Treatment for Juvenile Rheumatoid Arthritis (JRA)Oct 06, Author: JIA is a chronic disease characterized by periods of remission and flare. Treatment is aimed at inducing remission with the corticosteroid therapy of juvenile rheumatoid arthritis toxicity from medications with hopes of inducing a permanent remission. The success of therapy is monitored best with repeated physical examinations and history. The number of joints involved and the duration of rheumqtoid stiffness should demonstrate continued decrease, with elimination reflecting success.
Treatment of juvenile rheumatoid arthritis
Oct 06, Author: JIA is a chronic disease characterized by periods of remission and flare. Treatment is aimed at inducing remission with the least toxicity from medications with hopes of inducing a permanent remission. The success of therapy is monitored best with repeated physical examinations and history.
The number of joints involved and the duration of morning stiffness should demonstrate continued decrease, with elimination reflecting success. Surgery may be indicated in patients who are unresponsive to medical therapy.
Within each treatment group, choice of therapy is guided by the severity of disease activity and the presence or absence of features indicating a poor prognosis.
In September , the ACR released updated guidelines for the treatment of systemic JIA, which included the medications canakinumab, rilonacept, and tocilizumab. According to ACR guidelines, the treatment group that comprises patients who have developed active arthritis in only 4 or fewer joints total throughout their disease course includes patients in the International League of Associations for Rheumatology ILAR categories of persistent oligoarthritis, as well as patients with psoriatic arthritis, enthesitis-related arthritis, and undifferentiated arthritis.
NSAIDs alone may be adequate for patients with involvement of a single joint and other indications of low disease activity eg, normal inflammatory marker levels ; response should be evident within 2 months. Intra-articular injections of triamcinolone can be used for any joint involved with active arthritis, and should provide clinical relief for at least 4 months. If so, the injections can be repeated as needed. Alternatively, methotrexate is recommended as initial treatment for patients in this treatment group who have high disease activity and features indicating poor prognosis.
In patients with enthesitis-related JIA, sulfasalazine rather than methotrexate is recommended for patients who have an inadequate response to joint injection or an adequate trial of NSAIDs. The same is true of patients with enthesitis-related JIA who receive sulfasalazine. This group comprises patients who have developed active arthritis in 5 or more joints total throughout throughout their disease course.
Patients need not currently have active involvement in 5 or more joints. According to ACR guidelines, this group includes patients with the ILAR categories of extended oligoarthritis, rheumatoid factor RF negative and RF-positive polyarthritis, psoriatic arthritis, enthesitis-related arthritis, and undifferentiated arthritis.
After 1 month of NSAID treatment in patients with low disease activity, or months in those with moderate disease activity but without poor prognostic features ie, hip or cervical spine involvement, positive RF or anti-cyclic citrullinated peptide antibodies CCP , radiographic signs of joint damage , it is appropriate to escalate to methotrexate, plus adjunctive NSAIDs and joint injection as needed.
In patients with moderate disease activity and poor prognostic features, as well as in patients with high disease activity, treatment may start with methotrexate.
Leflunomide may be used as an alternative to methotrexate, after a failed NSAID trial, or as initial treatment in patients with high disease activity and poor prognostic features. Tocilizumab can be used alone or in combination with methotrexate. If these agents prove inadequate, patients may be started on rituximab; this agent may be most appropriate in patients with RF-positive polyarticular JIA.
A study confirmed the acceptable long-term tolerability of etanercept and adalimumab treatment in polyarticular juvenile idiopathic arthritis. This group includes all patients with clinical and imaging evidence of active sacroiliac arthritis.
This group includes all patients who fulfill the ILAR criteria for systemic arthritis and who have active fever of systemic JIA with or without other systemic features, but without active arthritis. Patients with high systemic disease activity eg, significant serositis may be started on steroids as a first step. There is virtually no published evidence regarding steroid doses or administration routes in this setting.
Patients who sustain or develop active fever while on systemic steroid therapy can be started on anakinra. This agent may be a first choice in patients who have had significant active systemic disease for at least 6 months. Additionally, nearly two-thirds of patients in the study were free of rash after 3 months. In this study, the safety profile similar to adults treated with tocilizumab for rheumatoid arthritis.
This category includes all patients who fulfill the ILAR criteria for systemic arthritis and who have active arthritis, but who do not have active systemic features. NSAID therapy, with intra-articular joint injections as needed, may be adequate for patients with low disease activity who do not have hip involvement or radiographic signs of joint damage.
After up to 1 month, however, methotrexate can be added for patients with any degree of disease severity who continue to have active arthritis. Inpatient care is required for persisting fevers of unknown origin or when children with known JIA have severe exacerbation of disease. The development of pericarditis in children with systemic-onset JIA is usually an indication for admission.
Exercise preserves joint range of motion and muscular strength, and it protects joint integrity by providing better shock absorption. Types of exercises that may be advised include a muscle-strengthening program, range-of-motion activity, stretching of deformities, and endurance and recreational exercises. Hydrotherapy is a good form of exercise that helps achieve the aforementioned objectives.
Rarely, children require splinting or serial casting to help decrease contractures in joints unresponsive to medical treatment. Leg-length discrepancy can result from neovascularization of growth plates of an affected knee. The problem may not be detected in patients with a knee flexion contracture until the contracture is corrected. Treatment consists of a shoe lift on the contralateral side. In a single-blind, randomized, controlled trial involving 60 children with JIA, Coda and colleagues found that the use of inexpensive prefabricated fitted foot orthoses reduced pain and improved quality of life compared with use of sham orthoses.
Changes in pain were measured with a visual analogue scale and quality of life was measured with the Pediatric Quality of Life questionnaire. The study included 60 children with lower joint involvement beginning at age years, previous failure of orthotic management with no use of orthoses for at least 3 months, the ability to walk 15 meters without assistive devices, and a 6-month history of disease-modifying antirheumatic drugs.
At 3- and 6-month follow-ups, children in the fitted orthoses group experienced significantly greater pain reduction and improvements in quality of life than controls. Advances in medical treatment have reduced the need for surgical intervention in JIA. Possible procedures include synovectomy, osteotomy and arthrodesis, and hip and knee replacement. Synovectomy is rarely needed, and long-term outcome is poor; however, it may be used in children in whom a single joint or just a few joints are involved and who have very active, proliferative synovitis.
Osteotomy and arthrodesis are salvage procedures for patients whose JIA is associated with severe joint destruction or deformity.
Arthrodesis is superior to arthroplasty for children who have rheumatic disease in the wrist and fingers and in the ankle. Total hip and knee replacements provide excellent relief of pain and restore function in a functionally disabled child with debilitating disease. The role of total hip replacement and total knee replacement in JIA is fraught with problems, however. Joint replacement is usually delayed until bone growth has completed, as indicated by epiphyseal closure. MAS is a rare but important complication of systemic-onset JIA in which numbers of all 3 bloodlines become rapidly decreased.
Hypofibrinogenemia, thrombocytopenia, and elevated aspartate aminotransferase levels are hallmarks. MAS often responds to cyclosporin A, and some case reports have detailed a response to anakinra. Treatment of MAS is a medical emergency and should be performed by physicians familiar with this complication. Uveitis is often asymptomatic. Patients are typically young girls who have positive levels of ANA.
Treatment with topical corticosteroid medication and with mydriatic agents to prevent closed-angle glaucoma often can prevent progression of disease to development of calcium deposition in the lens band keratopathy and adhesions of the iris to the lens posterior synechiae , in which an irregular pupillary margin develops.
Such complications may herald a chronic active disease in which vision is threatened. Immunosuppressive agents, such as methotrexate or cyclosporine, may help control chronic uveitis. Infliximab can be effective in some patients who are resistant to immunosuppressive agents. A multicenter, double-blind, randomized, placebo-controlled trial by Ramanan et al reported that adalimumab plus methotrexate therapy controlled inflammation and was associated with a lower rate of treatment failure than placebo among children and adolescents with active JIA-associated uveitis.
The study also reported a higher rate of adverse events eg, oropharyngeal pain, cough, arthralgia in the adalimumab group No specific diet helps in the treatment of JIA. However, because active JIA has been associated with decreased osteoblastic activity and a risk of osteopenia, encourage the inclusion of at least 3 servings of calcium-rich foods each day.
Consider supplementation when poor calcium intake persists. Rarely, overall caloric intake is poor and supplementation is required. Encourage patients to be as active as possible. Bed rest is not a part of the treatment. In fact, the more active the patient, the better the long-term prognosis. Children may experience increased pain during routine physical activities.
As a result, these children must be allowed to self-limit their activities, particularly during physical education classes.
A consistent physical therapy program, with attention to stretching exercises, pain modalities, joint protection, and home exercises, can help ensure that patients are as active as possible. In addition to a pediatric rheumatologist when available , the subspecialty team may include a nurse, physical and occupational therapists, social worker, ophthalmologist, and orthopedic surgeon. A nurse may provide patient education through nursing care. Although at presentation, arthritis may be so active as to preclude the use of an aggressive program of muscle strengthening, physical and occupational therapists are an important part of treatment.
The use of pain modalities during this period may permit the gradual introduction of an active program of exercises and stretching. Social work evaluation helps to determine how well each family is coping with their child's disease in terms of emotional and financial resources. Social workers can offer invaluable guidance that helps children maintain healthy relationships within their families and at school.
Transition programs for adolescents with arthritis can help to prepare them for higher education and vocation. A pediatric ophthalmologist provides slit-lamp examinations to exclude uveitis, and a pediatric orthopedic surgeon is essential when orthopedic diagnoses are being considered. The spectrum of specialists may be required such as pediatric hematologist for evaluation for malignancy or a pediatric gastroenterologist if inflammatory bowel disease is suspected.
A complete blood cell count and measurement of liver enzymes and serum creatinine should be part of routine follow-up in JIA patients. For JIA patients receiving NSAIDs on a long-term daily basis, these tests, plus urinalysis, should be done twice yearly; in patients taking these agents days per week, testing should be repeated annually. In JIA patients taking methotrexate, these tests should be conducted approximately 1 month after initiation of routine use and approximately months after any increase in dose.
If prior results were normal and the patient is on a stable dose, the tests can be repeated approximately every months. Tuberculosis screening should be repeated approximately once yearly.
Guidelines for the management of rheumatoid arthritis: Initiation and safety monitoring of therapeutic agents for the treatment of arthritis and systemic features. Arthritis Care Res Hoboken. Lamer S, Sebag GH.
MRI and ultrasound in children with juvenile chronic arthritis. Temporomandibular joint involvement in juvenile idiopathic arthritis: MRI findings of juvenile psoriatic arthritis.