the child with arthritis

The child with arthritis Carol B. Lindsley, M.D.

THE NEW YORK ARTHRITIS FOUNDATlC

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rthritis in children can be caused A by several different diseases, but the most common form of chronic arthritis is juvenile rheumatoid arthritis. This disease is a systemic disease characterized by arthritis of variable patterns and may or may not have associated systemic manifestations. Recent reviewers in the field have questioned the con- cept of juvenile rheumatoid arthritis (JRA) as a single disease.'

ETIOLOGY AND EPIDEMIOLOGY The prevalence of JRA is estimated to be 1 in 10,000 to 1 in 1,000, depending on the source of the statistics. The estimated frequency is from 150,000 to 250,000 affected children in the United States. Disease onset has been reported to range from under 6 months of age to adulthood. Peak ranges of onset are between 1 and 4 years of age and between 9 and 14 years of age.2

The cause of JRA is still un- known. Onset frequently follows an upper respiratory infection or a trauma. However, no causative re- lationship between these events and the subsequent disease has been

identified. There have been isolated cases in which viral agents have been clearly associated with the onset of the disease; however, this has involved a relatively small number of patients. The current theories with regard to causes primarily fo- cus on ( 1 ) an as yet unidentified viral agent versus (2) an exaggerated or abnormal immune response, or (3) a combination of these two. No immune defect has been demon- strated in this disease.

There is an increased incidence of rheumatic disease in families of JRA patients, but no specific genetic pattern has been associated with the disease in general. There does appear to be a genetic predis- position associated with the HLA- B27 antigen for the development of pauciarticular arthritis in boys as well as ankylosing spondylitis in both sexes.3 In JRA as a whole, there is a 2:l to 3:l female to male ratio, although the sex ratio varies with different subtypes.

PATHOLOGY Histologic studies of involved syn- ovia reveal nonspecific chronic inflammatory changes..' There is microscopic evidence of edema, hy-

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T H E MUSCULOSKELETAL SYSTEM 25

peremia, and synovial cell hyper- plasia, as well as infiltration by mononuclear cells. The thickened synovial membrane may form villi which protrude into joint spaces.

CLINICAL MANIFESTATIONS The onset of JRA may be insidious or fulminant and any joint can be involved initially. However, if the patients are followed carefully during the first 6 months after onset, they can usually be grouped in one of three modes of onset: systemic, polyarticular, and pauciarticular.

Until recently the lack of specific diagnostic criteria for JRA has made comparison of different patient pop- ulations difficult and has added to the confusion in understanding the disease. Recently the JRA Criteria Committee of the American Rheu- matoid Association has proposed a revision for JRA criteria and the definitions used in the following description follow this p r~posa l .~

Systemic Onset JRA Approximately one-fourth of the children with JRA have systemic onset disease. This group includes children who have persistent, inter- mittent fever with elevations of 103 to 106'F, and/or rheumatoid rash. The fever must have persisted for at least 2 weeks and is characterized by daily spikes. In between these fever spikes, there is typically a drop to normal or subnormal levels. Usu- ally the spikes occur in the late afternoon or early evening and are associated with periods of severe dis- comfort and illness for the child. Because the daily variations of the

fever are characteristic of this disease, the fever pattern itself, charted every 4 hours, can be very helpful in initially suspecting or making the diagnosis of systemic onset JRA.

In the majority of patients who have systemic onset JRA, a rheumatoid rash is associated with fever. Occasionally a child has only the rash but is still included in the systemic onset subtype. The rash is characterized by small maculopapu- lar lesions generally from 3 to 4 mm in diameter and often with central clearing. There may be areas of con- fluence as well. The rash generally occurs over the anterior chest, axillae, buttocks, and to a lesser ex- tent on the extremities. It is usually nonpruritic. Its most characteristic feature is its evanescence. It will come and go in minutes to hours and is usually present with the fever spikes. Other common manifestations of systemic onset JRA include hepatosplenomegaly and lymph- adenopathy and, less frequently pleuritis, abdominal pain, and pericarditis. Pericarditis is usually tran- sient and benign and appears in approximately 10 percent of patients with JRA. The majority of these children have the systemic onset subtype. Occasionally it can be associated with severe clinical symptoms including dyspnea and chest pain and can be complicated by cardiac tamponade, myocarditis, and congestive heart failure. Although the incidence of clinically symptomatic patients with pericarditis is low, there is echocardiograph evidence of pericardial involvement in from 35 to 40 percent of the patients.6 This latter statistic is consis-

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26 ISSUES IN COMPREHENSIVE PEDIATRIC N U R S I N G

tent with earlier evidence that the pericardium was involved in approximately 40 percent of the patients at autopsy.'

Although generally more common in the younger age group, systemic onset disease has been reported in adults. I t is the only subtype that has a male predominance. T h e systemic manifestations are al- most always present at disease onset. Although a few patients may have a monocyclic cause, the majority will have recurrent episodes. Usu- ally the severity of the systemic manifestations diminish and the severity of the arthritic symptoms increase. Approximately half of the children will not have evidence of overt arthritis at the beginning of the disease, but the majority will develop polyarthritis within 6 months' time. Unusually long intervals, up to 9 years, between systemic manifestations and the onset o€ arthritis have been reported.2 T h e disease may be suspected with evidence of the systemic manifestations, but one cannot make a definitive diagnosis without the presence of arthritis. Although the children are frequently very toxic with the onset of systemic disease, the disease itself is rarely a cause for morbidity. About 25 percent of patients have morbidity from the arthritis itself on long-term follow-up.

Polyarticular Onset JRA This subtype is characterized by involvement of five or more joints during the first 6 months of disease and exclusion of the systemic sub- tY Pee

This disease subtype is character-

ized by female predominance and symmetrical involvement of multi- ple joints, frequently small joints of the hands and feet, and occurs in approximately 25 to 30 percent of patients with JRA. In this subtype the joint manifestations are domi- nant although children may show mild to moderate systemic symptoms. These are usually characterized by low-grade fever, malaise, and morning stiffness. By definition they do not have the high inter- mittent fever. Younger children particularly may have involvement of the tendon sheath and develop swelling in these areas. T h e wrist and ankle are the most common sites of this tenosynovitis. There is frequently early involvement of the cervical spine. This is helpful diag- nostically as inflammation of cervical spine rarely occurs in other types of childhood arthritis.

A subgroup of polyarticular J R A involves those who are seropositive, i.e., have positive tests for rheumatoid factor in their serum. These children tend to have older age of onset, a higher incidence of rheumatoid nodules, more severe progressive arthritis, and a high incidence of morbidity. Except for the age of onset, these children for all intents and purposes have a disease course very similar to adult rheuma toid arthritis.

Pauciarticular Onset JRA By definition this subtype encom- passes patients with involvement of four joints or less in the first 6 months of the disease. Again there is a female predominance in this subgroup. T h e most commonly af-

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THE MUSCULOSKELETAL SYSTEM 27

fected joints are the large weight- bearing joints, most frequently the knees and ankles. From 40 to 50 percent of children with JRA have pauciarticular onset. In recent years it has become apparent that there are two subgroups that are of particular interest. One subgroup is boys with pauciarticular arthritis and presence of HLA-B27 antigen. A recent study of Schaller et al. has shown that there is a significant incidence of this histocompatibility antigen in both boys with pauciarticular arthritis and in children with ankylosing spondylitis.s It is difficult if not impossible to sort out from JRA those children with ankylosing spondylitis who present with peripheral arthritis as their initial manifestation. Long-term studies will be needed to answer the question of what percentage of boys with pauciarticular arthritis and the HLA-B27 antigen do indeed develop ankylosing spondylitis.

The other subgroup of particular interest is young girls with pauciarticular arthritis and chronic iridocyclitis. Approximately 80 percent of the iridocyclitis that occurs in JRA occurs in this relatively small group.8 Iridocyclitis is the prime cause of morbidity in the pauciarticular group. It involves inflammation of the anterior uveal tract and may take one of two forms, either acute or chronic. The chronic form is the one associated with young girls who have pauciarticular arthritis. This form also has a high association with antinuclear antibodies. The incidence of positive antinuclear antibodies in patients with chronic iridocyclitis ranges

from 50 to 75 percent, thus making this laboratory test a marker for increased risk of eye disease. Eye disease in young children may be en- tirely asymptomatic. For that rea- son, regular examinations for the early detection of inflammatory changes in the anterior chamber of the eye are mandatory. These should be done at least every 3 months in a high-risk child.

LABORATORY AND X-RAY FINDINGS There is no specific diagnostic test for juvenile rheumatoid arthritis. The common findings will be pre- sented but none of these allow one to make a definite diagnosis of JRA or to absolutely rule out JRA if the finding is not present. In the majority of patients with active disease the erythrocyte sedimentation rate (ESR) is elevated; it may be normal to mildly elevated in pauciarticular disease and reaches its highest ele- vation in systemic disease. Most frequently it falls somewhere between 20 and 50 mm/hour. The white blood cell count (WBC) may be normal or elevated. I t is frequently elevated (leukocytosis) in children with systemic disease. The elevations range from 15,000 to 30,000 cells/mm3 to very high levels of 50,000 to 75,000/mm3. There also may be a mild thrombocytosis. The immunoglobulin levels are usually normal or mildly elevated. There is evidence of an IgA deficiency in approximately 4 percent of the patients.R Antinuclear antibodies are present in from 20 to 40 percent of patients with JRA;a these usually are present in a relatively low titer

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28 ISSUES IN COMPREHENSIVE PEDIATRIC NURSING

but the titer and pattern vary depending on the substrates used. Their significance is that there is a higher association between the presence of antinuclear antibodies and chronic iridocyclitis. Rheumatoid factor (IgM autoantibody to IgG) is present in approximately 20 percent of patients with JRA.8 These are seen most commonly in girls with older-age-onset disease and a pattern similar to that seen in adult rheumatoid arthritis. It should be emphasized that by far the majority of patients with JRA are seronega- tive and thus the absence of the rheumatoid factor is not helpful in excluding the diagnosis of JRA.

Synovialysis involves the laboratory examination of the joint fluid and is particularly important in the work-up of new patients, especially those that have one or two joints involved. T h e purpose of synovialysis is primarily to rule out an infectious cause and also to 'document the presence of an inflammatory fluid as opposed to a noninflammatory or traumatic fluid. There are characteristic patterns for inflammatory fluid which differentiate it from a septic or a noninflammatory fluid. In general, the pattern seen in JRA is a poor mucin clot, increased WBC and protein, normal or mildly depressed glucose level, and normal or mildly depressed total complement level.

Radiographic findings in JRA occur much later than those in adult rheumatoid arthritis. Initially one sees only periarticular swelling and juxta-articular osteoporosis. This is followed at variable intervals by joint-space narrowing, ero-

sive changes, and joint fusions. One of the earliest sites of articular changes in patients with polyarticular disease is the cervical spine. Involvement of the cervical apophy- seal joints may be the first radiographic evidence of the specific disease process.

DIAGNOSIS Making the diagnosis of JRA is dependent upon the objective dem- onstration of chronic inflammation in one or more joints. An inflamed joint is characterized by the following findings: swelling, limitation of motion, pain or tenderness, and increased warmth. Pain alone is arth- ralgia, not arthritis, and is not adequate for the diagnosis. According to the recently proposed criteria for diagnosing JRA,6 arthritis must be present in at least one joint for more than 6 weeks. Although the disease may be suspected prior to this time, definitive diagnosis cannot be made. It is not sufficient to satisfy the diagnostic criteria alone, as other diseases must be excluded. T h e specific exclusions focused on for an individual patient depend very much on the type of disease onset. Other factors which may s u p port the diagnosis include com- patible laboratory findings and the specific systemic manifestations mentioned previously, i.e., pericarditis, typical fever, morning stiffness, and iridocyclitis.

Important exclusions in pauciarticular onset disease include septic arthritis, ankylosing spondylitis, and malignancy. Prime concerns in those with polyarticular arthritis would be exclusion of systemic

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THE MUSCULOSKELETAL SYSTEM 29

lupus erythematosus (SLE), derma- tomyositis, and progressive systemic sclerosis.

Systemic onset JRA presents the greatest diagnostic difficulties. Infec- tion and malignancy should be excluded. Particular attention should be given to the possibilities of infectious hepatitis, infectious mono- nucleosis, leukemia, and other rheumatic diseases including systemic lupus erythematosus and rheumatic fever.

TREATMENT AND MANAGEMENT The goal of treatment in JRA is to preserve joint function and to treat extra-articular manifestations. There are three main components to the long-term treatment. These include drug therapy, physical therapy, and education. The first drug of choice in the treatment of JRA is salicylate in an appropriate form. There are a variety of drugs available, some combined with antacids. The most readily available and least expensive form is plain aspirin in the dosage of 90 mg/ kg/ 24 hours. The initial dosage and the aggres- siveness of treatment obviously depend on the severity of the child’s illness. In an acutely ill child the 90 mg/kg/day dosage is the starting dosage and it may need to be increased up to 130 or occasionally 150 mg/kg/day in order to control severe toxic symptoms. This high dosage of salicylates must be closely monitored with blood salicylate levels. Therapeutic range is between 20 and 30 mg/ 100 ml. In individual patients small increments in blood levels can make a significant differ-

ence in the control of clinical symptoms. Also, at high daily doses small increments in the oral dose can re- sult in a relatively large increase in the serum level. Some form of aspirin or other salicylates will control adequately the clinical symptoms in 50 to 60 percent of patients with JRA. In those who have persistent polyarticular disease which has been unresponsive or inade- quately controlled by aspirin for 6 to 8 months, gold-salt therapy should be considered. The gold treatment program involves weekly injections for usually 18 to 20 weeks and thereafter at somewhat less he- quent intervals of every 2 to 3 weeks. If there is a good clinical response, then the medication is usually continued for at least a year and often longer. As there is significant toxicity with gold, it must be closely monitored with regular blood counts and urinalyses and must be given by persons experi- enced with its administration. There are other nonsteroidal anti- inflammatory agents available which can be very helpful in individual patients. These include hydroxy- chloroquine, indomethacin, and tolmetin.

Corticosteroids should generally be reserved for patients with iridocyclitis or cardiac involvement. They are very rarely needed to control the acute toxic manifestations that have not responded to aspirin. In this case they should be used for a short period of time, i.e., 2 or 3 months, and then the disease controlled with other medications.

The second component of treatment involves physical therapy, in-

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cluding exercise programs and splinting procedures. Painful joints are involuntarily held in flexion and used with decreased frequency. This situation can lead to flexion contractures within a very short period of time, as little as 2 to 3 weeks. The longer the symptoms en- dure without adequate therapy, the more difficult the changes will be to reverse. Therefore, as early in the disease process as possible, inflamed joints should receive range of motion at least once daily. With active inflammation this will con- sist only of passive range of motion initially. Thereafter a progressive program should be instituted under the direction of a physical therapist. As disease activity diminishes, passive exercises can be replaced by active ones and then in certain situations followed by progressive strengthening exercises. Patients must be considered individually, as there is a “balance point” for joint activity versus inactivity that is de- sirable for each patient. This point lies somewhere between total inactivity and overactivity or abuse of the joints; the latter may lead to an exacerbation of disease activity. It is mandatory that the patient and the family understand the exercises completely and can demonstrate them to the therapist before the child is started on a home program. There is a tendency to overwhelm the child with a long set of complicated exercises. The child soon gets discouraged and gives up all exercise regimens. Rather than attack- ing every symptomatic joint initially, it is better to have a list of priorities and begin with two or

three simple, well understood exercises, buiIding slowly toward a com- plete program. The importance of the exercise program must be reiterated continually to the child and parent, as seldom does a patient have real enthusiasm for the required exercise program. In addi- tion to prescribed exercises, swimming is an excellent all-around activity that will benefit the arthritic child. Many children have shoulder and hip involvement and swimming is particularly good for range of motion to these joints. Emphasis is al- most always on joint extension rather than flexion, as flexion contractures are a common problem. There are many small aids that are particularly helpful in the exercise program. These include pulleys that can be attached to doorways. Depending on the child’s position with regards to the pulley, different joint motions can then be exercised. These are commercially available or many times can be made by the child’s parent. Heat is particularly good in relieving associated muscle spasm and morning stiffness. Differ- ent ways of accomplishing the same results would be an early morning shower or bath, use of an electric blanket or a sleeping bag.

Splinting is a helpful procedure for maintaining joints in functional positions. This ‘is particularly important during sleep as children tend to curl up in a flexed position. Splints may be made out of a plastic material or from half of a plaster cast. They are particularly helpful when used on the wrists. Efforts should be made to make certain that the splints are well-padded and

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T H E MUSCULOSKELETAL SYSTEM 31

comfortable; otherwise the children will not wear them. It usually takes several weeks for them to get accus- tomed to sleeping with the appli- ance. Splints also need to be brought along for clinic visits so that their position and comfort can be checked and changed when indicated.

Many children with polyarticular disease will have metatarsal pain which can be relieved by the use of metatarsal bars attached to the bot- toms of shoes. Occasionally more vigorous splinting or orthopedic procedures are required, particularly when a child already has long- standing disease when first seen.

Education is an ongoing need for both the parent and the child. The child needs to have educational in- formation with regard to the disease, and the treatment should be reiterated, expanded, and modified. I t is particularly important that they understand the importance of regular drug therapy and physical therapy. The tendency is for a patient who is feeling good to discon- tinue the previously prescribed programs. In most cases exacerbation of disease promptly follows.

With regard to formal education and vocational training, the goal should be to have the child attend school regularly and participate in as many normal activities as possible. The children can participate in a modified gym program if the teacher is amenable to individual programs. They should be discouraged from contact sports and sports in which there is excessive use of the weight-bearing joints, i.e., long- distance running and sprint events.

Those with polyarticular involvement should be encouraged to select vocations that do not require excessive use of the small joints of the hands, e.g., typing.

Rest should not be over-emphasized in the child’s program. How- ever, short periods of rest, i.e., l to 2 hour rest periods in the afternoon during periods of disease activity, can be important in controlling the disease. Children should not be re- moved for long periods of time from their normal contacts and from their normal school environ- ment. Prolonged bed rest is contra- indicated.

PROGNOSIS The overall prognosis for a child with juvenile rheumatoid arthritis is good. Approximately two-thirds of the children will be in remission 10 years after disease onset. Most of these children will have a polycyclic course with periods of remissions and exacerbations over their disease course. A few will have progressive destructive disease with which is associated a much higher incidence of morbidity. The highest risk for long-term residual disease is with the small group of patients with polyarticular disease, positive rheumatoid factor, older age onset, and other findings similar to those of adult rheumatoid arthritis. Another cause of long-term morbidity is hip involvement. Hip pain can be severe in spite of relatively good disease control in other joints. It occurs a t one time or other in approximately half of the patients, but a few will go on to severe hip destruc- tion. A procedure now available to

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these patients is total hip replace- ment. Other complications include growth disturbances, particularly leg length discrepancy, scoliosis, and rarely amyloidosis. The mortality rate in the United States for JRA is approximately 1 percent. The prime causes are infection and cardiac disease. The overall good prognosis for a patient with JRA needs to be continually emphasized to the patients and parents. As it is a chronic disease, there are many periods of discouragement. Both parents and child need ongoing supervision and support in order to have a good long-term outcome.

REFERENCES 1. Schaller, J. and R. J. Wedg-

wood: Juvenile Rheumatoid Arthritis: A Review, Pediatrics

2. Calabro, J. J. and J. M. Marche- sano: The Early Natural His- tory of Juvenile Rheumatoid Arthritis, Med. Clin. North A m .

3. Schaller, J. G., H. D. Ochs, E. D. Thomas, B. Nisperos, P. Feigl,

50:940-953 (1972).

52:567-591 (1968).

and R. J. Wedgwood: Histocom- patibility Antigens in Child- hood-onset Arthritis, J . Pediatr.

4. Cassidy, J. T., G. L. Brody, and W. Martel: Monarticular Ju- venile Rheumatoid Arthritis, J . Pediatr. 70:867-875 (1967).

5 . JRA Criteria Subcommittee: Current Proposed Revision of JRA Criteria. Arth. Rheumat.

6. Bernstein, B., M. Takahashi and H. Hanson: Cardiac Involve- ment in Rheumatoid Arthritis, J . Pediatr. 85:313-317 (1974).

7. Lietman, P. S . and E. G. L. By- waters: Pericarditis in Juvenile Rheumatoid Arthritis, Pedi- atrics 32:855 (1963).

8. Chylack, L. T., D. C. Bienfang, A. R. Bellows, and J. S. Still- man: Ocular Manifestations of Juvenile Rheumatoid Arthritis, A m . J . Ophthalmol. 79: 1026- 1033 (1975).

9. Petty, R. E., J. T. Cassidy and D. B. Sullivan: Serologic Studies in Juvenile Rheumatoid Arth- ritis: A Review, Arth. Rheumat.

88:926-930 (1976).

20 (Suppl): 195-199 (1977).

20 (Suppl): 260-267 (1 977).

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the child with arthritis

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