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25% of cases progress to severe destructive arthritis. In the United States and Canada, mortality is estimated at about 4% and in Europe, mortality is estimated at 21.7%.
JIA occurs in both sexes, but like other rheumatological diseases, is more common in females. Symptoms onset is frequently dependent on the subtype of JIA and is from the preschool years to the early teenaged years.
The cause of JIA remains a mystery. However, the disorder is autoimmune — meaning that the body's own immune system starts to attack and destroy cells and tissues (particularly in the joints) for no apparent reason. The immune system is thought to be provoked by changes in the environment, in combination with mutations in many associated genes and/or other causes of differential expression of genes. Experimental studies have shown that certain mutated viruses may be able to trigger JIA. The disease appears to be more common in girls, and the disease is most common in Caucasians.
Associated factors that may worsen or have been linked to rheumatoid arthritis include:
- Genetic predisposition; When one family member has been diagnosed with rheumatoid arthritis or another autoimmune disorder, the chances are higher that other family members or siblings may also develop arthritis.
- Females are more likely to develop rheumatoid arthritis than males at all ages.
- A strong belief is held that psychological stress may worsen the symptoms of rheumatoid arthritis. However, when the emotional stress is under control, the arthritis symptoms do not always disappear, suggesting that the association is not straightforward.
- Though no distinct immune factor has been isolated as a cause of arthritis, some experts believe that the triggering factor may be something like a virus which then disappears from the body after permanent damage is done.
- Because rheumatoid arthritis is more common in women, perhaps sex hormones may play a role in causing or modulating arthritis. Unfortunately, neither sex hormone deficiency nor replacement has been shown to improve or worsen arthritis.
The cause of JIA, as the word "idiopathic" suggests, is unknown and an area of active research. Current understanding of JIA suggests that it arises in a genetically susceptible individual due to environmental factors.
In the US it affects about 250,000-294,000 children and teens making it one of the most common childhood diseases .
Of the children diagnosed with and treated for JDM, about half will recover completely. Close to 30 percent will have weakness after the disease resolves. Most children will go into remission and have their medications eliminated within two years, while others may take longer to respond or have more severe symptoms that take longer to clear up.
A common lasting effect of JDM is childhood arthritis.
The worldwide prevalence of inflammatory arthritis is approximately 3%. Rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, and undifferentiated spondyloarthritis are the most common subtypes of inflammatory arthritis. The diseases occur most commonly in the 30-40 age group.
There are several diseases where joint pain is primary, and is considered the main feature. Generally when a person has "arthritis" it means that they have one of these diseases, which include:
- Osteoarthritis
- Rheumatoid arthritis
- Gout and pseudo-gout
- Septic arthritis
- Ankylosing spondylitis
- Juvenile idiopathic arthritis
- Still's disease
Joint pain can also be a symptom of other diseases. In this case, the arthritis is considered to be secondary to the main disease; these include:
- Psoriasis (Psoriatic arthritis)
- Reactive arthritis
- Ehlers-Danlos Syndrome
- Haemochromatosis
- Hepatitis
- Lyme disease
- Sjogren's disease
- Hashimoto's thyroiditis
- Celiac disease
- Non-celiac gluten sensitivity
- Inflammatory bowel disease (including Crohn's disease and ulcerative colitis)
- Henoch–Schönlein purpura
- Hyperimmunoglobulinemia D with recurrent fever
- Sarcoidosis
- Whipple's disease
- TNF receptor associated periodic syndrome
- Granulomatosis with polyangiitis (and many other vasculitis syndromes)
- Familial Mediterranean fever
- Systemic lupus erythematosus
An "undifferentiated arthritis" is an arthritis that does not fit into well-known clinical disease categories, possibly being an early stage of a definite rheumatic disease.
Inflammatory arthritis can be disabling to the point where people with the diseases can lose their jobs, which can cause psychological distress. Because it is typically progressive, those who lose their jobs are unlikely to re-enter the workforce after leaving due to their diagnosis. Programs now aim to retain those with inflammatory arthritis by preventing work-related injuries and by making necessary accommodations in the workplace. A 2014 Cochrane review found low-quality evidence that work focused interventions, including counseling, education, advocacy, and occupational medicine consultations, were effective in retaining workers with inflammatory arthritis.
Mortality is increased in people with AS and circulatory disease is the most frequent cause of death. AS patients have an increased risk of 60% for cerebrovascular mortality, and an overall increased risk of 50% for vascular mortality. About one third of those with Ankylosing spondylitis have severe disease, which reduces life expectancy.
As increased mortality in ankylosing spondylitis is related to disease severity, factors negatively affecting outcomes include:
- Male sex
- Plus 3 of the following in the first 2 years of disease:
- Erythrocyte sedimentation rate (ESR) >30 mm/h
- Unresponsive to NSAIDs
- Limitation of lumbar spine range of motion
- Sausage-like fingers or toes
- Oligoarthritis
- Onset <16 years old
Juvenile arthritis, also known as Childhood arthritis (JA), is any form of chronic arthritis or chronic arthritis-related conditions which affects individuals under the age of 16. It is an autoimmune disease.
Systemic-onset juvenile idiopathic arthritis (also known as systemic juvenile idiopathic arthritis (sJIA) or the juvenile onset form of Still's disease) is a type of juvenile idiopathic arthritis (JIA) with extra-articular manifestations like fever and rash apart from arthritis. It was originally called systemic-onset juvenile rheumatoid arthritis or Still's disease.
Predominantly extra-articular manifestations like high fevers, rheumatic rash, enlargement of the liver and spleen, enlargement of the lymph nodes, and anemia. Others manifestations include inflammation of the pleura, inflammation of the pericardium, inflammation of the heart's muscular tissue, and inflammation of the peritoneum are also seen.
It is sometimes called "juvenile-onset Still's disease", to distinguish it from adult-onset Still's disease. However, there is some evidence that the two conditions are closely related.
The underlying cause of JDM is unknown. It most likely has a genetic component, as other autoimmune disease tend to run in the families of patients. The disease is usually triggered by a condition that causes immune system activity that does not stop as it should, but the trigger is almost certainly not the cause in most cases. Common triggers include immunizations, infections, injuries, and sunburn.
Between 0.1% and 1.8% of people are affected. The disease is most common in Northern European countries, and seen least in people of Afro-Caribbean descent. Although the ratio of male to female disease is reportedly 3:1, many rheumatologists believe the number of women with AS is underdiagnosed, as most women tend to experience milder cases of the disease. The majority of people with AS, including 95 percent of people of European descent with the disease, express the HLA-B27 antigen and high levels of immunoglobulin A (IgA) in the blood.
Adult-onset Still's Disease is rare and has been described all over the world. The number of new cases per year is estimated to be 1.6 per 1,000,000 population. The number of people currently affected is estimated at 1.5 cases per 100,000-1,000,000 population. Onset is most common in two age ranges, between ages 15–25 and between ages of 36–46 years.
Arthritis is the most common cause of disability in the USA. More than 20 million individuals with arthritis have severe limitations in function on a daily basis. Absenteeism and frequent visits to the physician are common in individuals who have arthritis. Arthritis can make it very difficult for individuals to be physically active and some become home bound.
It is estimated that the total cost of arthritis cases is close to $100 billion of which almost 50% is from lost earnings. Each year, arthritis results in nearly 1 million hospitalizations and close to 45 million outpatient visits to health care centers.
Decreased mobility, in combination with the above symptoms, can make it difficult for an individual to remain physically active, contributing to an increased risk of obesity, high cholesterol or vulnerability to heart disease. People with arthritis are also at increased risk of depression, which may be a response to numerous factors, including fear of worsening symptoms.
Adenocarcinoma of the bowel has been associated with coeliac disease.
Every year between 2.18 and 7.7 people per million receive a diagnosis of PM or DM. Around 3.2 children per million per year are diagnosed with DM (termed juvenile dermatomyositis), with an average age of onset of seven years. Diagnosis of adult DM commonly occurs between 30 and 50 years of age. PM is an adult disease, usually emerging after the age of twenty. PM and DM are more common in females, more common in Caucasians, and least common in Asians. At any given time, about 35.5 people per million have IBM; it emerges after the age of 30 (usually after 50), and may be more common in males.
The disease mechanism (pathophysiology) of RS3PE remains unknown. One study suggested a possible role for vascular endothelial growth factor. A study using magnetic resonance imaging found that tenosynovitis of the extensors of the hands and feet is the major contributor to edema.
Squamous carcinoma of the esophagus is more prevalent in coeliac disease. The increased prevalence may be secondary to GERD that results from chronic delayed gastric emptying. Other studies implicate the malabsorption of vitamin A and zinc as a result of multi-vitamin and mineral deficiencies seen in Coeliac disease.
Polymyositis and dermatomyositis are first treated with high doses of a corticosteroids
These are also referred to as systemic autoimmune diseases. The autoimmune CTDs may have both genetic and environmental causes. Genetic factors may create a predisposition towards developing these autoimmune diseases. They are characterized as a group by the presence of spontaneous overactivity of the immune system that results in the production of extra antibodies into the circulation. The classic collagen vascular diseases have a "classic" presentation with typical findings that doctors can recognize during an examination. Each also has "classic" blood test abnormalities and abnormal antibody patterns. However, each of these diseases can evolve slowly or rapidly from very subtle abnormalities before demonstrating the classic features that help in the diagnosis. The classic collagen vascular diseases include:
- Systemic lupus erythematosus (SLE) – An inflammation of the connective tissues, SLE can afflict every organ system. It is up to nine times more common in women than men and strikes black women three times as often as white women. The condition is aggravated by sunlight.
- Rheumatoid arthritis – Rheumatoid arthritis is a systemic disorder in which immune cells attack and inflame the membrane around joints. It also can affect the heart, lungs, and eyes. Of the estimated 2.1 million Americans with rheumatoid arthritis, approximately 1.5 million (71 percent) are women.
- Scleroderma – an activation of immune cells that produces scar tissue in the skin, internal organs, and small blood vessels. It affects women three times more often than men overall, but increases to a rate 15 times greater for women during childbearing years, and appears to be more common among black women.
- Sjögren's syndrome – also called Sjögren's disease, is a chronic, slowly progressing inability to secrete saliva and tears. It can occur alone or with rheumatoid arthritis, scleroderma, or systemic lupus erythematosus. Nine out of 10 cases occur in women, most often at or around mid-life.
- Mixed connective tissue disease – Mixed connective-tissue disease (MCTD) is a disorder in which features of various connective-tissue diseases (CTDs) such as systemic lupus erythematosus (SLE); systemic sclerosis (SSc); dermatomyositis (DM); polymyositis (PM); anti-synthetase syndrome; and, occasionally, Sjögren syndrome can coexist and overlap. The course of the disease is chronic and usually milder than other CTDs. In most cases, MCTD is considered an intermediate stage of a disease that eventually becomes either SLE or Scleroderma.
- Undifferentiated connective tissue disease (UCTD) is a disease in which the body mistakenly attacks its own tissues. It is diagnosed when there is evidence of an existing autoimmune condition which does not meet the criteria for any specific autoimmune disease, such as systemic lupus erythematosus or scleroderma. Latent lupus and incomplete lupus are alternative terms that have been used to describe this condition.
- Psoriatic arthritis is also a collagen vascular disease.
Myositis is inflammation or swelling of the muscles. Injury, medicines, infection, or an immune disorder can lead to myositis. It is a documented side effect of the lipid-lowering drugs statins and fibrates.
Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting disorder that typically affects the cervical lymph nodes. Recognition of this condition is crucial, especially because it can easily be mistaken for tuberculosis, lymphoma, or even adenocarcinoma. Awareness of this disorder helps prevent misdiagnosis and inappropriate treatment.
Kikuchi's disease is a very rare disease mainly seen in Japan. Isolated cases are reported in North America, Europe, and Asia. It is mainly a disease of young adults (20–30 years), with a slight bias towards females. The cause of this disease is not known, although infectious and autoimmune causes have been proposed. The course of the disease is generally benign and self-limiting. Lymph node enlargmeent usually resolves over several weeks to six months. Recurrence rate is about 3%. Death from Kikuchi disease is extremely rare and usually occurs due to liver, respiratory, or heart failure.
A person's sex also seems to have some role in the development of autoimmunity; that is, most autoimmune diseases are "sex-related". Nearly 75% of the more than 23.5 million Americans who suffer from autoimmune disease are women, although it is less-frequently acknowledged that millions of men also suffer from these diseases. According to the American Autoimmune Related Diseases Association (AARDA), autoimmune diseases that develop in men tend to be more severe. A few autoimmune diseases that men are just as or more likely to develop as women include: ankylosing spondylitis, type 1 diabetes mellitus, granulomatosis with polyangiitis, Crohn's disease, Primary sclerosing cholangitis and psoriasis.
The reasons for the sex role in autoimmunity vary. Women appear to generally mount larger inflammatory responses than men when their immune systems are triggered, increasing the risk of autoimmunity. Involvement of sex steroids is indicated by that many autoimmune diseases tend to fluctuate in accordance with hormonal changes, for example: during pregnancy, in the menstrual cycle, or when using oral contraception. A history of pregnancy also appears to leave a persistent increased risk for autoimmune disease. It has been suggested that the slight, direct exchange of cells between mothers and their children during pregnancy may induce autoimmunity. This would tip the gender balance in the direction of the female.
Another theory suggests the female high tendency to get autoimmunity is due to an imbalanced X chromosome inactivation. The X-inactivation skew theory, proposed by Princeton University's Jeff Stewart, has recently been confirmed experimentally in scleroderma and autoimmune thyroiditis. Other complex X-linked genetic susceptibility mechanisms are proposed and under investigation.
Serositis is seen in numerous conditions:
- Lupus erythematosus (SLE), for which it is one of the criteria,
- Rheumatoid arthritis
- Familial Mediterranean fever (FMF)
- Chronic renal failure
- Juvenile idiopathic arthritis
- Inflammatory bowel disease (especially Crohn's disease)
- Acute appendicitis
- Diffuse cutaneous systemic sclerosis