory autoimmune
disease, occasionally fatal. It is somewhat like an inflammatory syndrome for
there is no single abnormality that can definitely establish the diagnosis. As a
result criteria have been developed and modified in an attempt to make a precise
diagnosis with high sensitivity and specificity.
Pediatric SLE (pSLE) represents approximately 15-20 % of all SLE patients. It
is more common in females than in males, with a female to male ratio varying
from 2.3:1 to 9:1, depending on the study. The incidence of the disease varies
according to different ethnic groups. SLE is more common in African-American
females. McCarty DJ etc. reported the annual incidence of SLE is two and half
times more in African-American females than that in white females. Also in
Oriental population in North America, the disease appears to be aggressive and is
associated with a higher mortality. According to a survey conducted in two
decades ago in Shanghai, the prevalence of the disease is around 70 per 100,000
people and 113 per 100,000 in female population. It is estimated that there are

one million patients in China. Both genetic factors and sex hormone are
contributed to the developing of the disease. If a family member has SLE, the
likelihood of SLE increases with approximately 30 % for identical twins and 5%
for other first-degree relatives. In Caucasian females the incidence of SLE with
onset before age of 19 years is between 6 and 18.9 cases per 100,000, while it is
20-30 per 100,000 in African American females and 16-36.7 per 100,000 in
Puerto Rican females. The diagnosis of pSLE is rare before the age of 10, and the
average age at presentation is 12.1 years. Disease damage and mortality in pSLE
have been linked to different risk factors which include young age at diagnosis,
male sex and non-white ethnicity (African American, Asian, and Hispanic).
African Americans tend to have a greater prevalence and a more severe renal and
neuropsychiatric disease (NPSLE). However, the association between any of
these risk factors and a poor prognosis remains controversial.
Although SLE can occur at any age, more than 60 % of patients experience the
onset of disease between ages from 13 to 40 years old and female patients are
predominant which accounts 90 % of the patient population. So it is also a
disease that mainly involves the women of childbearing age. Anyhow the illness
in male is no milder despite the low incidence.