Introduction
Juvenile idiopathic arthritis (JIA) is the most common inflammatory joint disorder in childhood and may cause substantial intraarticular damage, long-term joint sequelae, and eventual disability. Arthritis occurring with other inflammatory rheumatic disorders in childhood, such as systemic lupus erythematosus, juvenile dermatomyositis, mixed connective tissue disease, and progressive systemic sclerosis is most often milder and nonprogressive. If joint contractures and deformities develop with these latter disorders, they are most often the result of severe extraarticular, intracutaneous, or subcutaneous fibrosis rather than severe intraarticular disease.
The clinical criteria for a diagnosis of JIA include (1) arthritis for more than 6 weeks, (2) onset below the age of 16 years, and (3) exclusion of other causes for arthritis. The advent of new pharmacotherapeutic agents has decreased the negative effects of persistent inflammation on the cartilage and ligamentous structures making the need for surgical evaluation and management of children with upper extremity inflammatory arthritis less common. The orthopedic or hand surgeon nonetheless will still occasionally be asked to evaluate a child with an undiagnosed inflammatory condition or to assess a patient with recalcitrant disease despite medical therapy.
This chapter will include the diagnostic approach, pharmacological management, and surgical considerations of children with pediatric elbow, hand, and wrist inflammatory arthritis.
Presentation and Diagnosis
Table 17.1 lists the major categories of JIA along with a description of the likelihood of upper extremity joint involvement. Hand arthritis is uncommonly seen in oligoarticular JIA, but the elbow and wrist are the third and fourth most commonly involved joints in this subgroup. All upper extremity joints are not commonly affected in the enthesitis-related subgroup. In other JIA subtypes, there is a high frequency of upper extremity joint involvement. In particular, dactylitis is quite pathognomonic of psoriatic arthritis ( Fig. 17.1 ). Shoulder arthritis is uncommon in early polyarticular or systemic disease but sometimes develops in more long-standing disease.
| Oligoarticular | Onset with four or fewer joints. Most often a younger female with an asymmetric pattern and the most commonly involved joints being the knee, ankle, and elbow. |
| Oligoarticular onset with polyarticular course | Similar to oligoarticular onset except with involvement of five or more joints 6 months after disease onset. |
| Polyarticular-rheumatoid factor negative | Onset with five or more joints. Most often a younger female with a symmetric pattern involving the wrists and small joints of the hands. |
| Polyarticular-rheumatoid factor positive | Onset with five or more joints. Most often a teenage female with a symmetric pattern involving the wrists and small joints of the hands |
| Psoriatic arthritis | Onset with either an oligo or polyarticular asymmetric pattern. Onset most often from 9 to 11 years old with slight female predominance, both upper and lower extremity involvement, and a propensity for the small joints of the hands (dactylitis)—particularly the DIP joints. |
| Enthesitis-related arthritis (formerly juvenile spondyloarthropathy) | Onset with oligoarticular arthritis in an asymmetric pattern primarily of the large joints of the lower extremities. Most often a male of age 13 years or older with accompanying lower extremity enthesitis and the possible later evolution of sacroiliitis and lumbosacral spine involvement. |
| Systemic | Onset with either a polyarticular or oligoarticular pattern with both upper and lower extremity involvement at any age with a slight male predominance. There are accompanying signs of systemic disease such as hectic high fevers, cutaneous eruption, lymphadenopathy, and hepatosplenomegaly. |
| Other or undifferentiated | A subset that does not meet the criteria for one of the designated categories or meets the criteria for two categories. |
At onset, most children present with morning stiffness and mild-to-moderate pain on motion. Shortly thereafter, the child develops recognizable joint swelling and warmth. Erythema is not usually present. Range of motion can be lost rather quickly, notably in extension about the elbow, wrist, and/or digits. Synovial cysts and small out-pouching of synovium are often seen, particularly over the proximal interphalangeal (PIP) joints and about the wrist joint. Extensor tenosynovitis on the dorsum of the hand is common. Limb or digital growth disturbances may occur, particularly when arthritis is poorly controlled. In contrast to adults with rheumatoid arthritis (RA), children with advanced hand arthritis develop ulnar deviation of the wrist with radial deviation of the fingers. Bone pain or tenderness is not common and should alert the clinician to the possibility of osteomyelitis or a malignancy. Night pain is particularly worrisome for the presence of a malignancy or a benign osteoid osteoma.
The most important responsibility of the surgical consultant is to make the correct diagnosis based upon the historical facts, physical examination, and radiographic changes ( Fig. 17.2 ). Patients often present with a history of recent trauma. Differentiating inflammatory mediated from traumatic articular changes is critical to making the diagnosis. The young skeleton is relatively resistant to trauma with thick peripheral cartilage protecting precious ossification centers and stout ligamentous attachments that shield against ligament injuries. The most typical presentation of JIA, particularly the oligoarticular subtype, is relatively painless restricted range of motion of the affected joint(s) along with radiographic abnormalities. Characteristic radiographic findings in children with inflammatory arthropathy include regional osteoporosis, decreased joint space, variation in bone outline because of erosions or bone cysts, carpal malalignment, and advanced skeletal maturity of the affected joint. Advanced skeletal maturity is the hallmark X-ray finding and secondary to the hyperemia and inflammation that results in earlier ossification compared to the unaffected side ( Fig. 17.3 ).
In comparison to adults with rheumatoid arthritis (RA), the rheumatoid factor screening test or anticyclic citrullinated peptide antibody (anti-CCP) is negative in all children with oligoarticular JIA. Similarly, most young polyarticular children (younger than 11 or 12 years old) are also rheumatoid factor or anti-CCP negative. Therefore, the absence of these laboratory markers does not rule out the diagnosis of inflammatory arthropathy. In children with both oligoarticular and polyarticular JIA, the antinuclear antibody is often positive. In children with the enthesitis-related arthritis subtype, the HLA B27 antigen is positive in 85%–90%. Inflammatory markers such as the erythrocyte sedimentation rate and C-reactive protein are slightly elevated in oligoarticular JIA, but may also be normal in many affected children.
A history of “trauma” and negative laboratory studies may result in an unnecessary surgical procedure. When in doubt, a team approach to diagnosis and management is helpful. Blending the knowledge and perspective of a pediatric rheumatologist, pediatric orthopedist and rehabilitation physician will facilitate appropriate diagnosis and disease specific management. For those colleagues without such resources, do not be afraid to reach out for this expertise. The internet and multiple list serve interfaces have expanded the resources for consultation services. It is imperative to remove all patient identifying information when transmitting data.
Treatment of JIA
The management of JIA has changed considerably within the last 10–15 years, primarily because of the recognition that earlier treatment limits irreversible articular cartilage and promotes better outcomes. The introduction of disease-modifying drugs and biologic agents has altered the clinical and radiographic consequences of the disease. This more focused therapeutic approach requires supervision by a pediatric rheumatologist with the knowledge and experience to plan the dosage of each medication, assess the risks and benefits of pharmacologic agents, and monitor the necessary parameters to assure safety and efficacy. In addition to the physician team, it is important to provide an occupational therapy program and provide social, psychological, and nutritional support.
The principles of surgical management remain unchanged. A team approach identifies those patients who would benefit from surgical intervention. The child is evaluated by the pediatric hand surgeon as well as the occupational therapist. Function is pivotal with the assessment of independence and ability to perform activities of daily living is pivotal in the decision-making process. Subtle difficulties can often be overcome by utilizing occupational therapy suggestions or simple adaptive equipment (e.g., buttoning and zippering devices).
Children with chronic inflammatory arthritis, systemic lupus erythematosus, and other autoimmune disorders rarely require salvage procedures to treat arthritic changes of their wrists or elbows. Early disease recalcitrant to noninvasive management, however, requires primary surgical treatment in the form of open or arthroscopic synovectomy. Once there is noteworthy loss of articular cartilage and/or the joint architecture is irreversibly altered, the goal is to preserve the unaffected painless portion of the joint for motion or to resurface or eliminate the damaged articular surfaces. Arthroplasty is suitable for the lower extremities of children with inflammatory arthropathies, although the lifelong activity restrictions are stringent and implant longevity remains uncertain. The preservation of lower extremity joint motion is more critical for ambulation and for activities of daily living. In the upper extremity (shoulder, elbow, wrist, and hand), biological interposition arthroplasties and limited fusions are more widely accepted, as the durability of upper extremity implant arthroplasty is even more dubious.
Early Medical Therapeutic Intervention
Intraarticular Corticosteroid Injections
This therapy is most commonly used for larger joints in oligoarticular JIA, but may also be done for selective joints in other subtypes when these remain active despite more advanced medical intervention. Injections also may be used as bridge management for polyarticular disease as further therapeutic agents are being introduced. Triamcinolone hexacetonide rather than triamcinolone acetonide is favored in children because of its extended therapeutic benefit (at least 6 months in more than 60% of joints treated). Early joint contractures may also respond favorably to intraarticular corticosteroids, and the occurrence of limb length discrepancies is decreased. Complications are limited, most often being mild localized subcutaneous atrophy and hypopigmentation at the injection site. These side effects should be discussed with the patient and family before injection.
Nonsteroidal Antiinflammatory Drugs
Ongoing use of nonsteroidal antiinflammatory drugs (NSAIDs) remains an integral part of medical therapy. If active arthritis persists for more than 2–3 months; however, it is currently recommended that additional medications be included in the regimen. NSAIDs approved for use in juvenile idiopathic arthritis include ibuprofen, naproxen, tolmetin, and celecoxib. Others such as meloxicam, nabumetone, and indomethacin are also regularly used. Serious gastrointestinal side effects in this group of medications remain rare when appropriate dosage is administered in children. During periods of dehydration NSAID’s should be stopped to avoid serious adverse renal effects.
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