What do you mean by JUVENILE RHEUMATOID ARTHRITIS?
Juvenile rheumatoid arthritis is the condition of chronic synovitis in children.
Still postulated that several distinct conditions were responsible for chronic arthritis in children. Still, unfortunately, little work was done in the field of pediatric rheumatology until recent years. Most modern observers have come to agree with Still that chronic arthritis in children encompasses several distinct disease subgroups. The relationship of juvenile rheumatoid arthritis to adult rheumatoid arthritis remains uncertain; some of the subgroups described in children are not recognized in adults, whereas classic adult-type rheumatoid arthritis occurs in children, but accounts for only a small percentage of total patients. The nomenclature for chronic childhood arthritis is quite confusing at present. The term
Juvenile rheumatoid arthritis
(JRA) has been retained in the United States to describe all children with chronic arthritis, whereas the term juvenile chronic arthritis is becoming more widely used in Europe. The term Still disease, although according to due honor to an important person, is confusing and should probably be discarded.
Juvenile RHEUMATOID arthritis
remains the major chronic and progressive crippling disease of childhood. In the United States alone, there may be as many as 175,000 cases, but this is only a rough estimate since no survey of any chronic children’s disease has ever been undertaken. Recent investigations, though chiefly descriptive, have added greatly to therapeutic judgment in the management of this disease. Yet as management has improved, certain basic problems, especially concerning etiology, seem no closer to being understood than they were some decades ago.
SYMPTOMS OF JUVENILE RHEUMATOID ARTHRITIS:-
Symptoms regarding ocular and systematic characteristics
The ocular and systemic characteristics of 160 patients with anterior uveitis and seronegative juvenile rheumatoid arthritis are reviewed. Chronic uveitis occurred in 131 patients, 76% of whom were girls. Both eyes were involved in 70% of the cases. Band keratopathy occurred in 41% of the eyes, cataract in 42%, and secondary glaucoma in 19%. Only 11 patients had uveitis before the onset of arthritis. Notable correlations included a pauciarticular onset of arthritis in 95% of the patients and positive tests for antinuclear antibody in 82%. Of 29 patients with acute anterior uveitis, 27 were boys. The inflammation responded well to therapy, and serious complications did not occur. At follow-up 21 patients had typical ankylosing spondylitis, and five had sacroiliitis. The incidence of positive results of tests for HLA-B27 antigen was 94%.
(A) Photograph of the patient at rest. (B) Photograph of the patient while coughing.
Symptoms of Juvenile rheumatoid arthritis in a population-based cohort of adults who had the disease during childhood
Of the 50 eligible cases, 44 (88%) responded to the survey. There were 102 age- and sex-matched controls (2–3 per case) who responded to the survey. Seventy-three percent of the cases had pauciarticular-onset Juvenile Rheumatoid Arthritis, 16% had polyarticular-onset Juvenile rheumatoid arthritis, and 11% had systemic-onset Juvenile rheumatoid arthritis. The average follow-up was 24.7 years and 24.5 years after the index date for cases and controls, respectively.
So the main symptoms like Greater disability, more bodily pain, increased fatigue, poorer health perception, and decreased physical functioning was reported by the cases compared with the controls. Juvenile rheumatoid arthritis cases reported significantly lower rates of employment and lower levels of exercise controls.
Also the level of educational achievement, annual income, health insurance status, and rate of pregnancy and childbirth were similar for both cases and controls. Adults who have had Juvenile rheumatoid arthritis during childhood experience long-term physical and psychosocial impairment of Juvenile rheumatoid arthritis.
Symptoms in children with juvenile rheumatoid arthritis (JRA).
Normally sleep abnormalities are seen in children having Juvenile rheumatoid arthritis. Patients with Juvenile rheumatoid arthritis had a higher total score on the CSHQ, as well as subscales assessing night wakings, parasomnias. sleep anxiety, sleep-disordered breathing, and morning wakening/daytime sleepiness. There were no correlations between CSHQ scores and Juvenile rheumatoid arthritis disease activity or pain variables, but the total score on the SSR did correlate with the pain. We conclude that sleep abnormalities are common in children with Juvenile rheumatoid arthritis, and are multi-dimensional.
Symptoms of Juvenile rheumatoid arthritis in children aged 0.9-13.7 years
In the incidence group, 4/47 children changed subtype during the disease. All did so within 2 years from disease onset, and the same observation was made in the cross-sectional group. Uveitis was described at onset in a single case, and no child developed uveitis later. In patients from the incidence group in the process of being transferred to adult rheumatology clinics, 48% were still taking medication. Patients who had involvement of proximal interphalangeal (PIP) joints at onset had an increased risk of being active or stable at follow-up (RR 12.3, 95% CI 1.4-108.3). A higher chance of no continuing disease activity at follow-up was observed in children with oligoarticular disease than in the other subtypes (RR 2.8, 95% CI 1.2-6.9). Uveitis associated with antinuclear antibody-positive JCA and psoriatic arthritis in Costa Rican children is uncommon, and the risk of developing uveitis remains low during the disease. The involvement of PIP joints predicts an increased risk of continuing disease. The course of JCA in Costa Rican children is not milder than in Caucasian populations, since 48% of the patients showed persistent disease activity at the transition to adult care.
During glucocorticoid treatment, the serum ferritin concentrations normalized rapidly, usually within a few weeks. The rate of normalization reflected the response of the fever to treatment. Later, subnormal concentrations were found, which were unrelated to the activity of arthritis. Thus, serum ferritin is a useful guide when tapering glucocorticoid dosage.
Symptoms regarding Consumption coagulopathy associated with systemic juvenile rheumatoid arthritis
A coagulopathy resembling disseminated intravascular coagulation may occur in systemic juvenile rheumatoid arthritis. We have seen this in seven patients with three different circumstances of disease activity or drug treatment. In one patient, a coagulopathy was not associated with drug therapy and required corticosteroid therapy for control. The second group of patients was receiving orally nonsteroidal anti-inflammatory drugs during an acute flare-up of disease associated with low serum albumin concentrations. Coagulopathy in these patients may be a result of reduced vascular endothelial cell cyclooxygenase activity secondary to increased levels of unbound nonsteroidal anti-inflammatory drugs. In these children, corticosteroid therapy was required for control. The third form of coagulopathy was seen in patients receiving a second injection of aurothiomalate. This form appears to be idiosyncratic, self-limiting, and relatively benignrcept in Children with Polyarticular Juvenile Rheumatoid Arthritis (A) CT scan of the thorax performed during inspiration shows no evidence of lung herniation. (B, C) CT scan of the thorax performed during a Valsalva maneuver shows herniated lung at the right apex.
1. Treatment with etanercept
We evaluated the safety and efficacy of etanercept, a soluble tumor necrosis factor receptor Fc fusion protein, in children with polyarticular juvenile rheumatoid arthritis who did not tolerate or had an inadequate response to methotrexate.
At the end of the open-label study, 51 of the 69 patients (74 percent) had had responses to etanercept treatment. In the double-blind study, 21 of the 26 patients who received placebo (81 percent) withdrew because of disease flare, as compared with 7 of the 25 patients who received etanercept (28 percent) The median time to disease flare with placebo was 28 days, as compared with more than 116 days with etanercept. In the double-blind study, there were no significant differences between the two treatment groups in the frequency of adverse events.
2. Treatment with methotrexate
New information on the treatment of juvenile rheumatoid arthritis emphasizes more aggressive control of arthritis, particularly the use of methotrexate, both in low- and higher-dose regimens. Information concerning drug toxicity, including that of the nonsteroidal anti-inflammatory drugs, second-line agents, and methotrexate, suggests that these drugs are well tolerated in children. A new corticosteroid, deflazacort, minimizes bone demineralization and growth retardation. Adjunctive measures, including erythropoietin, pain management techniques, conditioning programs, and nutrition, have demonstrated advantages in some children with juvenile rheumatoid arthritis.
3. Treatment with polyarticular disease course
Nowadays, Prognostic factors in juvenile rheumatoid arthritis (JRA) include polyarticular onset, polyarticular disease course, and rheumatoid factor positivity; in the systemic onset subtype, the persistence of systemic features at 6 months after onset confers a worse prognosis. Timely diagnosis and appropriately aggressive treatment of patients with poor prognostic features improve quality of life and outcome. After nonsteroidal anti-inflammatory drugs, methotrexate is the most commonly used second-line agent. However, approximately one-third of patients do not respond to methotrexate adequately. Randomized, placebo-controlled, clinical trials in patients with Juvenile rheumatoid arthritis are few, but one such trial with the tumor necrosis factor inhibitor etanercept shows that this drug is effective and well-tolerated. Other recently approved agents for rheumatoid arthritis, including infliximab, leflunomide, celecoxib, and rofecoxib, have not been adequately studied in pediatric patients, and the role of these agents in children with Juvenile rheumatoid arthritis remains to be determined.
4. Treatment with rheumatology
Recently interest in pediatric rheumatology has increased. This review tries to summarize our knowledge about pharmacological mechanisms, as well as practical application and adverse of antirheumatic drugs. In addition, forms of treatment that so far lack any scientific backing are mentioned and discussed. Various data about efficacy and side effects in children are poor or lacking, and current treatment in children is based on analogy conclusions derived from experiences with adult RA. The therapeutic concept presented in this article offers a good chance to improve the functional status of the rheumatic child. However, one should be aware that several controlled trials with antirheumatic drugs in children still have to be performed. Uncertainties have to be replaced by knowledge. This is true also for the surgical treatment mentioned.
5. Treatment in adults with rheumatology
Advances in pediatric rheumatology have closely paralleled those in adult rheumatology, but several unique features of Juvenile rheumatoid arthritis have led to some prominent differences. Methotrexate has become the gold standard for use in moderate to severe diseases. A variety of the second-line agents and newer methods of corticosteroid dosing can be used in milder disease or in patients who fail or refuse methotrexate. Biologic agents and newer immunosuppressive agents hold out significant promise for those with the most severe disease. As for adult disease, a cure for arthritis is far away, but the lives of these patients have truly been revolutionized by the wide array of newer therapies.
6. Treatment with MR imaging
Thirty-three joints of the appendicular skeleton in 15 children with Juvenile rheumatoid arthritis were examined with magnetic resonance (MR) imaging to determine if it could demonstrate synovial hypertrophy and the status of the articular cartilage. Presumed synovial hypertrophy was seen in 13 joints as masses of varying sizes of low to intermediate signal intensity on T1- and T2-weighted images; sometimes foci of increased signal intensity, most likely due to fluid or inflammation, were seen on T2-weighted images. Probable abnormal articular cartilage was detected in ten joints, and MR imaging also demonstrated epiphyseal overgrowth, bone erosions, joint effusions, and joint space narrowing. Because MR imaging appears to provide an objective method of evaluating both synovial hypertrophy and the status of articular cartilage, it may prove to be useful in monitoring the progression of juvenile rheumatoid arthritis and response to therapy.