Lupus erythematosus

Lupus erythematosus
Lupus erythematosus
Classification and external resources
[1]

ICD-10

L93

ICD-9

695.4

(ILDS L93.010)

[2]

Lupus erythematosus is a name given to a collection of autoimmune diseases in which the human immune system
becomes hyperactive and attacks normal, healthy tissues. Symptoms of these diseases can affect many different body
systems, including joints, skin, kidneys, blood cells, heart, and lungs.

Classification
Lupus erythematosus may manifest as a 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:[3]
acute cutaneous lupus erythematosus
subacute cutaneous lupus erythematosus
chronic cutaneous lupus erythematosus
discoid lupus erythematosus
childhood discoid lupus erythematosus

Lupus erythematosus

generalized discoid lupus erythematosus

localized discoid lupus erythematosus
chilblain lupus erythematosus (Hutchinson)
lupus erythematosus-lichen planus overlap syndrome
lupus erythematosus panniculitis (lupus erythematosus profundus)
tumid lupus erythematosus
verrucous lupus erythematosus (hypertrophic lupus erythematosus)
complement deficiency syndromes
drug-induced lupus erythematosus
neonatal lupus erythematosus
systemic lupus erythematosus

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 when taking a deep breath

fatigue
fever with no other cause
general discomfort, uneasiness, or ill feeling (malaise)
hair loss
mouth sores
sensitivity to sunlight
skin rash - a "butterfly" rash in about half people with SLE.
swollen lymph nodes[4]

Photosensitivity
Photosensitivity is a known symptom of lupus, but its relationship to and influence on other aspects of the disease
remain to be defined.[5] 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

Lupus erythematosus
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.[6]

Age Difference
Lupus can develop in any age group with varying results. Typically, the manifestation of the disease tends to be
more acute in those affected who are of younger age. Patients with juvenile onset Lupus in particular, are vulnerable
to mucocutaneous (alopecia, skin rash, and ulceration of the mucus membranes) manifestations of the disease more
so than any other age group. However, patients with late onset Lupus have a much higher morbidity rate. Nearly
50% of those with late onset Lupus died of their infection. This is most likely due to the age of the patients with late
onset Lupus since the manifestation of their disease is much less severe than younger patients. Women who are of
childbearing age are also particularly at risk.[7]

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. 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 of 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.[8]

Treatment
Main article: Systemic Lupus Erythematosus Treatment
Treatment consists primarily of immunosuppressive drugs (e.g., hydroxychloroquine and corticosteroids). 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.

Epidemiology
Worldwide

an estimated 5 million people worldwide have some form of lupus.[9]

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

Lupus erythematosus

United Kingdom
See also: Systemic Lupus Erythematosus
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.[10]
The estimated number of UK females with SLE is 21,900, and the number of UK males with lupus is 3000a
total of 24,700, or 0.041% of the population.
SLE is more common amongst certain ethnic groups than others, especially those of African origin.

United States
Systemic lupus erythematosus:
occurs from infancy to old age, with peak occurrence between ages 15 and 40.[]
affects U.S. females 6 to 10 times more often than males.
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.

References
[1] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ L93
[2] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=695. 4
[3] James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Chapter 8.
ISBN 0-7216-2921-0.
[4] http:/ / www. ncbi. nlm. nih. gov/ pubmedhealth/ PMH0001471/
[5] Scheinfeld NS, Deleo V. Photosensitivity in lupus erythematosus. Photodermatol Photoimmunol Photomed. 2004;20:272-9. PMID 15379880
[6] Kiriakidou, Marianthi."Systemic Lupus Erythematosus." Annals Of Internal Medicine 159.7 (2013): 4-1-4-16. Academic Search Premier.
Web. 3 Mar. 2014.
[7] "Associations Of Clinical Features And Prognosis With Age At Disease Onset In Patients With Systemic Lupus Erythematosus." Lupus 23.3
(2014): 327-334. Academic Search Premier. Web. 3 Mar. 2014.
[8] "Survival In Systemic Lupus Erythematosus, 19952010. A Prospective Study In A Danish Community." Lupus 22.11 (2013): 1185-1191.
Academic Search Premier. Web. 3 Mar. 2014.
[9] http:/ / www. lupus. org/ webmodules/ webarticlesnet/ templates/ new_newsroomreporters. aspx?articleid=247& zoneid=60
[10] http:/ / www. arthritisresearchuk. org/ arthritis-information/ data-and-statistics/ lupus. aspx

External links
Lupus Foundation of America (http://www.lupus.org/)
Lupus UK (http://www.lupusuk.org.uk/)
The Lupus Site (http://www.thelupussite.com/)

Article Sources and Contributors

Article Sources and Contributors

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Image Sources, Licenses and Contributors

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