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:[2][3]
- Acute cutaneous lupus erythematosus
- Subacute cutaneous lupus erythematosus
- Chronic cutaneous lupus erythematosus
- Discoid lupus erythematosus
- Childhood discoid 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)
- Discoid 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
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.
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.
- 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.
United Kingdom
See also: Systemic Lupus Erythematosus
- affects UK females far more than males at a ratio of 7:1, that is to say females are 7 times more likely to have the disease.[8]
- The estimated number of UK females with SLE is 21,900, and UK males with Lupus is 3000 - a total of 24,700, or 0.041% of the population.[8]
- SLE is more common amongst certain ethnic groups than others, especially those of African origin.[8]
United States
Systemic Lupus Erythematosus:
- occurs from infancy to old age, with peak occurrence between ages 15 and 40.
- affects US females far more than males at a ratio of 6-10:1, that is to say females are 6-10 times more likely to have the disease.
- prevalence data is limited. Estimates vary and range from 1.8 to 7.6 cases per 100,000 persons per year in parts of the continental United States.
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