Lupus erythematosus

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Lupus erythematosus

Specialty Rheumatology
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Lupus erythematosus is a collection of autoimmune diseases in which the human immune

system becomes hyperactive and attacks healthy tissues.[1] Symptoms of these diseases can affect
many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs. The
most common and severe form is systemic lupus erythematosus.

Contents
 1 Signs and symptoms
o 1.1 Photosensitivity
 2 Genetics
o 2.1 Causes
o 2.2 Age difference
o 2.3 Differences in ethnicity
 3 Diagnosis
o 3.1 Classification
 4 Treatment
  5 Epidemiology
o 5.1 Worldwide
o 5.2 United Kingdom
o 5.3 United States
 6 See also
 7 References
 8 External links

Signs and symptoms

Symptoms vary from person to person, and may come and go. Almost everyone with lupus has
joint pain and swelling. Some develop arthritis. Frequently affected joints are the fingers, hands,
wrists, and knees. Other common symptoms include:

 chest pain during respiration

 joint pain
 oral ulcer
 fatigue
 fever with no other cause
 general discomfort, uneasiness, or ill feeling (malaise)
 hair loss
 sensitivity to sunlight
 skin rash – a "butterfly" rash in about half people with SLE
 swollen lymph nodes[2]

Photosensitivity

Photosensitivity is a known symptom of lupus, but its relationship to and influence on other
aspects of the disease remain to be defined.[3] Causes of photosensitivity may include:

 change in autoantibody location

 cytotoxicity
 inducing apoptosis with autoantigens in apoptotic blebs
 upregulation of adhesion molecules and cytokines
 inducing nitric oxide synthase expression
 ultraviolet-generated antigenic DNA.
 tumor necrosis factor alpha also seems to play a role in the development of
photosensitivity.

Genetics
Causes

It is typically believed that Lupus is influenced by multiple genes. Lupus is usually influenced by
gene polymorphisms, 30 of which have now been linked with the disorder. Some of these
polymorphisms have been linked very tentatively however, as the role that they play or the
degree to which they influence the disease is unknown. Other genes that are commonly thought
to be associated with Lupus are those in the Human leukocyte antigen (HLA) family, which are
largely related to healthy functioning of the immune system. There have been several cases
where a single gene influence appears to be present, but this is rare. When a single gene
deficiency does cause Lupus, it is usually attributed to the genes C1, C2, or C4. The influence of
sex chromosomes and environmental factors are also noteworthy. Usually, these factors
contribute to Lupus by compromising the immune system.[4]

Age difference

Lupus can develop in any age but most commonly in ages 15 to 44 with varying results.
Typically, the manifestation of the disease tends to be more acute in those affected who are of
younger age. Women are more likely to get it than men. Patients with juvenile onset Lupus in
particular, are vulnerable to mucocutaneous manifestations of the disease (alopecia, skin rash,
and ulceration of the mucus membranes) more so than any other age group. However, patients
with late onset Lupus have a much higher mortality rate. Nearly 50% of those with late onset
Lupus die of their affliction. Women who are of childbearing age are also particularly at risk.[5]

Differences in ethnicity

Substantial data have been found to indicate that certain ethnic populations could be more at risk
for Lupus Erythematosus, and have a better or worse prognosis. Asian, African, and Native
Americans are more likely to get Lupus than Caucasians. Caucasians seem to generally have a
more mild manifestation of the disease. Their survival rates after five years were typically
around 94%-96%, while patients of African, and some Asian ethnicities had survival rates closer
to 79%-92%. The only documented ethnicity that had a higher survival rate than Caucasians
were Koreans, who had survival rates nearer to 98%. Among Caucasians, the most common
causes of death were complications involving the cardiovascular system, the respiratory system
and problems with malignancies.[6][7] Atherosclerotic cardiovascular disease is more prevalent in
African American Lupus patients compared to Caucasians with Lupus.[8]

Diagnosis
For the diagnosis of lupus 4 out of 11 signs must be present.[9]

Testing may include:

 Antinuclear antibody (ANA)

 CBC with differential
 Chest x-ray
 Serum creatinine
 Urinalysis[9]

Classification
Lupus erythematosus may manifest as systemic disease or in a purely cutaneous form also
known as incomplete lupus erythematosus. Lupus has four main types:

 systemic
 discoid
 drug-induced
 neonatal

Of these, systemic lupus erythematosus (also known as SLE) is the most common and serious
form.

A more thorough categorization of lupus includes the following types:[10][11]

 acute cutaneous lupus erythematosus

 subacute cutaneous lupus erythematosus
 discoid lupus erythematosus (chronic cutaneous)
o
 childhood discoid lupus erythematosus
 generalized discoid lupus erythematosus
 localized discoid lupus erythematosus
o chilblain lupus erythematosus (Hutchinson)
o lupus erythematosus-lichen planus overlap syndrome
o lupus erythematosus panniculitis (lupus erythematosus profundus)
o tumid lupus erythematosus
o verrucous lupus erythematosus (hypertrophic lupus erythematosus)
o cutaneous lupus mucinosis
 complement deficiency syndromes
 drug-induced lupus erythematosus
 neonatal lupus erythematosus
 systemic lupus erythematosus

Treatment
Main article: Systemic lupus erythematosus § Treatment

Treatment consists primarily of immunosuppressive drugs (e.g., hydroxychloroquine and

corticosteroids). An interesting second line drug is methotrexate in its low-dose schedule.[12] In
2011, the U.S. Food and Drug Administration (FDA) approved the first new drug for lupus in
more than 50 years to be used in the US, belimumab.[13] In addition to medicative therapy, due to
the psychological and social impacts that Lupus may have on an individual, Cognitive
Behavioural Therapy (CBT) has also been demonstrated to be effective in reducing stress,
anxiety, and depression in lupus sufferers.[14]

Epidemiology
Worldwide

 an estimated 5 million people worldwide have some form of lupus disease.[15]

 70% of lupus cases diagnosed are systemic lupus erythematosus.[15]
 20% of people with lupus will have a parent or sibling who already has lupus or may
develop lupus.[15]
 about 5% of the children born to individuals with lupus will develop the illness.[15]

United Kingdom

 SLE affects UK females far more than males at a ratio of 7:1. In other words, females are
seven times more likely to have the disease.[16]
 The estimated number of UK females with SLE is 21,700, and the number of UK males
with lupus is 3000—a total of 24,700, or 0.041% of the population.[16]
 SLE is more common amongst certain ethnic groups than others, especially those of
African origin.[16]

United States

 occurs from infancy to old age, with peak occurrence between ages 15 and 40.[13]
 affects U.S. females 6 to 10 times more often than males.[13]
 yields limited prevalence data. Estimates vary and range from 1.8 to 7.6 cases per
100,000 persons per year in parts of the continental United States.[13]