Lupus erythematosus
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is a collection of autoimmune diseases in which the
human immune system The immune system is a network of biological systems that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to bacteria, as well as cancer cells, parasitic worms, and also objects such a ...
becomes hyperactive and attacks healthy tissues. Symptoms of these diseases can affect many different body systems, including
joint A joint or articulation (or articular surface) is the connection made between bones, ossicles, or other hard structures in the body which link an animal's skeletal system into a functional whole.Saladin, Ken. Anatomy & Physiology. 7th ed. McGraw- ...
s,
skin Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation. Other animal coverings, such as the arthropod exoskeleton, have different ...
,
kidney In humans, the kidneys are two reddish-brown bean-shaped blood-filtering organ (anatomy), organs that are a multilobar, multipapillary form of mammalian kidneys, usually without signs of external lobulation. They are located on the left and rig ...
s, blood cells,
heart The heart is a muscular Organ (biology), organ found in humans and other animals. This organ pumps blood through the blood vessels. The heart and blood vessels together make the circulatory system. The pumped blood carries oxygen and nutrie ...
, and
lung The lungs are the primary Organ (biology), organs of the respiratory system in many animals, including humans. In mammals and most other tetrapods, two lungs are located near the Vertebral column, backbone on either side of the heart. Their ...
s. The most common and most severe form is .


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 A joint or articulation (or articular surface) is the connection made between bones, ossicles, or other hard structures in the body which link an animal's skeletal system into a functional whole.Saladin, Ken. Anatomy & Physiology. 7th ed. McGraw- ...
are the fingers, hands, wrists, and knees. Other common symptoms include: *
chest pain Chest pain is pain or discomfort in the chest, typically the front of the chest. It may be described as sharp, dull, pressure, heaviness or squeezing. Associated symptoms may include pain in the shoulder, arm, upper abdomen, or jaw, along with n ...
during respiration * joint pain (stiffness and swelling) * painless oral ulcer *
fatigue Fatigue is a state of tiredness (which is not sleepiness), exhaustion or loss of energy. It is a signs and symptoms, symptom of any of various diseases; it is not a disease in itself. Fatigue (in the medical sense) is sometimes associated wit ...
*
weight loss Weight loss, in the context of medicine, health, or physical fitness, refers to a reduction of the total body mass, by a mean loss of fluid, body fat (adipose tissue), or lean mass (namely bone mineral deposits, muscle, tendon, and other conn ...
* headaches *
fever Fever or pyrexia in humans is a symptom of an anti-infection defense mechanism that appears with Human body temperature, body temperature exceeding the normal range caused by an increase in the body's temperature Human body temperature#Fever, s ...
with no other cause * Skin lesions that appear worse after sun exposure * general discomfort, uneasiness, or ill feeling (
malaise In medicine, malaise is a feeling of general discomfort, uneasiness or lack of wellbeing and often the first sign of an infection or other disease. It is considered a vague termdescribing the state of simply not feeling well. The word has exist ...
) * hair loss * sensitivity to sunlight * a "butterfly" facial rash, seen in about half of people with SLE * swollen lymph nodes


Photosensitivity

Photosensitivity is the amount to which an object reacts upon receiving photons especially in visible light. Photosensitivity is a known symptom of lupus, but its relationship to and influence on other aspects of the disease remain to be defined. Causes of photosensitivity may include: * change in autoantibody location *
cytotoxicity Cytotoxicity is the quality of being toxic to cells. Examples of toxic agents are toxic metals, toxic chemicals, microbe neurotoxins, radiation particles and even specific neurotransmitters when the system is out of balance. Also some types of d ...
* induction of
apoptosis Apoptosis (from ) is a form of programmed cell death that occurs in multicellular organisms and in some eukaryotic, single-celled microorganisms such as yeast. Biochemistry, Biochemical events lead to characteristic cell changes (Morphology (biol ...
with autoantigens in apoptotic blebs *
upregulation In biochemistry, in the biological context of organisms' regulation of gene expression and production of gene products, downregulation is the process by which a cell decreases the production and quantities of its cellular components, such as R ...
of adhesion molecules and
cytokines Cytokines () are a broad and loose category of small proteins (~5–25 kDa) important in cell signaling. Cytokines are produced by a broad range of cells, including immune cells like macrophages, B cell, B lymphocytes, T cell, T lymphocytes ...
* induction of nitric oxide synthase expression *
ultraviolet Ultraviolet radiation, also known as simply UV, is electromagnetic radiation of wavelengths of 10–400 nanometers, shorter than that of visible light, but longer than X-rays. UV radiation is present in sunlight and constitutes about 10% of ...
-generated antigenic DNA


Genetics

It is typically believed that lupus is influenced by multiple genes. Lupus is usually influenced by
gene polymorphism A gene is said to be polymorphic if more than one allele occupies that gene's Locus (genetics), locus within a population. In addition to having more than one allele at a specific locus, each allele must also occur in the population at a rate of ...
s, 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. There have been several cases wherein 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 complement protein genes ''C1'', ''C2'', or ''C4''. The influence of sex chromosomes and environmental factors are also noteworthy. Usually, these factors contribute to lupus by influencing the immune system. Several studies also indicate a potential association of lupus with mutations in DNA repair genes.


Age difference

Lupus can develop in people at any age, but it does most commonly at ages 15 to 44, with varying results. Typically, the manifestation of the disease tends to be more acute in those of younger age. Women are more likely to get it than men. Patients with juvenile-onset lupus are more vulnerable to mucocutaneous manifestations of the disease (alopecia, skin rash, and ulceration of the mucous membranes) than any other age group, and they are also more susceptible to evaluation of pulmonary artery pressure. However, patients with late-onset lupus have a much higher mortality rate. Nearly 50% of those with late-onset lupus die of their condition. Women who are of childbearing age are also particularly at risk.


Differences in ethnicity

Substantial data have been found to indicate that certain ethnic populations could be more at risk for and to have a better or worse prognosis. Asian, African, and Native Americans are more likely to get lupus than Caucasians. Caucasians seem generally to have a milder 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 ethnic group that had a higher survival rate than Caucasians was 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 malignancies. Atherosclerotic cardiovascular disease is more prevalent in African Americans with lupus than in Caucasians with lupus.


Diagnosis

Diagnosis of lupus will vary from person to person. It is common to be diagnosed with other illnesses before a doctor can finally give a diagnosis of lupus because a lot of the symptoms overlap with other common illness. Diagnosis of requires a
physical examination In a physical examination, medical examination, clinical examination, or medical checkup, a medical practitioner examines a patient for any possible medical signs or symptoms of a Disease, medical condition. It generally consists of a series of ...
, blood and urine tests, and a skin or kidney biopsy. Some other tests that may need to be run include: * Antinuclear antibody (ANA) * CBC with differential *
Chest X-ray A chest radiograph, chest X-ray (CXR), or chest film is a Projectional radiography, projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures. Chest radiographs are the most common fi ...
* Serum creatinine *
Urinalysis Urinalysis, a portmanteau of the words ''urine'' and ''analysis'', is a Test panel, panel of medical tests that includes physical (macroscopic) examination of the urine, chemical evaluation using urine test strips, and #Microscopic examination, m ...


Classification

may manifest as systemic disease or in a purely cutaneous form also known as ''incomplete ''. Lupus has four main types: * systemic * discoid * drug-induced *
neonatal In common terminology, a baby is the very young offspring of adult human beings, while infant (from the Latin word ''infans'', meaning 'baby' or 'child') is a formal or specialised synonym. The terms may also be used to refer to Juvenile (orga ...
Of these, (also known as SLE) is the most common and serious form. A more thorough categorization of lupus includes the following types:James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Disease of the Skin: Clinical Dermatology''. (10th ed.). Saunders. Chapter 8. . * Childhood-onset systemic * acute cutaneous * * (chronic cutaneous) *** childhood *** generalized *** localized ** (Hutchinson) ** ** () ** ** ( hypertrophic ) ** * complement deficiency syndromes * drug-induced * neonatal * systemic


Treatment

There is still no cure for lupus but there are options to help control symptoms. The goal for treatment is to prevent flare ups and reduce organ damage. Doctors may prescribe a handful of different medications to help with their patients' symptoms. Some medications are: * Nonsteroidal anti-inflammatory drugs (NSAIDs). * Corticosteroids * Antimalarial drugs * BLyS-specific inhibitors * Immunosuppressive agents/chemotherapy After being diagnosed some treatment options that may be offered are: Treatment consists primarily of immunosuppressive drugs (e.g., hydroxychloroquine and corticosteroids). A second-line drug is methotrexate in its low-dose schedule. In 2011, the U.S.
Food and Drug Administration The United States Food and Drug Administration (FDA or US FDA) is a List of United States federal agencies, federal agency of the United States Department of Health and Human Services, Department of Health and Human Services. The FDA is respo ...
(FDA) approved the first new drug for lupus in more than 50 years to be used in the US,
belimumab Belimumab, sold under the brand name Benlysta, is a human monoclonal antibody that inhibits B-cell activating factor (BAFF), also known as B-lymphocyte stimulator (BLyS). It is approved in the United States and Canada, and the European Union to ...
. In addition to medical therapy,
cognitive behavioral therapy Cognitive behavioral therapy (CBT) is a form of psychotherapy that aims to reduce symptoms of various mental health conditions, primarily depression, PTSD, and anxiety disorders. Cognitive behavioral therapy focuses on challenging and chang ...
has also been demonstrated to be effective in reducing stress, anxiety, and depression due to the psychological and social impacts that lupus may have. People with SLE treated with standard care experience a higher risk of opportunistic infections and death than the general population. This risk is higher in men and in African Americans.


Epidemiology


Worldwide

* An estimated 5 million people worldwide have some form of lupus disease. * 70% of lupus cases diagnosed are . * 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

* Females in the UK are seven times more likely to be diagnosed with SLE than males. * The estimated number of females in the UK with SLE is 21,700, and the number of males is 3000 — a 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

* Lupus occurs from infancy to old age, with peak occurrence between ages 15 and 40. * Lupus affects females in the US 6 to 10 times more often than males. * Prevalence data are 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.


In popular culture

* In the early seasons of the television show ''
House A house is a single-unit residential building. It may range in complexity from a rudimentary hut to a complex structure of wood, masonry, concrete or other material, outfitted with plumbing, electrical, and heating, ventilation, and air c ...
'', members of the eponymous character's medical team often suggested lupus as a diagnosis for their patients, only to be rebuked. The rarity of legitimate lupus diagnoses in the show eventually became described as a running gag.


See also

*
List of cutaneous conditions Many skin conditions affect the human integumentary system—the organ system covering the entire surface of the Human body, body and composed of Human skin, skin, hair, Nail (anatomy), nails, and related muscle and glands. The major function o ...
* List of target antigens in pemphigoid * List of immunofluorescence findings for autoimmune bullous conditions * List of human leukocyte antigen alleles associated with cutaneous conditions * List of people with lupus


References


External links

{{Authority control Autoimmune diseases Connective tissue diseases de:Lupus erythematodes