334

J.F. Arévalo et al.

Statistics data was 4.6 per million, whereas the

Initial Considerations

proportion of in-hospital mortality from the nation-

and Epidemiology

wide inpatient sample in the USA was 2.9%.

African-Americans with lupus have twoto three-

Systemic lupus erythematosus (SLE) is a chronic,

fold higher lupus mortality risk than Caucasians.

idiopathic, multisystem inflammatory disease

The magnitude of the risk disparity is dispropor-

characterized by hyperactivity of the immune sys-

tionately higher than the disparity in all-cause

tem and prominent autoantibody production.

mortality, so a lupus-specific biological factor, as

Acute exacerbations of disease activity are fol-

opposed to socioeconomic and access-to-care fac-

lowed by periods of remission, and its course and

tors, may be responsible for this phenomenon [4].

spectrum of clinical manifestations are variable.

The disease can present in a variety of forms rang-

Systemic Lupus Erythematosus:

ing from mild cutaneous and joint involvement to

lethal renal, cardiac, and cerebral involvement.

General Diagnosis

According to a population-based study in a

geographically defined population over a 42-year

The diagnosis can be definitively established if 4

period, the incidence of SLE has nearly tripled

of the 11 American College of Rheumatology cri-

over the past four decades. [1] The average inci-

teria are met (Table 18.1 and “Controversies and

dence rate (age and sex adjusted to the 1970 US

Perspectives” section). Although ocular disease is

white population) was 5.56 per 100,000 (95%

associated with SLE (it may even be the first clini-

CI=3.93–7.19) during the period from 1980 to

cal manifestation of SLE), ocular lesions are not

1992, as compared with an incidence of 1.51 (95%

included among the 11 diagnostic criteria. Foster

CI=0.85–2.17) during the period from 1950 to

believes that this is an oversight, and that inclu-

1979. In general, studies reporting higher inci-

sion of ocular manifestations among the diagnos-

dence rates utilize more comprehensive case

tic criteria for SLE would lead to earlier diagnosis

retrieval methods. Later, two studies of self-

and therapeutic intervention in those instances.

reported diagnoses of SLE indicated that the prev-

Nowadays, ocular manifestations have been

alence of SLE in the United States might be much

included as one of the clinical parameters used to

higher than previously reported. One of these stud-

assess disease activity in the British Isles Lupus

ies validated the self-reported diagnoses of SLE by

Assessment Group 2004 index (BILAG-2004);

reviewing available medical records revealing a

furthermore, some studies suggest that ocular

prevalence of 124 cases per 100,000 [2].

manifestations themselves are associated with

The average age of onset is 30 years, with a

disease activity in lupus and others have shown

range from infancy to old age. In general, seven

ocular problems to be associated with particular

women are stricken with the disease for every

clinical manifestations of SLE such as neurologic

man, and women of childbearing age are 11 times

involvement and the presence of anticardiolipin

as likely to be affected as men, compared with a

antibodies [5].

2:1 female-to-male ratio in prepubertal girls and

postmenopausal women. This raises the question

of a potential role for hormones as either a caus-

Pathogenesis and Laboratory Findings

ative or exacerbating factor in SLE.

The survival rates for individuals with this con-

Systemic lupus erythematosus may derive from a

dition has significantly improved due to earlier

dysfunction in immunoregulation in a genetically

diagnosis, recognition of mild disease, increased

predisposed individual and may be triggered by

utilization of antinuclear antibody testing, and bet-

environmental agents such as microbes or chemi-

ter approaches to therapy [3]. According to

cals. Over the past decade, several studies have

Krishnan and Hubert [4], the overall, unadjusted,

helped uncover genetic associations and suscep-

lupus mortality in the National Center for Health

tibility loci in human lupus. More recently,