Juvenile Rheumatoid Arthritis
Juvenile rheumatoid arthritis, JRA
When most people think of arthritis they think of a disease common in old people, but Juvenile Rheumatoid Arthritis (JRA) is a group of diseases that strikes the young.
•What is it?
JRA refers to a grab bag of at least five distinct arthritis diseases with at least seven patterns of illness. They can range from relatively mild conditions that disappear at puberty, to relentlessly progressive, crippling arthritis.
The exact cause of the arthritides in JRA remains unknown. The two leading theories are that the diseases are caused by infections with as-yet-unidentified microorganisms, or that the diseases are the result of an over-exuberant autoimmune reaction — one’s own immune system turning against oneself.
•Who gets it?
Some children inherit a genetic predisposition to JRA.
As stated earlier, there are at least five major subtypes of rheumatic disease that fall under the name JRA. Two forms of the disease are considered polyarticular (many-joint) rheumatoid arthritis. The great majority of children affected by this sub-type of JRA are girls.
The polyarticular diseases account for about 35 percent of children with JRA. The two forms of the polyarticular disease are differentiated by a blood test for an immune compound called the rheumatoid factor. Rheumatoid Factor Positive Polyarticular Disease tends to appear in late childhood. Rheumatoid Factor Negative Polyarticular JRA may begin at any time in childhood.
The next group of diseases are called pauciarticular (few-joints) diseases. There are two pauciarticular types of JRA. Pauciarticular Disease Type 1 is the single most common form of JRA; it accounts for 30 to 40 percent of children with JRA. Pauciarticular Type 1 appears typically in girls and usually begins before the fourth birthday. Pauciarticular Type 2 accounts for 10 to 15 percent of patients with JRA and appears predominantly in boys. It usually begins after the age of eight.
The fifth type of JRA is called Systemic JRA. This accounts for 10 to 20 percent of children with JRA and affects boys and girls equally.
•What are the symptoms?
All forms of rheumatoid arthritis are characterized by chronic swelling and inflammation of the synovial membrane that lines the joints. If this chronic inflammation continues over many years, the joint cartilage and joint bone become eroded and are gradually destroyed. The amount of time the chronic inflammation can last before the damage becomes permanent varies a great deal, but it is a much longer period in Juvenile Rheumatoid Arthritis compared to Adult Rheumatoid Arthritis. Many children with JRA outgrow the disease and never develop permanent joint damage.
The polyarticular diseases are characterized by involvement of multiple joints, typically including the small joints of the hands. Rheumatoid Factor Positive Polyarticular Disease tends to be the more severe of the two polyarticular diseases. Rheumatoid nodules often appear at the joints. Rheumatoid Factor Negative Polyarticular JRA is typically mild. It is rarely associated with rheumatoid nodules at the joint spaces. The affected joints are typically swollen and warm. They tend to be particularly stiff and painful in the morning and following periods of inactivity. Joint involvement in both polyarticular diseases is typically symmetric, with both sides of the body mirroring each other.
The pauciarticular diseases are characterized by a joint involvement that is limited to four or fewer joints for the first six months of the disease. The large joints are primarily affected, and the arthritis is typically asymmetric. About 30 percent of the children with Pauciarticular Type 1 develop eye complications. Children with Pauciarticular Disease Type 2 frequently have involvement of the hip joints. They may also develop eye complications.
Systemic JRA typically begins with high, spiking fevers and a fleeting, salmon-colored rash. The fever usually spikes once or twice daily, with chills and a rash during the fever spikes. Joint problems can be present at the beginning, but may also occur much later.
•Is it contagious?
JRA is not contagious itself, but it can be triggered by certain viruses such as parvovirus, rubella, and Epstein-Barr virus. These infections are contagious.
•How long does it last?
Many children with JRA will get better at puberty. Some children will have active arthritis that continues into adulthood. Overall, those with Rheumatoid Factor Positive Polyarthritis and Systemic JRA have the highest likelihood for ongoing symptoms. About 25 to 50 percent of the children with Rheumatoid Factor Positive Polyarthritis and Systemic JRA will be affected as adults.
For most children with JRA, the long-term outlook is good. Nevertheless, at best this chronic disease will last for several years.
•How is it diagnosed?
The diagnosis of JRA is suggested by the history and by objective signs of arthritis on the physical exam. Blood tests can help to confirm the diagnosis and to exclude other causes for these symptoms. Anemia, in the face of increased white blood cells and platelets, is often present at the time of diagnosis.
•How is it treated?
The goal of therapy is to provide symptomatic relief, to preserve long-term joint function, and to prevent related complications such as eye involvement. The primary medicines used are anti-inflammatory medicines and, more recently, low-dose chemotherapy drugs that seem to normalize the over-exuberant immune system. Physical and occupational therapies are also important components of treatment.
Early detection and treatment of eye problems can prevent scarring and preserve vision. All children with pauciarticular disease should have examinations by an ophthalmologist three to four times per year for the first five years of the disease.
It is important to find a caring and knowledgeable physician, skilled in the treatment of JRA, to coordinate treatment, closely monitor progress, and to frequently adjust treatments as necessary.
With most JRA patients, there is a difficult and crucial balance to be learned — when to be physically active in spite of pain and when to accept limitations.
•How can it be prevented?
JRA is difficult to prevent. Children with a genetic susceptibility may benefit from measures to modulate the immune system, such as breastfeeding, probiotics (beneficial bacteria as found in yogurt), avoiding tobacco smoke, and avoiding unnecessary antibiotics.
•Related A-to-Z Information:
Anemia (Low hemoglobin), Arboviruses, Bowlegs, Celiac disease, Clubfoot, Congenital hip dislocation, Diphtheria, Exanthems (Childhood rash), Fractures, Herpes (Human herpesvirus), Influenza (Flu), Lyme disease, Measles, Mononucleosis (Mono), Mumps, Parvovirus B19, Rheumatic Fever, Rickets, Sickle Cell Anemia, Strep Throat, Ticks, Toxic Synovitis, Type I Diabetes
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Date : 05 May 2016