Juvenile rheumatoid arthritis is a form of chronic arthritis in children that is also referred to as Still’s disease, juvenile chronic polyarthritis, and juvenile chronic arthritis. It rarely begins before the age of one year, but it may start anytime after the first year. There are different types of juvenile rheumatoid arthritis that begin at different ages and more often in one sex or the other. It has been estimated that 5 percent of all cases of rheumatoid arthritis begin before the age of sixteen.
Juvenile rheumatoid arthritis (JRA) is defined as the onset of the disease under age sixteen. It has some features that distinguish it from rheumatoid arthritis (RA) in adults. These clinical differences include high fever, rash, high white blood cell count, eye involvement, growth disturbances, and increased tendency to have the disease localize in a single joint. In about 20 percent of cases the onset is acute and rapidly progressing, with general symptoms of illness such as fever and rash associated with enlargement of the spleen and inflammation of the pericardial sac around the heart, and in some cases these manifestations may be present for several weeks before the joint inflammation is evident.
In one third of these cases the disease begins in a single joint (monoarticular), and in half the cases the onset resembles adult RA and includes more than one joint (polyarticular). Pauciarticular JRA involves only a few joints and may affect the eyes. Skeletal growth in bones adjacent to inflamed joints may be either accelerated or retarded. If the heart is affected, the illness may be confused with acute rheumatic fever, but JRA frequently begins before age five, the joint symptoms do not migrate from joint to joint, the vertebrae in the neck are involved, the white blood cells are markedly increased, and rash frequently occurs.
The synovial tissue lining the joints becomes inflamed and the synovitis may last for weeks, months, or years. If the synovitis persists for a long enough period of time, the cartilage at the ends of the bones within the joint and the bone underlying the cartilage may be damaged. The cartilage may not regenerate at all, although it does have slight regenerative properties. Damage to the ligaments and tendons surrounding joints may also occur. Permanent joint damage occurs because the bones may be dislocated or fused together, tissue may be destroyed, and muscles and other structures surrounding the joint may shorten (contractures), causing deformities in the absence of damage within the joints. These serious complications do not occur often, and most children do not sustain permanent joint damage or deformity.
The diagnosis is based solely on the presence of the characteristic clinical findings and the careful exclusion of other conditions that also are associated with arthritis and joint pains. Destructive joint changes, occurring late in severe cases of the disease will be noted in X-rays, but there are no other specific diagnostic tests that establish the diagnosis.
Complete remission occurs in 50 percent or more of the children with JRA. The major consequences of this illness are permanently restricted joint function, heart disease, and chronic eye inflammation.
At least 75 percent of the children who have JRA will recover from their illness without having any important residual disability if they are given what is generally accepted as proper care during active periods of their illness. Currently approved methods of treatment are symptomatic – directed against the symptoms of the disease – and not curative. This is because the underlying factors responsible for JRA are poorly understood.
Treatment of this chronic illness consists of medication, physical and occupational therapy, psychological reinforcement (to offset the discouraging aspects of JRA), and surgery (to reconstruct joint damage). Education and counseling are important because the affected child’s needs and limitations may have a great impact on family activities and the uncertainty of the diagnosis and prognosis cause severe psychological stress for everyone concerned.
Although cortisone-related (steroid) drugs such as Prednisone can often work magic in this illness, their use must be limited to the severe manifestations that do not respond to aspirin and other non-steroid drugs. The possibility of cardiac damage and loss of vision require careful medical supervision. Another serious problem associated with steroid therapy is that steroids cause growth retardation, and although they may be necessary in the management of this illness, they may augment the growth retardation that is a natural feature of JRA.
It is possible that what we call JRA is more than one disease. If your child is among the estimated 250,000 cases in the United States, the specialist you consult will review this matter with you in depth, since each type of the illness has certain physical and laboratory characteristics, follows a particular course, affects more girls than boys or vice versa, and is associated with different kinds of complications.
*6/295/5*
RELIEVING ARTHRITIS: JUVENILE RHEUMATOID ARTHRITISJuvenile rheumatoid arthritis is a form of chronic arthritis in children that is also referred to as Still’s disease, juvenile chronic polyarthritis, and juvenile chronic arthritis. It rarely begins before the age of one year, but it may start anytime after the first year. There are different types of juvenile rheumatoid arthritis that begin at different ages and more often in one sex or the other. It has been estimated that 5 percent of all cases of rheumatoid arthritis begin before the age of sixteen.Juvenile rheumatoid arthritis (JRA) is defined as the onset of the disease under age sixteen. It has some features that distinguish it from rheumatoid arthritis (RA) in adults. These clinical differences include high fever, rash, high white blood cell count, eye involvement, growth disturbances, and increased tendency to have the disease localize in a single joint. In about 20 percent of cases the onset is acute and rapidly progressing, with general symptoms of illness such as fever and rash associated with enlargement of the spleen and inflammation of the pericardial sac around the heart, and in some cases these manifestations may be present for several weeks before the joint inflammation is evident.In one third of these cases the disease begins in a single joint (monoarticular), and in half the cases the onset resembles adult RA and includes more than one joint (polyarticular). Pauciarticular JRA involves only a few joints and may affect the eyes. Skeletal growth in bones adjacent to inflamed joints may be either accelerated or retarded. If the heart is affected, the illness may be confused with acute rheumatic fever, but JRA frequently begins before age five, the joint symptoms do not migrate from joint to joint, the vertebrae in the neck are involved, the white blood cells are markedly increased, and rash frequently occurs.The synovial tissue lining the joints becomes inflamed and the synovitis may last for weeks, months, or years. If the synovitis persists for a long enough period of time, the cartilage at the ends of the bones within the joint and the bone underlying the cartilage may be damaged. The cartilage may not regenerate at all, although it does have slight regenerative properties. Damage to the ligaments and tendons surrounding joints may also occur. Permanent joint damage occurs because the bones may be dislocated or fused together, tissue may be destroyed, and muscles and other structures surrounding the joint may shorten (contractures), causing deformities in the absence of damage within the joints. These serious complications do not occur often, and most children do not sustain permanent joint damage or deformity.The diagnosis is based solely on the presence of the characteristic clinical findings and the careful exclusion of other conditions that also are associated with arthritis and joint pains. Destructive joint changes, occurring late in severe cases of the disease will be noted in X-rays, but there are no other specific diagnostic tests that establish the diagnosis.Complete remission occurs in 50 percent or more of the children with JRA. The major consequences of this illness are permanently restricted joint function, heart disease, and chronic eye inflammation.At least 75 percent of the children who have JRA will recover from their illness without having any important residual disability if they are given what is generally accepted as proper care during active periods of their illness. Currently approved methods of treatment are symptomatic – directed against the symptoms of the disease – and not curative. This is because the underlying factors responsible for JRA are poorly understood.Treatment of this chronic illness consists of medication, physical and occupational therapy, psychological reinforcement (to offset the discouraging aspects of JRA), and surgery (to reconstruct joint damage). Education and counseling are important because the affected child’s needs and limitations may have a great impact on family activities and the uncertainty of the diagnosis and prognosis cause severe psychological stress for everyone concerned.Although cortisone-related (steroid) drugs such as Prednisone can often work magic in this illness, their use must be limited to the severe manifestations that do not respond to aspirin and other non-steroid drugs. The possibility of cardiac damage and loss of vision require careful medical supervision. Another serious problem associated with steroid therapy is that steroids cause growth retardation, and although they may be necessary in the management of this illness, they may augment the growth retardation that is a natural feature of JRA.It is possible that what we call JRA is more than one disease. If your child is among the estimated 250,000 cases in the United States, the specialist you consult will review this matter with you in depth, since each type of the illness has certain physical and laboratory characteristics, follows a particular course, affects more girls than boys or vice versa, and is associated with different kinds of complications.*6/295/5*