Delay in diagnosing juvenile arthritis.

Juvenile arthritis may present as swollen fingers or toes or joint swelling after minor trauma. The diagnosis can easily be overlooked, as small children do not complain about pain. Untreated arthritis can cause deformities or, with eye involvement, damaged vision. Three case histories are presented, as well as an audit of diagnostic delay in children with juvenile arthritis presenting to a paediatric rheumatology service over 12 months.

Prevalence of juvenile chronic arthritis in a population of 12-year-old children in urban Australia.

OBJECTIVE: To conduct a cross-sectional, community-based, point prevalence study of inflammatory joint disease and other rheumatic disorders in 12-year-old children in a metropolitan community. METHODS: After completion of a pilot study of 816 10-year-old children, a cross-sectional prevalence study was performed 2 years later on a randomized sample of 2241 12-year-old children (including the cohort from the pilot study) from a community of approximately 221 700 children aged 12 years or younger, with 17 300 children aged approximately 12 years. A rheumatologic examination was performed on each child by a single observer after perusal of completed questionnaires from parents and children. RESULTS: Three of 816 children in the pilot study were shown to have juvenile chronic arthritis (JCA), fulfilling the criteria of the European League Against Rheumatism for the diagnosis of JCA. Only 1 of 3 had a previous diagnosis of JCA. The prevalence was 3.7 per 1000. Of 2241 children examined 2 years later, 89% returned two questionnaires (one completed by the parent and one by the child). At examination, 38 swollen joints were identified in 32 children. Nine children were identified with JCA, of whom 7 had not had previous diagnoses. No questions from the questionnaires identified the 7 children with previously undiagnosed JCA. The point prevalence of JCA in this community was 4.0 per 1000. Although the children with newly diagnosed cases tended to have mild disease, it was associated with significant morbidity and the potential for serious morbidity. CONCLUSIONS: This is the first reported prevalence study of JCA in which case ascertainment was based on clinical examination by a rheumatologist of children within a community. The prevalence of 4.0 per 1000 was significantly higher than the accepted prevalence of 0.6 to 1.1 per 1000. A study based on known cases would have significantly underestimated the true prevalence of JCA in this community, with 7 of 9 cases being previously undiagnosed. Questionnaires were not effective in identifying children with undiagnosed JCA, clinical examination supported by a history from the parent and child providing the only reliable means of diagnosis. It is possible throughout the world that the numbers of undiagnosed cases of JCA significantly exceed the numbers of known cases with the true prevalence being significantly higher than the levels currently accepted.

Guillain-Barré syndrome presenting with severe musculoskeletal pain.

Three children with Guillain-Barré syndrome are described who presented with musculoskeletal pain to an orthopaedic clinic or to a paediatric rheumatologist at a children's hospital. In each case, osteomyelitis of the spine was considered to be the most likely diagnosis and bone scans were performed in two of the three patients. Two of the three children required care in an intensive care unit, within hours of diagnosis. Outside of the specialty of neurology, the presentation of Guillain-Barré syndrome with severe muscle pain is not generally well known. With greater awareness of this particular presentation, dangerous delays in diagnosis and inappropriate investigations will be minimized.

A multicentre, long-term evaluation of the safety and efficacy of ibuprofen syrup in children with juvenile chronic arthritis.

The safety, efficacy and acceptability of ibuprofen syrup were assessed in a multicentre controlled open study in children with juvenile chronic arthritis. Forty-six children aged 18 months to 13 years (mean 6.8 years) were studied. Dosage commenced at 10 mg/kg/day and increased to a maximum of 40 mg/kg/day depending on condition and individual disease control. Follow-up assessments of disease severity, active joint count and any side effects were made at each clinic visit, usually monthly or as often as deemed necessary by the physician. Thirty-nine children completed the minimum eight weeks treatment period, with average duration of treatment being eight months. Seven children did not complete the minimum required treatment period, of which four were lost to follow-up or non-complaint, two had suspected adverse reactions, and one had a taste complaint and nausea. Adverse reactions were predominantly gastrointestinal, but only one was severe enough for discontinuation from the study before the end of the protocol period. Two children had minimal benefit from treatment and were changed to other medication, 10 went into remission during the study period and four were lost to follow-up. Twenty-three children continued on ibuprofen syrup or tablets after the study period. This study demonstrates that ibuprofen is a well tolerated anti-inflammatory agent for children with juvenile chronic arthritis, and that the syrup form is particularly useful for small children who may not be able to swallow tablets.

Clinical considerations in Buschke-Ollendorff syndrome.

This brief report describes a mother and two daughters with the rare Buschke-Ollendorff syndrome. That the typical dermal connective tissue naevi lesions may become less obvious with time and that the condition is not always so "benign" are important clinical features not well recognised. Incorrect diagnosis may lead to embarkation upon hazardous management.

An immunogenetic study of juvenile chronic arthritis.

Ninety-seven patients from Western Australia and Victoria suffering from juvenile chronic arthritis (JCA) for longer than 1 year were studied with particular reference to immunogenetic markers in the sub-groups of the disease. In the pauci-articular group, the antigens DR5, DR8 and BW35 were increased in frequency. The phenotypes within the sub-groups appeared distinctive despite the absence of any increase in the frequency of single antigens. Complement allotyping did not prove to be different from controls or within the sub-groups for JCA. This study supports the concept that patients with JCA have specific genetic characteristics and that sub-groups of the disease are genetically distinct.

Slow-acting antirheumatic drug use in systemic onset juvenile chronic arthritis.

The records of patients with systemic onset juvenile chronic arthritis were reviewed to study the effects of slow-acting antirheumatic drugs on systemic features of the disease and joint manifestations. Frequency and severity of side effects were also evaluated. The following conclusions resulted from this study. Most children could be treated with an alternate-day corticosteroid regimen. Gold and D-penicillamine treatment was not effective or tolerated during the systemic phase. However, after that phase, joint disease was controlled in 42% and 60% of children, respectively. Chlorambucil treatment was helpful in patients with amyloidosis and in those patients in whom all other drugs had failed. The incidence of side effects of chlorambucil are high, however. Antimalarial drug treatment was well tolerated but ineffective during the systemic phase. It was effective on joint disease alone in 44% of a small number of patients. Azathioprine treatment was well tolerated and effective in 50% of patients.

A retrospective study of juvenile chronic arthritis.

During a 20 year period 214 patients had been admitted to a teaching hospital with a diagnosis of definite or possible juvenile chronic (rheumatoid) arthritis (JCA). Eighty-seven of these patients were reviewed clinically and were classified as having had JCA. Twelve of the 214 patients were later thought to have had rheumatic fever, while 12 had had an illness consistent with viral arthritis. There was a poor functional outcome in three subgroups of JCA: (i) seropositive polyarticular onset (ii) systemic onset, and (iii) pauciarticular onset, extending to polyarticular involvement. The prevalence of inflammatory eye disease was very low with no significant visual handicap detected in patients in this study.