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Lupus erythematosus

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Lupus erythematosus

Lupus erythematosus (Lupus)
Classification and external resources
Specialty Rheumatology
ICD-10 L93 (ILDS L93.010)
ICD-9-CM 695.4

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.[1] 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 systemic disease or in a purely cutaneous form also known as incomplete lupus erythematosus. Lupus has four main types:

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:[2][3]

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:

Genetics

The major histocompatibility complex (MHC) on chromosome 6, which contains the human lymphocyte antigens (HLA), was the first described genetic link to SLE.[1] The protein products of the HLA genes are critical components of cell-to-cell communication in the immune system. Indeed, in some cases, HLA genes are more highly related to lupus-associated autoantibodies than to the disease itself. Nonetheless, carriage of specific alleles of HLA imparts about a 2-fold risk of SLE above that of the general population.

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.[6]

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. It is more common for a Native American, African, or Asian person to get Lupus than a Caucasian. 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 mortality rate. Nearly 50% of those with late onset Lupus died of their affliction. 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. 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.[8][9]

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.[10]

Epidemiology

Worldwide

  • an estimated 5 million people worldwide have some form of lupus disease.[11]
  • 70% of lupus cases diagnosed are systemic lupus erythematosus.[11]
  • 20% of people with lupus will have a parent or sibling who already has lupus or may develop lupus.[11]
  • about 5% of the children born to individuals with lupus will develop the illness.[11]

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.[12]
  • 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.[12]
  • SLE is more common amongst certain ethnic groups than others, especially those of African origin.[12]

United States

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

See also

References

  1. ^
  2. ^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Disease of the Skin: Clinical Dermatology. (10th ed.). Saunders. Chapter 8. ISBN 0-7216-2921-0.
  3. ^
  4. ^ http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001471/
  5. ^
  6. ^
  7. ^
  8. ^
  9. ^ "Lupus Facts, Symptoms, Rash, Effects, Complications, and More." WebMD, n.d. Web. 6 October 2014. ;.
  10. ^ a b c d http://www.cdc.gov/arthritis/basics/lupus.htm
  11. ^ a b c d http://www.lupus.org/webmodules/webarticlesnet/templates/new_newsroomreporters.aspx?articleid=247&zoneid=60
  12. ^ a b c http://www.arthritisresearchuk.org/arthritis-information/data-and-statistics/lupus.aspx

External links

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