Triamcinolone acetonide and hexacetonide intra-articular treatment of symmetrical joints in juvenile idiopathic arthritis: a double-blind trial.

OBJECTIVE: Pharmacokinetic studies have shown that the biological effect of triamcinolone acetonide (TA) is equivalent to that of triamcinolone hexacetonide (TH), if used at double the dosage. In this study we compared the efficacy of intra-articular TA at a dose twice that of TH in symmetrically involved joints, in children with juvenile idiopathic arthritis (JIA). METHOD: Children with active arthritis and a similar degree of inflammation in two symmetrical joints were enrolled in the study. The symmetry was assessed by both clinical examination and synovial fluid analysis. The dose given was 1 mg/kg up to 40 mg of TH or 2.0 mg/kg up to 80 mg of TA. The identity of injected compound was blinded to the patient and to the physician. RESULTS: Thirty-seven patients, 30 female, seven male, with JIA, entered the study. A total of 86 joints were injected. Twenty-one (53.8%) of the joints injected with TA relapsed first compared with only six (15.4%) of the joints injected with TH. In three (7.7%) relapse occurred simultaneously. Nine (23%) were still in remission after 24-month follow-up. The percentage of joints with lasting remission was higher with TH than with TA (80 vs 47.5% after 12 months and 63.6 vs 32.4% after 24 months, respectively; log rank test P = 0.003). CONCLUSION: Even when TA is given at higher doses, TH is more effective and should be considered the drug of choice for intra-articular treatment of JIA.

Nomenclature and classification of juvenile idiopathic arthritis: where to after Durban?

Abstract The Durban classification system for juvenile idiopathic arthritis (JIA) is reviewed in a historical context and in association with a review of problems that have become apparent. These include: (i) a family history of psoriasis as an exclusion factor (oligoarthritis and enthesitis-related arthritis) and as in inclusive factor (psoriatic arthritis); (ii) a family history of HLA-B27-associated disease as an exclusion factor (oligoarthritis) and an inclusive factor (enthesitis-related arthritis); (iii) the requirement of a dermatological opinion for psoriasis; (iv) the absence of HLA-B27 antigen in proband, with the presence of antigen in the family history; (v) the definition of time of onset; (vi) the presence of rheumatoid factor (RF) with oligoarthritis; (vii) HLA-B27-positive males with an onset of arthritis after 8 years of age. Modifications are suggested to maintain the homogeneity of groupings for research, whilst providing a practical scheme for clinicians. Three main modifications are suggested. (A) That the family history be included in descriptors rather than as inclusive or exclusion criteria. (B) Further development of the hierarchical system, which is partly used in the Durban classification. (C) That the following changes be made: rheumatoid-factor-positive oligoarthritis and polyarthritis be classified together; extended oligoarthritis and polyarthritis be classified together; HLA-B27-positive disease be classified with fewer inclusive and exclusion criteria; the criteria for psoriatic arthritis be modified; the classification of the disease in a particular child be changed in the event of relevant changes in the child's disease or laboratory profile. These suggestions are made to stimulate discussion.

Chronic recurrent multifocal osteomyelitis with Crohn's disease exacerbation and vasculitis after granulocyte colony-stimulating factor therapy.

Chronic recurrent multifocal osteomyelitis (CRMO), of unknown etiology, is characterized by recurring non-suppurative lesions of bone in multiple sites, and has been considered to be self-limiting. Reported therapies include prolonged antibiotics, corticosteroids and anti-inflammatory medications. This case is presented to illustrate the following: 1) CRMO may be severe, on-going, and unresponsive to treatment; 2) it may be associated with Crohns' disease; 3) the use of granulocyte colony-stimulating factor (G-CSF) may be associated with severe gastrointestinal vasculitis. A male was treated from ages 11-20 years for CRMO (manifesting as multiple bone lesions), with therapies of variable efficacy (anti-inflammatories, antibiotics, corticosteroids, gammaglobulin and methotrexate). With increasing disruption to his life, a 10-day course of granulocyte colony-stimulating factor (G-CSF) was given with benefit seen on magnetic resonance imaging (MRI). With exacerbation of symptoms one month later, G-CSF was re-commenced but ceased after 3 weeks because of abdominal pain, rectal blood loss, and progression of bone lesions with subsequent removal of portions of ileum, colon and appendix, which showed vasculitis. Months later, a colonoscopy revealed perianastomotic ulcers and continuing gastroenterological ulceration not unlike Crohn's disease. With azathioprine, gut and bone symptoms improved. We conclude that 1) CRMO may adversely affect life for years; 2) proven treatments are unavailable; 3) gastroenterological vasculitis/ Crohn's may be associated with CRMO; 4) MRI is useful for monitoring CRMO; 5) In this patient, G-CSF seemed beneficial initially, but later, vasculitis (possibly Crohn's) manifested, leading to bowel resection; 6) Crohn's disease may have been present for years, masked by corticosteroid, and unmasked by reduction of steroids and use of G-CSF.

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.

Are growing pains a myth?

BACKGROUND: 'Growing pains' are the commonest musculoskeletal problem of children. OBJECTIVE: This article reviews the current understanding and provides a practical approach to diagnosis and management of growing pains. DISCUSSION: Growing pains is an accepted medical term, though the pains are not due to growing. The typical case of growing pains occurs in a healthy, clinically normal, young child, in the middle of the night, causing intense pain for 10 to 15 minutes in both legs (knees, thighs, calves or shins). Management is simple analgesic measures for the child, and reassurance for the parents that a serious condition is not present.

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.

Genetic studies in familial fibrosing alveolitis. Possible linkage with immunoglobulin allotypes (Gm).

A family containing 12 subjects spanning three generations and including six cases with clinical evidence of definite or probable fibrosing alveolitis has been investigated. Histologic confirmation was available for three cases. The subjects were between 15 and 54 years of age at diagnosis. Although the size of the sample is small, the mode of inheritance of fibrosing alveolitis within this family appeared to be dominant with incomplete penetrance. HLA typing showed that at least one of the affected siblings did not share any HLA haplotypes with other affected siblings in the third generation. This makes it unlikely that a disease gene would be in association with HLA genes on chromosome 6. In contrast, all affected siblings, as well as two as yet unaffected siblings, carried the immunoglobulin haplotype Gm 1. These studies indicate that familial fibrosing alveolitis in this family may be inherited by a dominantly inherited gene located on chromosome 14 close to the loci encoding for Gm.

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.