SYSTEMIC-ONSET JUVENILE CHRONIC ARTHRITIS AND BONE MARROW HYPOPLASIA

A two-and-a-half-year-old girl with systemic-onset JCA developedpancytopaenia 21 days after her illness began. Bone marrow examinationrevealed hypoplasia, with no evidence of erythrophagocytosis.Two weeks later peripheral blood specimens showed evidence ofbone marrow regeneration. No definite cause for the hypoplasiawas found subsequently. Persistent haematological abnormalitiesafter resolution of the hypoplasia included anaemia of chronicinflammation, leucocytosis and thrombocytosis. Unexplained bonemarrow hypoplasia has not previously been described in systemic-onsetJCA. KEY WORDS: Systemic-onset JCA, Bone marrow hypoplasia, Pancytopaenia, JCA, Aplastic anaemia     

SIALOCHEMISTRY IN JUVENILE CHRONIC ARTHRITIS

Stimulated parotid gland secretions collected from 16 patientswith juvenile chronic arthritis (JCA) were analysed and theresults compared with those obtained from 83 healthy sex-, age-and socioeconqmic status-matched children. Parotid salivaryflow rate was measured and the saliva samples were assayed forcalcium, phosphorus, potassium, chloride, sodium, urea, lysozyme,amylase and immunoglobulin levels (IgA, IgG, IgM). Our resultsshowed that parotid flow rate (PFR) values in JCA patients werenot statistically different from those in healthy controls.However, the mean salivary concentrations of calcium, phosphorus,potassium, lysozyme and IgA were significantly lower in thepatients. These data could provide an explanation for the increasedincidence of caries and gingivitis observed in JCA. KEY WORDS: Parotid flow, Calcium, Phosphorus, Lysozyme, IgA, Dental caries     

FOOT FUNCTION IN JUVENILE CHRONIC ARTHRITIS

Measurement of the force distribution under the foot has beenused to assess the effect of juvenile chronic arthritis on footfunction. The results obtained from the feet of eight patientswere compared with the feet of 11 normal children. The peakforces and contact times were measured for functional areasof the foot. When considered as a group, the patients had significantlyreduced loading under all the toes and the two medial metatarsalheads, and increased contact time for the heel.     

INFLAMMATORY CYTOKINE RESPONSES IN JUVENILE CHRONIC ARTHRITIS

The inflammatory cytokines interleukin 1ß (IL-1ß)interleukin 6 (IL-6), tumour necrosis factor alpha (TNF     

SENSORINEURAL HEARING LOSS IN JUVENILE CHRONIC ARTHRITIS

An 18-year-old female with pauciarticular juvenile chronic arthritisis described who has developed a profound bilateral sensorineuralhearing loss. The association of sensorincural hearing losswith juvenile chronic arthritis has not been reported before. KEY WORDS: Juvenile chronic arthritis, Sensorineural hearing loss     

THE PAIN THRESHOLD IN JUVENILE CHRONIC ARTHRITIS

An algometer was used to study the pain threshold (PT) on pressureat the joint capsules of the wrists, elbows, knees, ankles andat paravertebral soft tissues in 57 patients with juvenile chronicarthritis (JCA) and 69 healthy controls, aged 6–17 yr.The PTs were correlated to visual analogue scales (VAS) andvalues of articular disease activity, to define their relationwith pain perception and joint inflammation. The PTs in JCApatients were significantly lower than in their healthy peers,both in children with active inflammation as well as in childrenwithout detectable inflammation. The JCA group with active inflammationshowed significant lower PTs than without detectable inflammation.Disease activity and VAS correlated significantly with PT (r= -0.5). There is an enhanced sensitivity to noxious stimuliin all measured body areas, suggesting a change in the painprocessing system in JCA. The sensitivity endures in JCA patientswithout clinically active disease, probably due to prolongedcentral sensitization after periods of active disease. KEY WORDS: Algometry, Pain measurement, Pain modulation, Noxious stimulation, Pressure (pain) threshold, Children, system, Juvenile chronic (rheumatic) arthritis     

THE FUNCTIONAL AND PSYCHOLOGICAL OUTCOMES OF JUVENILE CHRONIC ARTHRITIS IN YOUNG ADULTHOOD

The functional and psychological outcome of 43 patients withpolyarticular juvenile arthritis was assessed in order to evaluatethe impact of disease on their quality of life. Mean diseaseduration was 19.7 yr (range 10–39 yr), mean age 26.7 yr(range 18–54 yr) with sex ratio 1:3 (male : female). Severedisability was present in 8% of systemic onset, 34% of RF negative(Rh – P), 38% of RF positive (Rh + P) and 86% of extendedpauci-articular (ExP) juvenile arthritics—this last highpercentage was due to ocular impairment. The Rh + P had morehip (100% of the group) and knee prostheses (31%) compared withRh – P (77 and 11% respectively). The Rh + P and ExP groupswere the most disabled with the highest proportions of patientswith currently active disease (85 and 71% respectively). Psychological testing showed that 21% of the patients were clinicallydepressed and the rate increased (P = 0.06) with the degreeof disability. The proportion of patients demonstrating an anxiouspreoccupation with their disease increased (P = 0.002) withthe degree of disability. Despite this, 66% of patients wereemployed and 38% felt that their arthritis had no effect ontheir ability to form relationships. KEY WORDS: Juvenile chronic arthritis, Outcome, Prognosis, Functional, Psychological, Disability     

INCREASE OF ACTIVATED FACTOR VIIA AND HAEMOSTATIC MOLECULAR MARKERS IN JUVENILE CHRONIC ARTHRITIS

Activated factor VIIa (FVIIa), von Willebrand factor antigen(vWF:Ag), D-dimer and thombin-antithrombin III complex (TAT)were measured to monitor coagulation status in patients withjuvenile chronic arthritis (JCA). Subjects included 14 patientswith systemic JCA, 16 with pauciarticular JCA and 16 with polyarticularJCA without disseminated intravascular coagulopathy, thrombosisor liver dysfunction. All types of JCA showed an increase ofFVIIa, D-dimer and TAT, indicating enhanced activation of coagulation.In systemic JCA only there was also characteristically an elevationof vWF:Ag. We conclude that all types of JCA constitute a stateof subclinical hypercoagulopathy caused by tissue damage andthat additionally systemic JCA involves a prothrombotic stateassociated with or precipitated by vasculitis. KEY WORDS: Factor VIIa, Juvenile chronic arthritis     

SURGICAL MANAGEMENT OF THE HIP IN JUVENILE CHRONIC ARTHRITIS

The hip joint is the most important joint as far as surgicalmanagement of JCA is concerned. Approximately a third of childrenwill develop hip involvement, leading to pain and deformity.A wide spectrum of hip joint abnormalities may occur, beingthe result of either under-development or over-development ofthe bones and erosive change secondary to the synovitis. Forthe acutely irritable hip with no fixed deformity an intra-articularinjection of Triamcinolone Hexacetonide may be beneficial. Forthose hips with a fixed deformity, resulting in functional impairmentin children prior to the closure of the proximal femoral capitalepiphysis, a soft tissue release of selected muscles aroundthe hip joint can correct deformity and improve function. Forthe painful destroyed hip joint, total joint replacement isindicated. Cemented hip replacements have been shown to havea high incidence of loosening in this patient group and considerationis being given to the use of customized uncemented femoral components. KEY WORDS: Juvenile chronic arthritis, Soft tissue release, Hip joint replacement     

STUDY OF ANTIBODIES TO HISTONES AND HISTONE SYNTHETIC PEPTIDES IN PAUCIARTICULAR JUVENILE CHRONIC ARTHRITIS

The objective of this study was to determine the prevalenceof histone antibodies in early onset pauciarticular juvenilechronic arthritis (JCA), and to assess any association withdisease activity in terms of arthritis or uveitis. Forty-four children with early onset pauciarticular JCA havebeen assessed prospectively using clinical and laboratory parameters.Sera were collected from these patients during clinical follow-up.Some of them had serial measurements during the period of study.Patient sera were analysed for histone antibodies by ELISA.A further study using 30 synthetic peptides of calf thymus corehistones was performed by ELISA. Seventy-five per cent of children have IgM antibodies to histone,usually to more than one type. The highest values were antiH3 and H4 antibodies. There was no correlation between the levelof anti-histone antibodies and anterior uveitis. There were increased levels of IgG antibodies to histone peptides,in particular the N- and C-terminal peptides of H3 in 70.5%of children. Antibodies to H3 peptides tend to be in patientswith chronic anterior uveitis (P = 0.04). KEY WORDS: Juvenile chronic arthritis, Juvenile rheumatoid arthritis, Chronic anterior uveitis, Chronic iridocyclitis, Anti-histone antibodies, histone synthetic peptides     

EPSTEIN-BARR VIRUS-ASSOCIATED HAEMOPHAGOCYTIC SYNDROME IN A PATIENT WITH JUVENILE CHRONIC ARTHRITIS

We describe a case of reactive haemophagocytic syndrome (RHS)in a patient with previous polyarticular juvenile chronic arthritis(JCA). This is the first reported association of these conditionsand may be indicative of defective immunological responses toviral infections in this form of JCA. KEY WORDS: Epstein–Barr virus, Haemophagocytic syndrome, Juvenile chronic arthritis, Acquired coagulation disorder, Interferon-     

SUCCESSFUL TREATMENT OF. JUVENILE CHRONIC ARTHRITIS WITH A SPECIFIC ANTIVIRAL AGENT

In a previous paper we identified a group of patients with teenageonset chronic progressive arthritis (JCA or JRA) with raisedantibody titres to influenza A (H₂N₂), an epidemic of whichwas present in the year they were born. On the basis that theymight be chronic carriers of influenza A, and that this mightbe related to their arthropathy, it was decided to use the anti-influenzaA drug amantadine to treat the virus and observe whether therewas any effect on the joint disease. A 4-month, double-blind,placebo-controlled trial followed by a 4-month open study showedthat amantadine could under these circumstances be of considerabletherapeutic benefit while having no effect on patients withoutelevated antibody titres against influenza A. KEY WORDS: Influenza A, Amantadine, Rheumatoid arthritis, Carrier state, Remission     

ANTIBODIES TO INFLUENZA A IN A CLUSTER OF CHILDREN WITH JUVENILE CHRONIC ARTHRITIS

In this study of 41 patients with progressive juvenile chronicarthritis (JCA), born between 1946 and 1970, it was noticedthat 14 were born in the same year (1963). The possibility ofa common environmental factor was therefore investigated. Recordsshowed that an epidemic of influenza A H₂N₂ was present in thatyear, and the study shows that JCA patients born in 1963 stillhave a higher level of antibody to influenza A H₂N₂ than JCApatients born in other years or age-matched controls. This elevationis not seen in a survey of three control viruses. Since thisgroup developed their clinical JCA after the appearance of influenzaA H₂N₂ in 1977, it is suggested that these patients developeda progressive arthropathy because they had been pre-sensitizedto influenza A by contact with an earlier strain when in utero. KEY WORDS: Influenza A, Juvenile chronic arthritis, Birthdate, Clustering     

PHYSIOTHERAPY AND OCCUPATIONAL THERAPY FOR JUVENILE CHRONIC ARTHRITIS: CUSTOM AND PRACTICE IN FIVE CENTRES IN THE UK, USA AND CANADA

Physiotherapy and occupational therapy are widely accepted asbeing of central importance for the treatment of juvenile chronicarthritis (JCA). However, these approaches have rarely beensubject to critical scrutiny. The aims of this report are tohighlight some of the inter-centre similarities and differencesobserved in the implementation of physical and occupationaltherapy for JCA, and to emphasize the need for scientificallycontrolled research in this area. During a series of visitsto several paediatric rheumatology units in the UK, USA andCanada, three aspects of the service were noted: treatment philosophy,physical interventions used for the treatment of JCA and quality-of-lifeand independence training activities. There was general consensuswith the philosophy that early physical intervention was a vitalpart of the treatment plan for JCA, although all therapistswere concerned that compliance with treatment modalities waspoor. Differences between units in the approach to acute arthritis,the use of foot orthoses and wrist splints, the treatment ofjoint contractures and the use of general quality-of-life trainingactivities were noted. Although it was widely recognized thatcontrolled research into the efficacy of physical interventionwas needed, no centre had a co-ordinated plan for such investigations. KEY WORDS: Juvenile chronic arthritis, Physiotherapy, Occupational therapy     

RADIOGRAPHIC TEMPOROMANDIBULAR JOINT ABNORMALITIES IN PATIENTS WITH JUVENILE CHRONIC ARTHRITIS DURING A CONTROLLED STUDY OF SODIUM AUROTHIOMALATE AND D-PENICILLAMINE

Sixty-two patients with juvenile chronic arthritis (JCA) wererandomized to 50-week treatment with either sodium aurothiomalate(G) or D-penicillamine (PEN) and followed with regard to radiographicchanges of the temporomandibular joints (TMJ). The radiographicmethods comprised panoramic radiography and oblique lateraltranscranial radiography, occasionally supplemented with othermethods. The radiographs were read by a single assessor whodid not know the type of drug treatment. Radiographic progressionwas seen in one G-treated and six PEN-treated patients withpolyarticular JCA. and in one G-treated patient with pauci-articularJCA. A relation between changes in radiographic TMJ findingsand disease activity was demonstrated. Radiographic examinationof the TMJ seems to be of value in therapeutic trials in patientswith JCA. KEY WORDS: Juvenile chronic arthritis, Temporomandibular joint, Radiography, Gold, Penicillamine     

CHRONIC SARCOID ARTHRITIS

Sarcoidosis can involve almost any organ or tissue. The outcomemay be clinical recovery with radiographic evidence of residua,impairment of function of organs involved, or a continued chroniccourse. This report is of a patient who presented with chronicsarcoid arthritis without evidence of active disease in otherorgans, 20 years after sarcoidosis had been diagnosed. The clinicalpicture superficially resembled rheumatoid disease with keratoconjunctivitissicca and basal lung fibrosis.     

LACK OF TEMPORAL ASSOCIATION OF IRIDOCYCLITIS WITH IgG REACTIVITIES TO CORE HISTONES AND NUCLEOSOME SUBPARTICLES IN PAUCIARTICULAR JUVENILE CHRONIC ARTHRITIS

This study aimed to measure IgG reactivities to DNA-free nucleosomesubparticles (H2AH2B, H3H4) and nucleosome subparticles (H2AH2B–DNA,H3H4–DNA) and to evaluate the temporal relation of thesereactivities, as well as those to single core histones, withiridocyclitis (IC) in patients with antinuclear antibody positive(ANA +) pauciarticular juvenile chronic arthritis (JCA). Reactivitiesto nuclear substrates were determined by enzymatic immunoassaysin 120 sera from 45 children with ANA + pauciarticular JCA.Significantly elevated IgG levels to H3 and H4, to DNA-freenucleosome subparticles, and to the nucleosome subparticle H3H4–DNAwere present in patients with ANA + pauciarticular JCA; no evidenceof recognition of conformational epitopes was found. In bothhorizontal and follow-up studies, no relation between the reactivitiesstudied and the development, presence, or history of IC wasfound. Our results show the absence of a relation of antibodiesto histone molecules or to nucleosome subparticles with IC inpatients with ANA+ pauciarticular JCA. KEY WORDS: Juvenile chronic arthritis, Antinuclear antibodies, Iridocyclitis ^*Present address: Laboratorio Biotecnologie e Tecnologie Biomediche,IRCCS Policlinico San Matteo, 27100 Pavia, Italy.     

GAIT ANALYSIS: A PILOT STUDY--A POSSIBLE MODE OF ASSESSMENT OF LOWER LIMB FUNCTION IN JUVENILE CHRONIC ARTHRITIS

There is a need for some form of objective assessment of gaitwhich can be used clinically. A method which measures the temporaland distance factors has been employed to assess the gait ofpatients. Variables have been selected which reflect the gaitsymmetry and others have been chosen which show the speed ofwalking and how this is achieved. The method of presentationof results is discussed with particular attention to the useof these results in monitoring the progress of the patient.     

HYPERMOBILITY SYNDROME MIMICKING JUVENILE CHRONIC ARTHRITIES

It has been estimated that hypermobility of joints occurringin subjects not believed to be suffering from clinicaly identifiableheritable disorders of connective tissue is present in approximately10% of bealthy subjects (1,2). It seems highly likely that inthe majority of hypermobile subjects symptoms are not experienced.IT is now well established that hypermobility may present ina rheumatology clinic with articular complaints which includerecurrent joint efusions, predisposition to the effects of trauma,backache, recurrent dislocation of joints, and probably prematureosteoarthrosis(3). The purpose of this comunication is to describe an unusual presentationof hypermobility in which an adolescent presented with a chronicarthropathy of her fingers which mimicked juvenile chronic arthritis