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Chapter 25 Childhood Rheumatic Diseases

Manual of Rheumatology and Outpatient Orthopedic Disorders

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Chapter 25 Childhood Rheumatic Diseases

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Thomas J. A. Lehman

Etiology and pathogenesis
Prevalence
Differential diagnosis
Diseases with unique manifestations in childhood
Miscellaneous conditions
Arthritis associated with primarily vasculitic conditions
Arthritis associated with metabolic and inherited conditions in childhood
Treatment

I. Etiology and pathogenesis. The rheumatic diseases of childhood represent a diverse group . Their etiologies are varied and their pathogenesis unclear. Lyme disease and acute rheumatic fever result from exposure to known infectious agents , but the majority of childhood rheumatic diseases result from the combination of genetic predisposition, autoimmunity, and unknown environmental factors.

II. Prevalence. Reactive arthritis (acute episodes of arthritis and arthralgia following an infectious illness ) is common in childhood, but chronic rheumatic disease is infrequent. Nonetheless, there are more than 250,000 children with arthritis in the United States. [Prevalence estimates are confused between the number of children with juvenile rheumatoid arthritis (100,000) and the number of children with any form of arthritis (250,000).] This dichotomy is responsible for an ongoing process of redefinition. The term juvenile rheumatiod arthritis (JRA) is being discarded. It is to be replaced by idiopathic childhood arthritis (ICA). At present, eight distinct subtypes of ICA have been described, and it is expected that more subtypes will be delineated before the full redefinition is completed. With this new definition, the spondyloarthropathies and many other subtypes that currently fall outside the spectrum of JRA will be included in ICA. Spondyloarthropathies, for example, will be termed enthesitis-associated arthritis, which will be a subtype of ICA.

ICA (including what was previously termed JRA and spondyloarthropathies), Henoch-Schnlein purpura, Kawasaki disease, systemic lupus erythematosus (SLE), dermatomyositis, and scleroderma are the most common forms of chronic arthritis in childhood.

III. Differential diagnosis. A careful history and physical examination are key to the proper diagnosis of childhood arthritis. The examining physician must have a clear knowledge of the differential diagnosis because children are often poor historians. A useful algorithm is illustrated in Table 25-1. The examining physician must determine whether inflammation is present (objective pain, swelling, warmth, or limitation of motion), whether the inflammation is articular or periarticular, and whether the inflammation is acute or chronic.

Table 25-1. Common forms of chronic synovitis in childhood

1. No inflammation present
   1. Growing pains. This is the most common and most misused diagnosis for musculoskeletal pain in childhood. The true syndrome of growing pains occurs in young children, peaking at 4 to 5 years of age. The pain classically occurs in the popliteal fossa. It is relieved by gentle massage or reassurance and occurs only at night. Pain during the day does not represent growing pains. Growing pains are benign and self-limited. Often, there is a family history of similar complaints, which may aid in the diagnosis. Growing pains are typically relieved by acetaminophen and do not require specific therapy .
   2. Psychogenic rheumatism. Joint pains and fatigue occur frequently as somatization disorders. The child who is unable to attend school or participate in normal activities despite an unremarkable physical and laboratory evaluation is worrisome. Some will respond to gentle reassurance, but for others, the complaints mask a significant psychological disorder . Children with persistent complaints despite normal findings should be evaluated carefully by an experienced pediatric rheumatologist to exclude undiagnosed illness. However, once this has been completed, the physician should be aware that the complaints may stem from problems within the family. These families are troubled but usually reject an immediate recommendation of psychological counseling . However, physicians who establish a trusting relationship with the family may be able to bring about gradual resolution or an acceptance of the need for psychological intervention.
   3. Reflex neurovascular dystrophy represents an extension of psychogenic rheumatism in which the somatization has progressed to include hyperesthesias, often with mottled skin coloring and vascular instability. The condition often begins with a well-documented injury that fails to improve. The syndrome typically occurs in perfect children under excessive parental pressure. Any psychological stress may initiate this syndrome. Excessive pressure to perform and sexual abuse are well-recognized causes. Although the specific complaints may be resolved with intensive physical and occupational therapy, failure to resolve the underlying psychological issues often results in recurrence of similar problems within a short time period.
2. Periarticular inflammation. Children with periarticular (i.e., soft tissue , tendon, ligamentous bursal) inflammation must be carefully evaluated for osseous disorders.
   1. Orthopedic disorders. Acute periarticular pain may result from a stress fracture or osteomyelitis. Small children with fractures may not report trauma. Battering must be considered when a child presents with unsuspected fractures. Bone scan may be helpful in the evaluation of these entities.
   2. Neoplastic disorders associated with infiltration of the bone marrow include leukemia, lymphoma, and neuroblastoma. All may present with difficulty walking or joint pains. Disproportionate anemia, thrombocytopenia, hyperuricemia, lymphadenopathy, or hepatosplenomegaly should prompt further investigation and bone marrow aspiration.
   3. Rheumatic disorders. The juvenile spondyloarthropathies often present with both periarticular and articular inflammation. The periarticular manifestations may predominate, but articular inflammation is usually present. Lumbar stiffness, enthesitis, and heel pain should be specifically sought. Often, these children are thought to have recurrent sprains or strains. The possibility of arthritis is often incorrectly dismissed by inexperienced physicians because the erythrocyte sedimentation rate (ESR) is normal.
3. Articular inflammation. Children with true articular inflammation must be subdivided according to whether they have inflammation that is acute or chronic (of more than 3 months' duration).
   1. Acute articular inflammation
      1. Infection. An acutely inflamed joint must be considered septic until proved otherwise . Staphylococci, streptococci, and Haemophilus influenzae are frequent causes of septic arthritis in childhood. Lyme disease is a frequent infectious arthritis in areas where Ixodes ticks are endemic.

         Septic arthritis typically presents with a single inflamed joint accompanied by fever and an elevated ESR. It is less common but not impossible for other infectious agents to involve multiple joints. Not infrequently, Lyme disease may involve several joints simultaneously .

      2. Reactive arthritis may accompany or follow bacterial, viral, or fungal infection. Toxic synovitis is the most common reactive arthritis in childhood. The typical child with toxic synovitis is 3 to 5 years of age. Classically, they have been well except, perhaps, for symptoms of an upper respiratory infection the prior evening. The following morning, the child awakens unable to walk, with a decreased range of motion in one hip. There is only low-grade or no fever, without significant elevation of the white blood cell count or ESR. Unless an experienced physician is comfortable with the clinical picture, the joint must be aspirated to rule out bacterial infection. The joint symptoms of a child with toxic synovitis typically begin to improve within a few hours; in contrast, those of a child with a truly septic hip often rapidly worsen.
      3. Post-streptococcal reactive arthritis deserves special consideration. This disorder is not classified as acute rheumatic fever because it does not fulfill two of Jones' major criteria. Nonetheless, children with arthritis and elevated ESRs following a documented streptococcal infection should receive rheumatic fever prophylaxis. Cardiac damage has been recorded with subsequent streptococcal infections in some children who did not receive such long-term prophylaxis.
      4. Acute expression of a collagen vascular disease. Serum sickness, acute rheumatic fever, Henoch-Schnlein purpura, and the chronic collagen vascular diseases (e.g., SLE) may present with acute arthritis. Most are discussed elsewhere in this manual; only those that are unique to childhood or that have unique manifestations in children are discussed in this chapter.
   2. Chronic articular inflammation
      1. Infection. Chronicity does not exclude infection. Tuberculosis is a frequent cause of smoldering septic arthritis, but other bacterial infections, including staphylococcal arthritis, may present with such a clinical picture. Additionally, physicians must remain aware that in children with known collagen vascular disease, especially those on immunosuppressive drugs, complicating septic arthritis or osteomyelitis may develop.
      2. Collagen vascular diseases. All the chronic collagen vascular diseases may occur in children. Most are discussed elsewhere.

IV. Diseases with unique manifestations in childhood

1. Idiopathic childhood arthritis (previously called juvenile rheumatoid arthritis). As noted above, ICA is replacing the term JRA. Although many will be confused by this new term, it should be appreciated that its use has arisen because of the many children with arthritis who do not fulfill the classic criteria for JRA. ICA is used to describe any noninfectious or traumatic arthritis in childhood. There are currently eight recognized subtypes, and it is expected that there will be more. It should be appreciated that ICA is an umbrella term and does not refer to a specific disease. Each subtype of ICA is distinct, most likely with a distinct etiology, pathogenesis, prognosis , and optimal therapy. Because the subtypes represent different diseases, it is very important that the subtypes be properly differentiated from one another. Oligoarticular onset (four or fewer joints involved) and polyarticular onset are defined on the basis of the number of joints involved during the first 6 months after disease onsetnot the number of joints involved at the time the child is first seen by the physician.
   1. Oligoarticular-onset ICA involves four or fewer joints. It most commonly occurs in young girls but may affect persons of either sex. This group is divided between the subset that is antinuclear antibody (ANA)-positive and at greater risk for complicating eye disease (iridocyclitis) and the subset that is ANA-negative. Young girls with early involvement of small joints (i.e., finger and toe joints) are at high risk for progression to polyarticular involvement and have a poor prognosis. The condition of a child who has fewer than four joints involved during the first 6 months of disease, but who progresses to have more than four involved joints later, is termed extended oligoarticular disease in the new nomenclature .

      Some children have sausage digits and probably psoriasis-associated arthritis (psoriatic arthritis sine psoriasis). Children with a close family history of psoriasis or nail pitting may be differentiated as having psoriasis-associated arthritis. However, other children, who have neither a family history of psoriasis nor nail lesions, have arthritis with an identical appearance.

      Adolescents with involvement of four or fewer large joints are more likely to have a spondyloarthropathy (now called enthesitis-associated arthritis) (see section IV.B ).

   2. Polyarticular-onset idiopathic childhood arthritis has at least two distinct subtypes. Rheumatoid factor (RF)-positive adolescent girls have typical adult-type RA. Young children with polyarticular ICA are typically RF-negative. Both of these entities carry a guarded prognosis. Some children previously labeled as having polyarticular ICA may in fact have the psoriasis-associated subtype of ICA.
   3. Systemic-onset idiopathic childhood arthritis presents with high spiking fever, rash, and variable joint involvement. It occurs with a more equal sex ratio than the other forms of ICA, which have a female predominance. Children with systemic-onset ICA are striking for their ill appearance during episodes of fever, with a relatively benign appearance between episodes. The fleeting salmon pink rash and a temperature that falls to normal or below at least once each day are characteristic. Although many children with systemic-onset ICA do well, significant internal organ involvement develops in others, or they progress to chronic destructive arthritis.
2. Spondyloarthropathies. The spondyloarthropathies, occurring in both male and female patients , are now defined as enthesitis-associated arthritis. Their hallmark is asymmetric large-joint arthritis associated with limited lumbar flexion and tenosynovitis. These children are ANA-and RF-negative. They are at risk for acute, painful iritis but in general not chronic iridocyclitis. HLA-B27 is present in about half of these children.
   1. Ankylosing spondylitis (AS) is the classic spondyloarthropathy. It occurs predominantly in male patients positive for HLA-B27 who have limited lumbar flexion. Because definite AS cannot be diagnosed in the absence of radiographic sacroiliitis, many children who are suspect fail to fulfill the diagnostic criteria. These children should be given a diagnosis of juvenile spondyloarthropathy.
   2. Juvenile spondyloarthropathy (seronegative enthesopathy/arthropathy syndrome). These are children with asymmetric large-joint arthritis and enthesopathic findings who do not meet the criteria for AS. They are easily differentiated from patients with other forms of ICA by their later age at onset (usually age 10 or older), the early presence of back or hip involvement, and the frequent occurrence of asymmetric metatarsal joint pain or Achilles tendinitis. Although many of these patients are boys positive for HLA-B27, girls and HLA-B27negative persons of either sex may also be affected. It was initially thought that classic AS would develop in most of the boys when they reached adulthood . It is presently thought that significant disease will develop in many of the HLA-B27positive boys, but in only a small percentage of the others.
   3. Reiter's syndrome. The full combination of arthritis, urethritis, and conjunctivitis occurs infrequently in childhood. When it does, its manifestations are the same as in adults. It is not important to differentiate children with incomplete Reiter's syndrome from others with juvenile spondyloarthropathy because the therapy and prognosis are similar.
   4. Psoriatic arthritis/psoriasiform arthritis, subset with psoriasis-associated idiopathic childhood arthritis. True psoriatic arthritis with typical skin lesions and bony changes is infrequent in childhood. However, a subgroup of children without psoriatic skin changes present with asymmetric dactylitis (sausage digits), a family history of psoriasis in a first-or second-degree relative, and variable degrees of asymmetric joint inflammation. In contrast to the other spondyloarthropathies, this constellation of findings affects not only adolescents but also young girls who would otherwise be labeled as having oligoarticular ICA. The proper nomenclature for this group is currently psoriasis-associated ICA.
   5. Inflammatory bowel disease. The arthritis accompanying inflammatory bowel disease is expressed as a typical spondyloarthropathy. Because arthritis may be the initial manifestation of inflammatory bowel disease, any child with a spondyloarthropathy in whom chronic or recurrent abdominal pain or persistent unexplained anemia develops should be carefully evaluated for the presence of Crohn's disease or ulcerative colitis.

V. Miscellaneous conditions. Several forms of arthritis that occur predominantly in children are not easily characterized. Recognition of these conditions is important to prevent their being confused with more serious entities and ensure proper therapy and counseling.

1. Plant thorn synovitis results from retention of a fragment of plant material within the joint following a puncture injury. When the injury is recalled and the condition suspected, it is easily diagnosed. Confusion arises when a small child falls and the parents are unaware that foreign matter may have entered the joint. The onset of joint swelling and limitation is usually delayed by 4 to 6 weeks. The arthritis is often quite painful and unresponsive to normal measures. The proper diagnosis is often made following surgical biopsy of patients with intractable synovitis. The pathologic specimen reveals plant fibers under polarized light microscopy. Synovectomy is the treatment of choice.
2. Benign hypermobile joint syndrome typically occurs in girls during or just before early adolescence . They are most often gymnasts who practice extensively and have great flexibility that can be attributed to marked ligamentous laxity. As a result of their athletic activities and ligamentous laxity, their joints are subjected to repeated episodes of microtrauma. Acute episodes may be treated with nonsteroidal antiinflammatory drugs (NSAIDs), but more prolonged difficulty should prompt review of the athletic program. Osteochondritis dissecans (particularly of the knee) may present in this group of patients. In rare cases, children who have continued their activities despite chronic pain have suffered permanent disability.
3. Immunization-associated arthritis. The development of a benign polyarthritis affecting primarily the small joints of the hands 10 to 14 days following rubella immunization is well documented. The arthritis is typically mild and resolves during 7 to 10 days with only symptomatic therapy. Similar episodes have been reported less frequently with other viral immunizations.
4. Arthritis associated with immunoglobulin deficiency. Children with IgA deficiency are most often asymptomatic, but this immunodeficiency occurs with a greater than expected frequency in populations of children with arthritis. The arthritis commonly consists of benign recurrent joint effusions; however, some children present with typical erosive ICA. A benign arthritis may also occur in children with mild transient hypogammaglobulinemia. This arthritis may recur with viral infections, but ultimately it resolves as the child's immune system matures and the immunoglobulin levels normalize. IgA deficiency will be detected only if quantitative immunoglobulins are routinely measured. Pan-hypogammaglobulinemia may be suspected if the total protein is decreased with a normal serum albumin.
5. Linear scleroderma occurring in childhood is a gradually progressive, bandlike tightening of the skin that may occur over the face, trunk, or an extremity . It typically does not cross the midline. There may be progressive loss of underlying muscle and bony tissue with markedly disturbed growth when a limb is involved in a young child. Findings on laboratory evaluation are usually entirely normal. Physical therapy may be beneficial, but surgical intervention is required in extreme cases. Medical therapy with low-dose methotrexate has been beneficial for more severe cases in which the skin involvement crosses a joint line, but its efficacy remains anecdotal.
6. Linear scleroderma en coup de sabre . This entity is a variant of linear scleroderma characterized by primary involvement of one side of the scalp. It is incompletely differentiated from the Parry-Romberg syndrome of progressive facial hemiatrophy. Recognition of Parry-Romberg syndrome is important because affected children may be afflicted with neurologic disorders, including learning disability and seizuresfindings that are not normally associated with linear scleroderma.

VI. Arthritis associated with primarily vasculitic conditions. Arthritis is a well-recognized complication of many forms of vasculitis that occur in childhood, including SLE, Wegener's granulomatosis, Takayasu's arteritis, and Henoch-Schnlein purpura. These diseases are not unique to childhood and are discussed elsewhere.

Kawasaki disease and juvenile-onset dermatomyositis are vasculitic diseases with unique manifestations in childhood.

1. Kawasaki disease typically affects children in the first 5 years of life. It presents with fever accompanied by a pleiomorphic rash, conjunctivitis, and cervical adenopathy. As the disease progresses, changes in the oral mucosa become evident, with dryness and cracking of the lips. Indurative edema of the hands and feet followed by peeling of the skin from the tips of the fingers or toes (but sometimes beginning in the perineal region) is characteristic. An acute arthritis may accompany the disease, but most often there is diffuse swelling of the hands or feet. Marked elevations of the ESR, white blood cell count, and platelet count evolve during the first 10 days of illness. Prompt recognition and echocardiographic evaluation are desirable. Untreated Kawasaki disease is associated with a 1% to 3% mortality rate caused by aneurysmal dilatation of the coronary arteries with subsequent thrombosis and myocardial infarction. The illness should be suspected whenever the characteristic findings are present. Early intervention with large doses of intravenous gamma globulin has been shown to decrease the frequency of aneurysms and improve outcome. For children who fail to respond after two courses of intravenous gamma globulin, the diagnosis should be reevaluated and consideration given to corticosteroid therapy.
2. Childhood-onset dermatomyositis most often presents with the gradual onset of weakness and fatigue but may have an explosive onset with fever, weakness, and vascular collapse. A heliotropic rash, although not always present, is characteristic of the disease. Childhood-onset dermatomyositis is divided into three subtypes.
   1. Unicyclic disease usually presents with the gradual onset of proximal muscle weakness, responds well to prednisone, and disappears completely within 1 year.
   2. Polycyclic disease is similar to unicyclic disease but recurs whenever the corticosteroids are tapered. It may be associated with a poor prognosis secondary to chronic skin manifestations, subcutaneous calcification, or vasculitic involvement of internal organs.
   3. A third form of childhood dermatomyositis consists of prominent skin manifestations and muscle enzyme elevation in children who are not strikingly weak. These children often have persistent vasculitis. Children with any evidence of dysphonia or cough when eating are at high risk for aspiration.

VII. Arthritis associated with metabolic and inherited conditions in childhood. Many inherited disorders, such as hemophilia, sickle cell disease, mucopolysaccharidoses, sphinoglipidoses, and epiphyseal dysplasias, may present with arthritis or periarticular pain in childhood. Characteristic nonarticular manifestations usually predominate.

1. Marfan syndrome. Children with Marfan syndrome characteristically are tall with arachnodactyly. They typically exhibit ligamentous laxity and present with complaints similar to those of the hypermobile joint syndrome. Characteristic findings are an arm span greater than the height and a leg length greater than trunk length. Recognition is important because these children are vulnerable to dissecting aortic aneurysms. Aortic root dilatation may be evaluated by routine echocardiography.
2. Ehlers-Danlos syndrome. Children with Ehlers-Danlos syndrome suffer from an extreme form of joint hypermobility associated with abnormal connective tissue. Recurrent joint injury secondary to chronic subluxation is common. In typical cases, marked cutaneous laxity is present with characteristic cigarette paper scarring. However, milder cases that lack the cutaneous manifestations occur.
3. Cystic fibrosis in childhood will be recognized by the pulmonary and gastrointestinal manifestations. Occasionally, however, benign effusions of the large joints or immune complex- related synovitis develops in these patients, which will prompt rheumatologic referral. Hypertrophic osteoarthropathy may also occur in children with cystic fibrosis.

VIII. Treatment

1. Nonsteroidal antiinflammatory drugs are the first-line agents of choice for children with chronic synovitis. Although these drugs are often criticized because they are not disease-modifying agents, this criticism is unjustified. NSAIDs directly modify disease outcome by reducing pain and inflammation. As a result of decreased pain and inflammation, the patient is better able to preserve strength, range of motion, and endurance , which in turn preserve function. Preservation of function has a definite positive impact on outcome. Thus, NSAIDs definitely are disease-modifying agents, but they are not remission-inducing agents.
   1. Aspirin remains the nominal drug of first choice because of its cost advantages, but it has been supplanted by naproxen and tolmetin in many centers because of less frequent dosing and reduced risk for both hepatotoxicity and Reye's syndrome. The dosage schedules and pharmacologic characteristics of the NSAIDs are discussed in Appendix E.
   2. Indomethacin deserves special mention in childhood because it was regarded as unsafe for children under 12 years of age for many years. This restriction has been removed from recent issues of the Physicians' Desk Reference. Indomethacin is a potent NSAID that is very effective for childhood rheumatic diseases. It is frequently effective in children with severe, systemic-onset ICA or spondyloarthropathies that have not responded to other NSAIDs. Care must be taken in its use because of potential hepatotoxicity and gastric irritation. Headaches are a frequent side effect during the initial stages of therapy but usually respond to symptomatic therapy. The accepted dosage in childhood is 1 to 3 mg/kg daily.
2. Slow-acting antirheumatic drugs (SAARDs) provide useful adjunctive therapy in children with chronic rheumatic disease.
   1. Gold salts . There is an extensive literature regarding the long- and short-term efficacy of gold salts. Although the patient must be monitored carefully for evidence of toxicity, injectable gold salts (aurothioglucose and gold sodium thiomalate) are highly effective for children with specific subtypes of ICA. The accepted dosage of injectable gold salts in childhood is 1 mg/kg weekly after initial evaluation of the response to a series of gradually increasing test doses.
   2. Auranofin has been demonstrated to be effective in JRA but is less effective than injectable gold salts, which are preferred.
   3. Methotrexate is frequently used in the therapy of childhood rheumatic disease. The short-term effectiveness of methotrexate is quite good, but it is difficult to wean patients from this agent. Long-term studies of efficacy and toxicity in childhood have not been completed. The accepted dosage regimen for methotrexate in childhood is 10 mg/M ² per week up to a maximum of 20 mg/M ² per week. Hematologic toxicity and hepatic toxicity require close monitoring. The long-term risks for hepatic cirrhosis or pulmonary fibrosis in childhood appear low. The major concern with methotrexate therapy is that in up to 50% of children, the disease flares when the methotrexate is withdrawn. Furthermore, a significant subset of these children does not improve when the methotrexate is reinstituted.
   4. Hydroxychloroquine (Plaquenil) has been used with success in children with chronic arthritis. However, the onset of efficacy is extremely slow. The accepted dosage regimen is 7 mg/kg daily up to 200 mg/day. Children receiving Plaquenil require ophthalmologic examinations every 6 months to monitor for evidence of retinal toxicity.
   5. Enternacept (Enbrel), a recombinant tumor necrosis factor-alpha blocker, has recently become available for use in children with severe arthritis. Early results have been extremely promising in children with systemic-onset disease. Administered at a dose of 0.4 mg/kg as a subcutaneous injection twice weekly, Enbrel has not been associated with significant toxicity. At present, long-term efficacy and toxicity are unknown, and the drug is being used primarily in those who have not responded adequately to methotrexate. The full potential and proper utilization of Enbrel in childhood should become apparent within the next few years.
3. Surgery. Replacement of severely damaged and painful hip, knee, and elbow joints is now routinely performed in adolescents at larger orthopedic centers. For younger and smaller patients, the procedures require a center with extensive surgical expertise, the ability to manufacture custom prostheses, and extensive rehabilitation facilities. Although there is a risk that further replacements may be required in the future, the physical and psychological benefits of maintaining independent function throughout adolescence far outweigh the risks associated with the potential need for subsequent surgery. Soft-tissue releases and synovectomies have been performed on many children with ICA. The loss of function associated with postoperative pain and loss of strength that accompanies these procedures generally outweigh the transient gains in alignment and range of motion. However, the ability to perform arthroscopic synovectomy has led to renewed interest in this technique.
4. Physical and occupational therapy are vital components of the care of children with rheumatic disease. Appropriate exercises to maintain strength and range of motion coupled with splinting to maintain proper alignment are important to the ultimate outcome.
5. Family support remains a major component of the overall program of care for children with chronic disease. The primary burden of family support may be carried by social workers, nurse clinicians, or psychologists in different institutions. Each group has different strengths to bring to these families, and optimally all will be available for the families. The presence of a child with chronic disease in the family creates profound stress in even the best-adjusted families. A program of care that does not provide for the emotional needs of the affected child's parents and siblings may result in profound psychological scarring.
6. Ophthalmologic evaluation. The ophthalmologist is a major participant in the care of children with rheumatic disease. All children with JRA are at risk for the development of iridocyclitis. Those with ANA-positive pauciarticular onset require slit-lamp evaluation every 4 months, and those who are ANA-negative should be checked every 6 months.

Bibliography

Cassidy JT, Petty RE. Textbook of pediatric rheumatology, 3rd ed. New York: Churchill Livingstone, 1994.

Hicks RV. Vasculopathies of childhood. Littleton, MA: PSG, 1988.

Southwood TR. Classifying childhood arthritis. Ann Rheum Dis 1997;56:79.

Books@Ovid
Copyright 2000 by Lippincott Williams & Wilkins
Stephen A. Paget, M.D., Allan Gibofsky, M.D., J.D. and John F. Beary, III, M.D.
Manual of Rheumatology and Outpatient Orthopedic Disorders

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