Arthritis is inflammation of synovial tissue within a diarthrodial joint*. Infection-related arthritides are discussed in Chapter 152, but idiopathic arthritides constitute another group. Because the pathogenesis of the idiopathic forms is not completely understood, a variety of schemes have been devised to classify childhood arthritides according to the pattern of involvement, associated symptoms, and genetic factors. The classification criteria for juvenile idiopathic arthritis (JIA) proposed by the International League of Associations for Rheumatology is the most current,1 and is followed in this chapter. Based on the number of inflamed joints, laboratory features, family history, and extra-articular features, seven subtypes are recognized: oligoarticular arthritis, rheumatoid factor (RF)-positive polyarthritis, RF-negative polyarthritis, systemic onset JIA, enthesitis-related arthritis (ERA), psoriatic arthritis, or undifferentiated arthritis (Table 150-1).
TABLE 150-1Clinical Features of Juvenile Idiopathic Arthritis ||Download (.pdf) TABLE 150-1 Clinical Features of Juvenile Idiopathic Arthritis
|Type ||Peak Age of Onset ||Frequency (%) ||Typical Features |
|Oligoarthritis ||2–4 years ||27–56 || |
Arthritis of ≤4 joints in the first 6 months
Few or no complaints of pain, but swelling is noticeable, especially in large joints
Morning limp or refusal to walk
Girls >> boys
|Polyarticular, RF negative ||2–4 years ||11–28 || |
Arthritis of ≥5 joints for at least 6 weeks
Insidious onset of joint symptoms
Small joints of hands and feet are commonly involved
Girls > boys
|Polyarticular, RF positive ||Late childhood or adolescence ||2–7 || |
Arthritis of ≥5 joints for at least 6 weeks
Small joints of hands and feet are commonly involved
Early joint erosions
Girls >> boys
|Systemic onset ||Throughout childhood ||5–15 || |
Quotidian spiking fever
Transient, salmon-colored rash
Systemic features may precede arthritis
Pericarditis and macrophage activation syndrome are potential complications
Girls = boys
|Enthesitis-related arthritis ||Late childhood or adolescence ||3–11 || |
Arthritis, more often in lower extremity joints
Enthesitis involving heels, ankles, and knees
Inflammatory spinal pain
At risk for acute iritis
Often positive for HLA B27
Boys >> girls
|Psoriatic arthritis || |
|2–11 || |
Arthritis and psoriasis
Dactylitis, nail pitting, or family history of psoriasis
Girls > boys
|Undifferentiated || ||11–21 ||Variable |
The diagnosis of JIA is clinical, defined as joint swelling or limitation of joint range of motion with joint pain or tenderness. These signs must be present consistently for at least 6 weeks in a patient younger than age 16 years. The annual incidence of JIA is estimated at 10 per 100,000 children, and the prevalence of JIA in the United States is about 1 in 1000 children younger than 16 years.2 Estimates of the actual number of American children with JIA vary between 70,000 and 100,000, including both active and inactive cases.
Children with arthritis typically demonstrate stiffness after prolonged periods of inactivity, such as upon arising in the morning or after naps, long car rides, or sitting in class at school. Conversely, children with arthritis typically feel better after a warm bath or several minutes of activity. Cold, damp weather or swimming in cool water tends to be more difficult for children with arthritis, whereas warm weather generally relieves symptoms. Thus a child with arthritis may suffer joint stiffness in the morning but may be quite comfortable exercising strenuously later in the day. Pain is an unusual complaint in a child with JIA, and nighttime awakening is uncommon as well.3
Systemic-onset JIA, or Still’s disease, accounts for about 10% to 15% of all children with JIA. This is the subtype of JIA that most closely resembles an infectious disease. Although systemic-onset JIA can occur at any age, the peak age of onset is between 1 and 6 years. Boys and girls are equally affected. As its name implies, this is a systemic illness characterized by fever, rash, and arthritis. These children are often admitted to the hospital for evaluation of fever of unknown origin. The fever of systemic-onset JIA is described as quotidian or double quotidian; children typically have one or two spikes of fever greater than 39°C at about the same time every day. In between episodes of fever, the temperature returns to the baseline level or lower. The fever must be present for at least 2 weeks to be considered a diagnostic criterion. The rash of systemic-onset JIA is salmon pink, transient (lasting minutes to a few hours), nonpruritic, and migratory. It consists of discrete macules 2 to 5 mm in size, mostly on the trunk and proximal extremities. The rash often accompanies fever spikes, and it is typically seen after a shower. Rubbing or lightly scratching the skin elicits erythema (Koebner phenomenon) and might be followed by the appearance of transient macular lesions. Fever and rash are found in systemic-onset JIA but are not part of the clinical picture of other subtypes of JIA. These symptoms may precede the onset of arthritis by days to months. Most children with systemic-onset JIA develop chronic polyarthritis over time. Joint involvement is variable at disease onset, with anywhere from no joints to numerous joints being involved. Children with systemic-onset JIA also have laboratory evidence of systemic inflammation. The white blood cell count is usually elevated, with a predominance of neutrophils, accompanied by anemia and thrombocytosis; the erythrocyte sedimentation rate (ESR) is moderately to significantly elevated. Hepatosplenomegaly, lymphadenopathy, and serositis can also be seen in systemic-onset JIA.
Polyarticular JIA is characterized by the presence of arthritis of at least 6 weeks’ duration in five or more joints. Polyarticular JIA accounts for 30% to 40% of cases of JIA and typically affects more girls than boys. Two distinct subtypes are recognized, based on the presence or absence of rheumatoid factor (RF). If the onset occurs during the first peak, at age 1 to 3 years, patients are generally RF negative. Girls affected during the second peak, just before puberty, have a disease that is more like adult rheumatoid arthritis, are RF positive, and have early erosions and often subcutaneous nodules. In both types of polyarticular JIA, the onset is generally insidious, with children gradually accruing additional swollen joints over time. Typically, the arthritis is symmetrical above and below the waist and between the left and right sides, and it often involves the small joints of the hands and feet. Fever is generally absent, but children may complain of fatigue, malaise, and anorexia. Polyarticular JIA may be accompanied by mild anemia and a modest elevation of the ESR. Synovitis in multiple joints may lead to chronic joint changes and bony erosions. Up to 50% of those with polyarticular JIA show radiographic abnormalities within 2 years of onset if not treated aggressively.
Oligoarticular JIA, the most common type, accounts for about 40% of all children with JIA. It is characterized by the presence of arthritis in four or fewer joints during the first 6 months of disease. Oligoarticular JIA is more common in girls, and the onset age peaks between 2 to 4 years. Large joints are often involved, with the knee being most commonly affected. The arthritis can be asymmetric, and often only one joint is affected at presentation. Inflammatory markers are often normal or mildly increased. Antinuclear antibodies (ANAs) are detected in about 60% of patients with oligoarticular JIA. The International League of Associations for Rheumatology (ILAR) classification distinguishes two subcategories of oligoarticular JIA depending on the number of joints involved after the first 6 months of disease. In persistent oligoarthritis, the arthritis is limited to four or fewer joints, and in extended oligoarthritis, the arthritis extends to more than four joints. About one-third of children with oligoarticular JIA go on to have extended oligoarthritis. In younger children, oligoarticular JIA often presents insidiously with refusal to walk or a limp in the morning that typically improves as the day goes on. Children seldom complain of pain, although parents may notice swelling of a knee or other large joint. This type has relatively few systemic signs such as fever.
Enthesitis-related arthritis (ERA), affects ~10% of children with JIA. This subtype is characterized by arthritis and enthesitis (inflammation of tendon and ligament attachments). Most often the heels and knees are involved with enthesitis. The arthritis is more likely to involve the lower extremities. Inflammatory spinal pain and sacroiliac joint involvement can also be seen with this subtype. ERA typically affects boys after the age of 6. Most children are also positive for HLA-B27. In some cases, children present with just arthritis but have a positive family history of spondyloarthropathies in a first-degree relative. Children with ERA are at risk for painful acute anterior uveitis.
Psoriatic arthritis is characterized by the presence of arthritis and psoriasis. In children without a characteristic rash of psoriasis, the diagnosis requires any two of the following: family history of psoriasis in a first-degree relative, dactylitis (swelling of a digit extending beyond joint margins), and nail pitting or onycholysis. Psoriatic arthritis comprises 2% to 11% of all JIA. There is a biphasic distribution, with an early peak at 2 to 4 years and a later one at 9 to 11 years. This subtype is more common in girls. Chronic asymptomatic uveitis can be seen with this subtype.
Undifferentiated arthritis includes patients with JIA who do not satisfy criteria for inclusion in any of the other categories, or fulfill criteria for more than one category. As such, they do not have a characteristic pattern of arthritis. The ILAR criteria define several stringent exclusion criteria, which make it difficult to accurately classify some children with chronic arthritis. For instance, a child with an oligoarticular presentation who has two positive tests for RF, or systemic JIA in a boy after the age of 6, can result in a child being placed in the “undifferentiated” category. Clinical management depends on the features of the disease in an individual patient.
Macrophage activation syndrome (MAS) is rare, but it is the one complication of systemic JIA that is most likely to cause significant morbidity or mortality. It is discussed more fully in Chapter 151. MAS resembles hemophagocytic lymphohistiocytosis, which occurs as a familial form as a result of genetic abnormalities in natural killer T lymphocytes. It also occurs sporadically as a complication of malignancy or infection.4 In systemic-onset JIA, MAS most commonly occurs during the first 6 months of disease, during periods of active systemic inflammation that are often associated with a change in medication. MAS manifests as unremitting fever, unlike the spiking fevers of the underlying illness. Children may demonstrate bruising and mucosal bleeding from the consumptive coagulopathy seen in MAS, as well as mental status changes, hepatosplenomegaly, and diffuse lymphadenopathy. Activation of macrophages results in phagocytosis of erythrocytes, platelets, and leukocytes, so MAS should be suspected when patients with JIA present with a sudden drop in platelets, hematocrit, and white blood cells accompanied by an elevation of hepatic transaminases. Laboratory studies also demonstrate elevated triglycerides, ferritin, D-dimers, prothrombin time and partial thromboplastin time, and a fall in fibrinogen, ESR, and serum albumin. Other subtypes of JIA are not usually associated with MAS.
Pericarditis is seen in about 3% to 9% of children with JIA, almost always in association with systemic-onset JIA. Older children are more likely to develop pericarditis, which may present as precordial chest pain, dyspnea, or discomfort referred to the back, shoulder, or neck, or it may be noted incidentally on chest imaging. Physical examination findings include tachycardia, cardiomegaly, or a pericardial friction rub at the lower left sternal border. Cardiac tamponade is a rare but serious complication of constrictive pericarditis. It is characterized by venous distention, hepatomegaly, and peripheral edema. Urgent evaluation and management are necessary to avoid progressive cardiovascular instability. JIA seldom causes parenchymal lung disease, but pleuritic chest pain due to a pleural effusion may accompany pericarditis or occur in isolation.
Anterior uveitis, or iridocyclitis, is a well-recognized and potentially serious complication of JIA. Uveitis in JIA is usually asymptomatic and chronic; it is most common in children with oligoarticular JIA (approximately 10%) and to a lesser extent RF-negative polyarticular JIA. A younger age, female gender, and positive ANA test increase the risk of iritis in most studies, although boys are at risk as well. Uveitis is rare in systemic-onset JIA, occurring in less than 1% of cases. Acute uveitis, accompanied by pain and redness, may be associated with ERA. The detection of acute uveitis in a child suspected of having systemic-onset JIA should prompt a consideration of other causes of fever and rash, such as Kawasaki disease and acute viral infections.
Children with longstanding polyarticular or systemic-onset JIA may develop involvement of the joints of the cervical spine. This can result in instability or fusion of the posterior elements, so care should be exercised when these children undergo procedures that require sedation or anesthesia. Preoperative flexion and extension views of the cervical spine may aid in the detection of cervical involvement and the avoidance of subluxation and injury to the spinal cord or brainstem with hyperextension.
JIA is a clinical diagnosis made by history and physical examination. Care must be exercised in labeling a child with JIA before symptoms have been present for more than 6 weeks, because infectious arthropathies may be prolonged but ultimately transient. Conversely, although chronic inflammatory synovitis is seldom an emergency, acutely dangerous conditions must be excluded urgently in all cases. Thus a child with an acute febrile monoarthritis must be considered to have septic arthritis or osteomyelitis until proved otherwise, especially in the presence of elevated inflammatory markers. A new monoarthritis in an otherwise healthy-appearing child may represent an acute post-infectious arthritis (see Chapter 152). Common causes of post-infectious arthritis include parvovirus B19, group A streptococcus, and Borrelia burgdorferi (Lyme disease).
Systemic-onset JIA may be difficult to distinguish from severe infections, particularly when children present with fever before the onset of arthritis. The fevers of infectious diseases are usually hectic and spike less predictably than the fevers of systemic-onset JIA, and the fever often does not return to baseline between spikes. Additional studies are essential in most cases to exclude other causes of prolonged unexplained fever such as infections, malignancies, and inflammatory bowel disease. When migratory arthritis and arthralgias accompany fever, acute rheumatic fever and serum sickness should be considered. Cutaneous and cardiac manifestations usually allow these conditions to be distinguished, although echocardiography may be necessary to exclude rheumatic fever. Children with either dermatomyositis or systemic lupus erythematosus can present with polyarthritis and fevers, but additional clinical or laboratory features of the underlying disease are typically present.
Vasculitides such as Kawasaki disease and Henoch-Schönlein purpura often include arthritis among their symptoms. Other entities to consider are transient synovitis of the hip, slipped capital femoral epiphysis, and traction apophysitides (e.g. Osgood-Schlatter syndrome).
Laboratory tests may help rule out alternative diagnoses and classify the form of arthritis, but they are insufficient to confirm a diagnosis. Expected results from the complete blood count, ESR, and ANA are provided in Table 150-2. A positive ANA increases the risk for chronic uveitis. Rheumatoid factor is seen in about 5% to 10% of children with JIA, primarily in adolescent girls with RF-positive polyarticular JIA. Children with RF-positive polyarticular JIA are also often positive for anticyclic citrullinated peptide antibodies.
TABLE 150-2Key Laboratory Features of Juvenile Idiopathic Arthritis ||Download (.pdf) TABLE 150-2 Key Laboratory Features of Juvenile Idiopathic Arthritis
|Type ||CBC ||ESR ||Likelihood of Positive ANA (%) ||RF/CCP |
|Oligoarthritis ||Normal ||Normal or mildly elevated ||70 ||Negative |
|RF-negative polyarthritis ||Mild anemia, mild thrombocytosis ||Normal or mildly elevated ||40 ||Negative |
|RF-positive polyarthritis ||Mild anemia, mild thrombocytosis ||Mildly to moderately elevated ||40 || |
Positive for RF
About 80% positive for CCP
|ERA ||Normal to mild anemia ||Normal to mild elevation ||5–10 ||Negative |
|Systemic ||Leukocytosis, anemia, thrombocytosis ||Elevated ||5–10 ||Negative |
|Psoriatic ||Normal ||Normal or mildly elevated ||30–60 ||Negative |
In healthy-appearing patients with a new monoarthritis, parvovirus B19 and Lyme titers and antistreptococcal antibodies are generally included in the initial evaluation. Evaluation for a septic joint is pursued if clinically indicated.
At disease onset, imaging studies are usually normal, or they can show soft tissue swelling or effusions. Periarticular demineralization, narrowing of joint spaces, subchondral cysts, or bony erosions on plain radiographs are indicative of longstanding inflammatory arthritis. Plain radiographs are also helpful for identifying children with cervical spine abnormalities. Imaging is particularly useful when other diagnoses must be excluded. For example, lucent metaphyseal bands in the long bones of a child older than 2 years suggests a diagnosis of leukemia, especially when unexpectedly severe anemia or thrombocytopenia is also present. A radionuclide bone scan may be helpful when osteomyelitis or malignancy is suspected, although the incidence of false positive results owing to minor trauma, altered weight bearing, and normal growth centers can significantly limit its utility. Similarly, magnetic resonance imaging is usually not necessary in a child with arthritis, although a contrast-enhanced scan may confirm the presence of subtle synovitis that is not clearly demonstrable on physical examination. When periarticular disorders are under consideration, magnetic resonance imaging may be useful because it provides clear images of adjacent soft tissues and bone.
Arthrocentesis can be helpful in diagnosing JIA and excluding other causes of arthritis. Typically, synovial fluid in JIA is yellowish and cloudy, with white blood cell counts between 15,000 and 20,000 and a predominance of neutrophilic forms (75%). In septic arthritis, the fluid is usually serosanguineous and turbid, and the cell count is higher, often between 50,000 and 300,000, with more than 75% neutrophilic forms. Unlike in JIA, in septic arthritis the fluid has a low glucose level, and bacteria may be observed on Gram stain.
All subtypes of JIA tend to be characterized by remissions and relapses. In addition, it is not unusual for children with JIA to experience a flare of symptoms as a result of intercurrent infection. Systemic-onset JIA may follow a systemic course in which the fever, rash, and laboratory abnormalities persist. More commonly, it follows a polyarticular course, with arthritis persisting after resolution of the other systemic symptoms.5 Polyarticular JIA usually follows a chronic course over several years. About 30% of children with oligoarticular JIA have an extended oligoarticular course. Children with ERA may develop features of spondyloarthritis or inflammatory bowel disease. All subtypes of JIA have the potential to develop erosions or joint space narrowing after 2 to 5 years of persistent synovitis. Active synovitis can be detected in 30% to 55% of children with JIA 10 years after disease onset. Estimates of mortality in JIA range from 0.29 to 1.1 per 100 patients, severalfold higher than the standardized mortality rate for a similarly aged US population.
Children with JIA are best served by a comprehensive interdisciplinary team of healthcare providers that includes the primary care provider, pediatric rheumatologist, rheumatology nurse, social worker, and physical and occupational therapists. The aims of management in all types of JIA are to control pain, prevent and restore loss of motion in affected joints, improve overall functioning, and minimize the effects of inflammation on normal growth and development.6 Nonpharmacologic modalities such as moist heat can be especially helpful for relieving morning stiffness.
The first line of pharmacologic management for all forms of JIA traditionally has been a nonsteroidal anti-inflammatory drug (NSAID). Naproxen is preferred by most rheumatologists for its ease of administration and pediatric labeling. It is usually administered at a dose of 10 to 20 mg/kg per day in two to three divided doses; as with all NSAIDs, 2 to 4 weeks of therapy is necessary before a child’s response to naproxen can be assessed. Alternative NSAIDs include meloxicam, nabumetone, ibuprofen, sulindac, tolmetin, and indomethacin. All should be administered with food to minimize gastrointestinal side effects such as abdominal pain, nausea, heartburn, or anorexia. Surveillance for gastrointestinal side effects of NSAIDs is extremely important, and H2 blocker or proton pump inhibitor therapy is often used in conjunction with NSAID therapy. Naproxen is associated with pseudoporphyria (small blisters that scar) or skin fragility in up to 10% of children; fair skin and blue or gray eye color are reported to be risk factors. Other NSAIDs are associated less often with increased skin fragility. Bruising or bleeding can also be seen with NSAIDs. In patients with systemic JIA, indomethacin can be particularly helpful to treat fever and pericarditis. Patients should be cautioned not to use other over-the-counter NSAIDs while on naproxen or a similar agent. However, acetaminophen can be used occasionally for fever or extra pain relief. Children on NSAIDs need a complete blood count, urinalysis, and levels of serum liver transaminases, blood urea nitrogen, and serum creatinine checked at baseline and every 3 to 6 months.
It is important to note that all forms of JIA often require additional therapy beyond the use of NSAIDs.7 For children with oligoarticular JIA, arthrocentesis and injection of corticosteroids is often the second line of therapy. For children with other forms of JIA, medical management frequently requires aggressive escalation of therapy using multiple agents.
Corticosteroid therapy is often used as an adjunct to anti-inflammatory therapy in patients with JIA. The high frequency of side effects and lack of objective evidence that corticosteroids alter the long-term articular outcome weigh against the prolonged use of systemic corticosteroids in JIA. However, oral corticosteroids used at the lowest possible doses to minimize side effects may be useful in children with systemic-onset disease and significant and persistent systemic features such as fever. Because corticosteroids can mask other diagnoses and worsen infections, they should be used only after the diagnosis has been clearly established. Intra-articular steroid therapy in the form of triamcinolone hexacetonide is often effective in treating oligoarticular JIA, and can be used in other subtypes as well when there is persistent pain or inflammation in a few joints amenable to therapy. Topical corticosteroid ophthalmic preparations are used in the treatment of uveitis.
Second-line or disease-modifying antirheumatic drugs (DMARD) are generally instituted by pediatric rheumatologists or others experienced in their use. However, the hospitalist is likely to encounter patients with an established diagnosis of JIA who are on these medications and are hospitalized for a flare of arthritis or other unrelated conditions. The following is a brief synopsis of some of the more commonly used medications.
Methotrexate, a folate antagonist, is the most commonly used DMARD to treat polyarticular or systemic-onset JIA, or refractory oligoarticular JIA. Methotrexate is also used to treat children with uveitis with or without arthritis. Methotrexate is administered weekly by oral or subcutaneous routes; the latter is associated with better absorption and fewer gastrointestinal adverse effects. Although well tolerated in the majority of children, methotrexate may be associated with headache, nausea, or fatigue, usually within 1 or 2 days of administration. Oral mucosal ulcers are common unless children receive concurrent folic acid. More serious but less frequent side effects of methotrexate include leukopenia, hypersensitivity pneumonitis, and elevation of transaminases. These side effects may require modification of the dose or discontinuation of the drug. In some children intolerant to methotrexate, leflunomide may be administered. It is given orally daily or every other day. Leukopenia and abnormal liver function tests can be observed which necessitate lowering the dose or discontinuing therapy. Older second-line medications such as hydroxychloroquine and sulfasalazine are used infrequently because more effective agents are available.
BIOLOGIC RESPONSE MODIFIERS
The availability of biologic agents in particular has increased the therapeutic options for children with JIA. The most frequently used are directed against the cytokines tumor necrosis factor (TNF), interleukin 1, or interleukin 6. In addition, agents targeting T-cell costimulation and B-cells are also options for some children with JIA. All biological agents increase the risk of infection, including viral illnesses, severe bacterial infections, and reactivation of latent tuberculosis. A tuberculosis skin test should be performed before initiating treatment with a biologic agent and repeated annually. Biological agents can also exacerbate other serious bacterial or opportunistic fungal infections. Hence if a serious infection is suspected in a child on a biological agent, the medication should be withheld until the child is evaluated and appropriate antimicrobial treatment has been instituted. There is also concern that biological agents might increase the risk of malignancy in children with JIA.
TNF is a proinflammatory cytokine known to be elevated in children with JIA. There are many agents targeting TNF, but three of them are currently used widely in children with JIA. Etanercept (Enbrel) is a soluble TNF receptor that binds TNF, thereby blocking its binding to cell surface receptors. It is given by subcutaneous injection once or twice weekly. The most frequent side effects are injection site reactions. Usually these are mild, manifesting as erythema, and do not warrant discontinuation of etanercept. Infliximab (Remicade) is a chimeric anti-TNF monoclonal antibody that binds to TNF, thereby neutralizing its biologic activity. The antibody is administered by intravenous infusion every 4 to 8 weeks. These infusions may be accompanied by fever, chills, myalgia, or headache. Premedicating with acetaminophen and diphenhydramine is often helpful. Adalimumab (Humira) is a fully humanized anti-TNF antibody given subcutaneously every other week. The most common adverse effect is a mild injection site reaction manifested by a self-limited red rash or swelling. Cold compresses can be helpful in providing symptomatic relief. Adalimumab and infliximab are also used to treat refractory uveitis. Golimumab and certolizumab pegol are two other anti-TNF agents used in adults with RA.
Anakinra (Kineret) is an interleukin-1 receptor antagonist. It is used primarily in the treatment of systemic onset JIA. Anakinra is given as a subcutaneous injection daily. Common side effects include injection site reactions. Other less commonly used anti-interleukin 1 agents include rilonacept and canakinumab. Tocilizumab (Actemra) is a monoclonal antibody against interleukin-6, which is elevated in systemic JIA. Actemra is indicated for use in children with JIA. It is also used in adults with RA. Actemra is given as an intravenous infusion every 2 weeks in systemic JIA. Infusion reactions might occur, and are minimized by premedicating with acetaminophen and diphenhydramine. Periodic monitoring is required to detect abnormal liver function tests, leukopenia, and thrombocytopenia or abnormal cholesterol. Abatacept (Orencia) targets T-cell activation via blockade of the costimulatory signal needed for T-cell proliferation. It can be administered either intravenously monthly, or by subcutaneous formulations. Rituximab (Rituxan) is a chimeric anti-CD20 antibody that binds and destroys CD20-positive B cells. It is used in the treatment of adults with RA, and might be an option for some children with RF-positive polyarticular JIA. It is given as a set of intravenous infusions.
Ongoing pediatric rheumatology evaluation is important in the care of children with JIA. In addition, periodic evaluation by an ophthalmologist is needed to detect and treat chronic anterior uveitis. Uveitis in patients with JIA is often silent; they do not complain of eye pain and rarely present with a red eye. If undetected and untreated, uveitis can result in significant and irreversible loss of vision. For this reason, there is a recommended schedule of screening for uveitis based on type of JIA, age of onset, and ANA status. These guidelines should be strictly adhered to and can be found in the published policy statement of the American Academy of Pediatrics.8 Generally, children with JIA do well at school, and arthritis per se seldom accounts for absences. Rather, truancy is a warning sign of psychosocial difficulties. Nonetheless, children with significant arthritis might need special accommodations at school. Usually there is no need to restrict physical activity apart from avoiding direct trauma to joints, but children should be allowed to limit their activities as needed. Follow-up with a physical or occupational therapist should also be arranged to initiate a home exercise program to prevent or treat contractures, or for splints.
JIA is a chronic illness, and families often have many concerns about the long-term consequences of the disease. The term arthritis may evoke images of crippled digits or wheelchairs, which are fortunately rare with today’s therapeutic options. The treatment of JIA should include education that addresses important issues such as peer relations, family dynamics, social adjustment, and vocational planning. Young adults with JIA treated by comprehensive multidisciplinary teams can surpass community standards in terms of level of schooling and professional attainment. The American Juvenile Arthritis Organization, a branch of the Arthritis Foundation, is an excellent source of information for newly diagnosed patients with JIA and their families; its link can be found at the Arthritis Foundation website (www.arthritis.org). Other sources of information include the National Institute of Arthritis, Musculoskeletal and Skin Diseases at the National Institutes of Health, (www.niams.nih.gov) and the American College of Rheumatology (www.rheumatology.org).
Although a diagnosis of JIA can be straightforward, this is ultimately a clinical diagnosis that requires the exclusion of other causes of acute and chronic arthritis. The long-term management of children with JIA can be challenging, often involving the use of medications that require monitoring for adverse effects. Consultation with a pediatric rheumatologist, when available, can facilitate an early diagnosis and the prompt institution of appropriate medical management. Subspecialty consultation may also be helpful in the diagnostic evaluation and treatment of patients with JIA (Table 150-3).
TABLE 150-3Subspecialty Consultation to Consider in the Evaluation of Patients with Juvenile Idiopathic Arthritis ||Download (.pdf) TABLE 150-3 Subspecialty Consultation to Consider in the Evaluation of Patients with Juvenile Idiopathic Arthritis
|Subspecialty ||Potential Role |
|Infectious Diseases ||To assist in evaluation of patients when fevers secondary to infectious disorders are suspected or in diagnostic evaluation when septic arthritis or osteomyelitis are included in the differential diagnosis |
|Cardiology ||To assist in evaluation and management of chest pain, pericarditis, and pericardial effusions |
|Hematology/Oncology ||To assist in the evaluation of MAS or when malignancy is suspected |
|Orthopedic Surgery ||To assist with diagnostic evaluation to exclude septic arthritis or osteomyelitis |
|Physical/Occupational Therapy ||To preserve and improve range of motion and strength |
|Ophthalmology ||To screen for chronic (and clinically silent) anterior uveitis |
|Social Services ||To assist in locating financial, educational, and social assistance |
|Mental Health Services ||To assist in the management of affective disorders associated with chronic disease and to improve coping skills needed to manage a chronic disease and long-term medication therapy |
Evaluation of patients with suspected systemic-onset JIA for persistent fever, potential malignancy, or serious infection.
Management of serious flares of disease associated with pain, disability, or non-articular organ system complications.
Evaluation and teaching for patients with chronic disease and poor compliance with therapeutic regimens.
Completion of evaluation for possible malignancy or serious infection.
Resolution of fever and evidence of improving inflammatory markers.
Establishment of a therapeutic regimen, including medication, physical or occupational therapy, ophthalmology, and social services.
Discharge planning, including patient and family education and general pediatric and pediatric rheumatology follow-up.
If on steroids, a plan for tapering the medication and monitoring for side effects, including measuring blood pressure and following urinalyses for glycosuria, should be considered.
JIA is among the most prevalent chronic diseases of childhood, affecting 1 in 1000 children younger than 16 years.
Children with arthritis typically complain of joint swelling or morning stiffness; pain is unusual and suggests a mechanical rather than an inflammatory process.
Aggressive treatment of synovitis with disease-modifying agents such as methotrexate and biologic agents such as anti-TNF monoclonal antibodies effectively controls arthritis and prevents long-term complications in most children.
Periodic ophthalmic evaluation is an essential component of the care of children with JIA.
Further refinement of biologic response modifiers, including targeted B- and T-cell monoclonal antibodies, will continue to improve the safety and efficacy of therapy for JIA.
Identification of genetically determined disease subsets will allow therapy to be individualized.
Improved understanding of the pathophysiology of systemic-onset JIA may necessitate its reclassification as an autoinflammatory rather than an autoimmune disorder.
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