What is juvenile rheumatoid arthritis?

Causes and Symptoms

Juvenile rheumatoid arthritis is an autoimmune disease
of children that attacks the
joints. It appears in three different subgroups that vary according to severity and type of extra-articular manifestation. Other significant variations within these subgroups include age at disease onset, the sex of affected children, genetic predisposition, and prognosis.

The first major clinical pattern is systemic disease, which includes about 10 percent of children with juvenile rheumatoid arthritis. This type has the most dramatic onset and is the least common form of the illness. It affects boys more than girls and can begin at any age. The most characteristic manifestations are high, intermittent fevers and a temporary red rash occurring during periods of fever. Occasionally, more serious complications are involved. In some cases, systemic onset is marked by polyarthritis, which affects large and small multiple joints, and moves subsequently to disease of the knee or hip with no further involvement of other joints. The systemic complaints, which are often sudden and explosive in nature, may recur months or years later. Some children with systemic onset, however, never develop lasting arthritis.

Polyarticular juvenile rheumatoid arthritis, the second subgroup, includes children in whom five or more joints have been involved in the first six months of illness. It affects more girls than boys and can begin at any age. This subgroup includes about 40 percent of children with juvenile rheumatoid arthritis and is frequently mild. Within this subgroup exists a smaller subgroup, which includes about 5 percent of children with juvenile rheumatoid arthritis and rarely begins before the eighth birthday. The arthritis is often severe, with joint destruction occurring within the first year. Polyarthritis may involve swelling of the joints over a period of time, in which pain is not a prominent feature, or a sudden articular swelling, in which pain can be severe. Weight-bearing joints, usually knees and ankles, are often involved initially.

The third major subgroup is pauciarticular disease, which involves about 50 percent of all children with juvenile rheumatoid arthritis. In the initial episode of this type, only one joint is involved. More joints usually become involved within a few weeks or months, although occasionally involvement is restricted to one joint. Most often, the knee is the primary area, with the ankles and hips being the sites of minimal involvement.

Treatment and Therapy

The first step in treating juvenile rheumatoid arthritis is the identification of the child’s problems and potential problems, which include active joint disease, disabilities, ocular disease, growth retardation, and psychosocial disability. Because the cause of chronic arthritis is not known, treatment suppresses the symptoms and does not cure the disease itself.

Drug therapy is used to relieve inflammatory pain and immobility. The main type of medication used are the standard nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin, ibuprofen, and naproxen. If NSAIDs are ineffective, another pharmacological intervention is disease-modifying antirheumatic drugs (DMARDs), of which the most common is methotrexate. DMARDs slow the progression of the arthritis, but take a long time to act. Another powerful drug treatment is corticosteroids, administered either by mouth or injected directly into the joint; these are used sparingly, however, because of their serious side effects for children..

Physical and occupational therapy attempts to maintain strength and stamina and to preserve and increase the range of motion in the joints and in supporting muscles. All children with significant joint involvement should have a daily home program of activities and exercises directed toward the prevention and correction of disabilities that can be supervised by their parents. In addition to exercise, the program consists of the use of moist heat, adequate rest, and proper diet, which are aimed at maintaining strength. Children with severe disabilities may need hospitalization for intensive therapy.

Beyond physical therapy, orthopedic treatment for the joints may include splinting to preserve or repair joint motion. In the rehabilitation of older children who have suffered serious damage, the replacement of affected joints may provide good results.

Juvenile rheumatoid arthritis offers significant challenges for families. The family pediatrician or physician must act as teacher and adviser both to the patient and to family members. Other health care professionals who may be important to the care of a child with juvenile rheumatoid arthritis are ophthalmologists, pediatric nurses, social workers, and psychiatrists, as well as the child’s schoolteachers.

Perspective and Prospects

George R. Still’s finding in 1896 that juvenile rheumatoid arthritis includes at least three distinct joint afflictions first brought the subgroups to the attention of the medical profession and has fostered a greater understanding of the disease; in fact, systemic arthritis was originally known as Still's disease.

For most children with juvenile rheumatoid arthritis, early diagnosis and appropriate therapy point to a good prognosis. Of the children with systemic onset, at least 75 percent will enjoy a good outcome, whereas the rest may suffer severe arthritis, possibly resulting in disability. Between 80 and 90 percent of children with polyarthritis escape without permanent joint damage, although the disease itself may be chronic. The overall prospects for children with pauciarticular disease are not known.

Although most children suffering from juvenile rheumatoid arthritis eventually outgrow it, it is difficult for most parents to accept that accurate prediction for the ultimate outcome for their child is impossible. Nevertheless, a positive attitude, careful medical management, physical therapy, and psychological support can improve the quality of life for all children with juvenile rheumatoid arthritis.

Bibliography

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