Bibliography December 2003

1. BALEV S, VARBANOVA B, NIKOLOV K: Antinuclear antibodies in patients with pauciarticular and polyarticular juvenile chronic arthritis. Rheumatology (Bulgaria ) 11:1-2 48-52, 2003
   Abstract: The purpose of the research is to study the nuclear specificity of the antinuclear antibodies (ANA) in patients with pauciarticular and polyarticular juvenile arthritis and the relation between the different ANA and various parameters and forms of the disease. 92 children with pauciarticular and polyarticular juvenile chronic arthritis (JCA) of different duration have been studied. Various methods have been used to determine: ANA to native DNA, single and double-stranded DNA antibodies, antibodies to extractable nuclear antigens, and other specific nuclear antigens - Sm, RNP, Ro, La, Jo-1, Scl-70. ANA have been positive in 34%, more frequent in girls and mostly associated with pauciarticular form. Antibodies to other specific nuclear antigens have no clinical significance in any of the forms of JCA. ANA correlate with the disease activity and its duration and are risk factor for the development of uveitis, especially in girls with an infant onset

2. BUXBAUM JN: The systemic amyloidoses. Curr Opin Rheumatol 16:1 67-75, 2004
   Abstract: SUMMARY: PURPOSE OF REVIEW Clinical management of the amyloidoses has historically been the province of rheumatologists, because of the relation to long-standing inflammation in rheumatoid arthritis, ankylosing spondylitis, and juvenile chronic arthritis. Currently, nephrologists, hematologist-oncologists, neurologists, and transplant surgeons all have a diagnostic or therapeutic interest. Current advances, using the tools of physical biochemistry, cell biology, and genetics, have begun to impact the diagnosis and clinical management of these disorders and raise questions regarding our notions of protein conformation in vivo and how nonnatively folded proteins may produce disease.RECENT FINDINGS It appears that all amyloidogenic precursors undergo some degree of misfolding that allows them to populate an immediate precursor pool from which they rapidly aggregate. Depending on the particular protein, a variety of mechanisms appear operative, some of which involve nonphysiologic proteolysis, defective physiologic proteolysis, mutations involving changes in thermodynamic or kinetic properties, and pathways that are yet to be defined. Whatever the particular process, the result is a tendency toward oligomeric aggregation followed by the assembly of higher order structures that become insoluble under physiologic conditions. Detailed analyses have been described for transthyretin (senile systemic amyloidosis and familial amyloid polyneuropathy), immunoglobulin light chains (light-chain amyloid), beta2 microglobulin (dialysis-related amyloid), and apolipoprotein A1, and are in process for others.SUMMARY Therapies have been proposed based on precursor stabilization (transthyretin), elimination of the synthesizing cell (light-chain amyloid), fibril disruption and immunization to induce host-mediated aggregate clearance (Alzheimer disease, light-chain amyloid, prions), and aggressive therapy of a primary inflammatory process (amyloid A). During the next decade, the value of these therapies, and others, suggested by studies on the basic properties of cells and proteins, will become clear

3. GROH BP: Current concepts in pediatric rheumatology. Current Opinion in Orthopaedics (United States ) 14:6 385-391, 2003
   Abstract: Purpose of review: The discipline of pediatric rheumatology has derived significant benefit from recent advances in diagnosis and therapy. As the molecular understanding of inflammatory joint disease has increased, more specifically targeted biologic therapies for arthritis have come available. Children with advanced joint disease on biologic therapies may be referred to orthopedists for consideration of surgical procedures. Orthopedists should be familiar with both newer diagnostic methods and treatments for childhood arthritis. Recent findings: Improved ultrasound and magnetic resonance imaging now afford better sensitivity in the diagnosis of inflammatory arthritis in both peripheral and axial joints. Laboratory assessment of pediatric patients with suspected joint disease remains supportive with clinical exam findings being far more reliable indicators of disease. Juvenile rheumatoid (idiopathic) arthritis is the focus of this review. Other etiologies for inflammatory joint findings, such as rheumatic fever, periodic fever, and drug-induced disease, are also briefly considered. Therapies with biologic agents, anti-tumor necrosis factor constructs in particular, have significantly changed the care and early outcomes of patients with juvenile arthropathies. Reports of more aggressive use of previously available agents, such as methotrexate and injected corticosteroids, are also encouraging. Summary: Orthopedists are a critical link in the recognition of inflammatory joint diseases in children. A better understanding of the appropriate laboratory work-up, the most sensitive imaging, and the most effective available therapies will go far toward improving the quality of care and long-term outcomes for these children

4. HEILIGENHAUS A, MINGELS A, NEUDORF U, GANSER G: Juvenile Idiopathic Arthritis and Uveitis: Screening and Anti-Inflammatoty Therapy
   JUVENILE IDIOPATHISCHE ARTHRITIS UND UVEITIS: SCREENING UND ANTIENTZUNDLICHE THERAPIE. Klinische Monatsblatter fur Augenheilkunde (Germany ) 220:11 738-753, 2003
   Abstract: This article provides an overview of the rheumatic diseases in childhood, their pathogenesis, epidemiology, diagnosis and clinical course. EULAR, ACR and the current ILAR-classification are compared. The antiinflammatory medical treatment is described. The characteristics of uveitis in the various forms of arthritis are reviewed. The prognostic factors for uveitis manifestation, the clinical course, complications, the prognostic factors for visual loss, and the diverse antiinflammatory medical regimens are evaluated. The suggestions of the "uveitis in childhood study group" concerning screening and treatment of uveitis in patients with juvenile idiopathic arthritis are provided, and a nation-wide documentation of these patients is described

5. KADAYIFCILAR S, ELDEM B, TUMER B: Uveitis in childhood. J Pediatr Ophthalmol Strabismus 40:6 335-340, 2003
   Organism: Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, TurkeyFAU - Kadayifcilar, Sibel
   Abstract: PURPOSE: To review the etiologic factors and complications of uveitis in patients younger than 16 years. PATIENTS AND METHODS: Between January 1989 and December 1999 in the Department of Ophthalmology of Hacettepe University School of Medicine, 219 patients were diagnosed or observed as having pediatric uveitis. After complete ocular and physical examinations, routine and specific laboratory and radiologic investigations were performed. Medical or surgical treatment was employed when necessary. RESULTS: Of the 219 patients, 112 were girls, with a mean age of 7.4 +/- 4.2 years, and 107 were boys, with a mean age of 8.3 +/- 3.4 years. In 24.2% of the cases, no etiologic factor could be ascertained; these cases comprised the idiopathic group. Among the remaining cases, the most common etiologies were toxoplasmosis, juvenile rheumatoid arthritis (JRA), pars planitis, Behcet's disease, and Fuchs' heterochromic iridocyclitis. Anatomically, anterior uveitis was the most common form. The mean follow-up time was 37 +/- 6.2 months. Complications for which surgical treatment was employed were identified in 71 eyes (20.9%), most of which were due to JRA, pars planitis, or Behcet's disease. CONCLUSION: Uveitis in childhood may be idiopathic or most commonly due to toxoplasmosis, JRA, and pars planitis. Due to inflammation itself or to prolonged therapy especially with corticosteroids, pediatric uveitis entities (mostly JRA, pars planitis, or Behcet's disease) may result in complications necessitating a surgical approach

6. KORCZOWSKI B, KOWALCZYK JR, BIJAK M, RUSIN J: [In Process Citation]. Pol Merkuriusz Lek 15:86 155-157, 2003
   Organism: Instytut Fizjoterapii Uniwersytetu Rzeszowskiego, Oddzial Dzieciecy Szpitala Wojewodzkiego nr 2 w RzeszowieFAU - Korczowski, Bartosz
   Abstract: Procalcitonin (PCT), a 116 amino acid prohormon of calcitonin is a new acute phase reactant with features different to other markers of inflammatory response. The aim of the study was to compare PCT and C-reactive protein (CRP) levels and erythrocyte sedimentation rate (ESR) in active autoimmune diseases in children. PCT, CRP and ESR were determined in 28 children with active autoimmune process in course of various diseases: ulcerative colitis (n = 7), Lesniowski-Crohn disease (n = 2), juvenile chronic arthritis (n = 9), autoimmune haemolytic anaemia (n = 4), dermatomyositis (n = 2), sklerodermia (n = 1), systemic lupus erythematodes (n = 1), Behcet's syndrome (n = 1), primary sclerosing cholangitis (n = 1). Serum PCT levels were measured by immunoluminometric assay, CRP by turbidometric assay, ESR was counted in millimetres after one hour. PCT exceeded 0.5 ng/ml in 5 cases (range: 0.0-2.4 ng/ml; mean: 0.4 +/- 0.1 ng/ml); CRP was above 0.5 mg/dl in 24 children (range: 0.2-25.9 mg/dl; mean: 7.1 +/- 1.2 mg/dl); ESR was above 10 mm/h in all but 2 cases (range: 3-124 mm/h; mean: 57 +/- 7 mm/h). CONCLUSION: Contrary to CRP and ESR, serum PCT level in children with autoimmune diseases remains low. However in some children with highly active autoimmune process slight elevation of PCT concentration is observed without evidence of bacterial infection

7. KOTEVOGLU-SENERDEM N, TOYGAR B, TOYGAR B: Thiemann Disease. Journal of Clinical Rheumatology (United States ) 9:6 359-361, 2003
   Abstract: Thiemann disease is a rare genetic disorder that is considered to be a form of avascular necrosis of the proximal interphalangeal joints of the fingers and toes. The clinical symptoms usually appear in adolescence or puberty and may be confused with juvenile rheumatoid arthritis. The characteristic symmetrical, firm, relatively painless deformity and x-ray findings of the epiphysical irregularities should suggest the diagnosis. As rheumatologists become more familiar with the disease, it may be more frequently and promptly diagnosed

8. LEE DH, DAUD U, WIPFL J, PEPMUELLER PH, DAVITT BV, MOORE TL: The Decreasing Prevalence of Uveitis Associated with Juvenile Rheumatoid Arthritis: Do NSAIDs Play a Role? Journal of Clinical Rheumatology (United States ) 9:3 151-155, 2003
   Abstract: We studied the prevalence and characteristics of chronic uveitis in a population of children diagnosed with juvenile rheumatoid arthritis (JRA). Uveitis is one of the most important, potentially debilitating extra-articular manifestations of JRA and has been observed in as many as 20% of cases. The medical records of 230 patients diagnosed with JRA and treated at a tertiary care hospital ophthalmology clinic between 1992 and 2000 were retrospectively reviewed. Seventeen patients (7.4%) were found to have clinical features of uveitis. There was a preponderance of female patients (16/17) and pauciarticular disease (13/17). Only 12 of 17 were ANA positive. Six had uveitis at diagnosis. Patients who were receiving naproxen had less incidence of uveitis compared with those receiving other nonsteroidal antiinflammatory drugs. Despite a relatively low prevalence of uveitis, complications occurred in about 240% (4/17) of the patients, even with adequate treatment and close monitoring. The prevalence of uveitis in JRA seems to be decreasing and may be secondary to the increased use of naproxen. However, routine ophthalmologic screening should be continued in patients with JRA to avoid potential complications of chronic uveitis

9. LETHBRIDGE-CEJKU M, HELMICK CG, POPOVIC JR: Hospitalizations for arthritis and other rheumatic conditions: data from the 1997 National Hospital Discharge Survey. Med Care 41:12 1367-1373, 2003
   Organism: Division of Adult and Community Health, National Center for Chronic Disease Prevention and Health Promotion, Centers for Disease Control and Prevention, Atlanta, Georgia 30341-3724, USAFAU - Lethbridge-Cejku, Margaret
   Abstract: OBJECTIVE: To describe the impact of arthritis and other rheumatic conditions on hospitals by describing the magnitude and characteristics of these hospitalizations. METHODS: Data from the 1997 National Hospital Discharge Survey were used to examine this impact. Arthritis was defined using International Classification of Diseases, 9th Revision, Clinical Modification, codes specified by the National Arthritis Data Workgroup. Arthritis-related hospitalizations were analyzed by principal diagnosis of arthritis and by any-listed arthritis diagnosis. RESULTS: In 1997, there were an estimated 744,000 hospitalizations with a principal arthritis diagnosis (3% of hospitalizations). Compared with nonarthritis hospitalizations, persons hospitalized with a principal arthritis diagnosis were older, had fewer comorbidities, had shorter hospital stays, were more likely to undergo a procedure, and were more likely to be discharged to short- and long-term care facilities. The most common diagnoses and procedures related to osteoarthritis. This profile was consistent with a healthier-than-average hospital population electively admitted for specific procedures and subsequent rehabilitation. There were an estimated 2.5 million hospitalizations with an any-listed arthritis diagnosis (>9% of hospitalizations). Persons hospitalized with an any-listed arthritis diagnosis were older, had more comorbidities, and had longer hospital stays than those with principal arthritis or nonarthritis hospitalizations. This profile was consistent with a sicker-than-average hospital population nonelectively admitted for reasons other than their arthritis, especially cardiovascular disease. CONCLUSION: Arthritis has a sizable impact on the hospital care system. As our population ages, this impact, in both human and economic terms, is likely to increase

10. LEVY PY, COREY R, BERGER P, HABIB G, BONNET JL, LEVY S, MESSANA T, DJIANE P, FRANCES Y, BOTTA C, DEMICCO P, DUMON H, MUNDLER O, CHOMEL JJ, RAOULT D: Etiologic diagnosis of 204 pericardial effusions. Medicine (Baltimore) 82:6 385-391, 2003
    Organism: Unite des Rickettsies, Universite de la Mediterranee, Marseille, FranceFAU - Levy, Pierre-Yves
    Abstract: The etiologic evaluation of pericardial effusion is frequently unsuccessful when noninvasive methods are used. To determine the cause of the current episode, all patients with echographically identified pericardial effusion from May 1998 to December 2002 underwent noninvasive diagnostic testing of blood, throat, and stool samples. Patients with postpericardiotomy syndrome were excluded. To analyze the value of our tests, we tested randomly selected blood donors as negative controls. Among 204 included patients, 107 (52.4%) had a final etiologic diagnosis: the etiology of 52 was highly suspected at first examination and later confirmed (thyroid deficiency, 5 cases; systemic lupus erythematous, 7; rheumatoid arthritis, 7; scleroderma, 3; cancer, 25; and renal insufficiency, 5). A definite etiologic diagnosis was made in 11 patients from pericardial fluid analysis (cancer, 5 cases; tuberculosis, 3; Streptococcus pneumoniae, Citrobacter freundii, and Actinomyces, 1 case each). Among 141 patients considered to have idiopathic pericarditis, 44 (32.1%) gained an etiologic diagnosis by our systematic testing strategy. This included serologic evaluation of serum (Coxiella burnetii, 10 cases; Bartonella quintana, 1; Legionella pneumophila, 1; Mycoplasma pneumoniae, 4; influenza virus, 1), viral culture of throat swabs (enterovirus, 8 cases; and adenovirus, 1), high-level antinuclear antibodies (>1/400, 3 cases), and thyroid-stimulating hormone (15 abnormal results). Antibodies to Toxoplasma and cytomegalovirus, enterovirus recovered from rectal swabs, and low-level antinuclear antibodies were seen with equal frequency in patients and controls.Using our evaluation strategy, the number of pericardial effusions classified as idiopathic was less than in other series. Systematic testing for Q fever, Mycoplasma pneumoniae, thyroid abnormalities, and antinuclear antibodies, accompanied by viral throat cultures, frequently enabled us to diagnose diseases not initially suspected in patients with pericardial effusion

11. LINDEHAMMAR H: Hand strength in juvenile chronic arthritis: a two-year follow-up. Acta Paediatr 92:11 1291-1296, 2003
    Organism: Department of Neuroscience and Locomotion, Division of Clinical Neurophysiology, Faculty of Health Sciences, Linkoping University, Sweden hanslindehammar@lioseFAU - Lindehammar, H
    Abstract: AIM: To describe changes in muscle strength in the hands of children with juvenile chronic arthritis (JCA) and to examine the relationship between muscle strength, presence of local arthritis and disease subtype. METHODS: Twenty children (10 girls and 10 boys) with JCA aged 7 to 18 y were followed for two years. Isometric muscle strength in wrist dorsiflexors and handgrip strength were measured repeatedly. The results were compared with reference values for the same methods. Arthritis severity in the hand was scored every third month. Nerve conduction velocities were measured twice. RESULTS: Seven out of 20 patients had initially low or decreasing strength in one or both of the two tests. Five out of 20 children had reduced strength (more than two standard deviations below the mean of the reference group) in at least one test. Four children showed a significant reduction in muscle strength in at least one test during the observation time. The greatest reduction in strength was measured in four children with polyarticular disease. These children also had local arthritis in the hand. A greater proportion of children with polyarthritis had low or decreasing strength compared with children with oligoarthritis. The same was true for children with active arthritis in the hand. Nerve conduction velocities were normal in all cases and did not change. CONCLUSION: The majority of children with JCA have normal strength in the hand. Some children, especially those with polyarthritis and hand arthritis, have reduced muscle strength in the hand. Risk factors for low or decreasing strength are polyarthritis and/or active arthritis in the hand

12. MACHADO CS, ORTIZ K, MARTINS AL, MARTINS RS, MACHADO NC: [Antistreptolysin O titer profile in acute rheumatic fever diagnosis]. J Pediatr (Rio J) 77:2 105-111, 2001
    Organism: Faculdade de Medicina de Botucatu, SP, BrazilFAU - Machado, C S
    Abstract: OBJECTIVE: To determine ASO titer profile by establishing ARF differential diagnoses of other diseases with high levels of ASO antibodies. METHODS: We investigated 78 patients with ARF at onset and follow-up, 22 with isolated chorea at onset, 45 with recurrent oropharyngeal tonsillitis, and 23 with recent flare of juvenile idiopathic arthritis. We tested ASO with automated particle-enhanced immunonephelometric assay (Behring(R)-Germany). The ASO (IU/ml) titers were assessed at the following time intervals: 0-7 days, 1-2 weeks, 2-4 weeks, 1-2 months, 2-4 months, 4-6 months, 6-12 months, 1-2 years, 2-3 years, 3-4 years, and 4-5 years after onset of ARF. RESULTS: ASO titers in patients diagnosed with ARF had a significant increase up to the 2-4-month time interval (P<0.0001). Baseline levels were observed afterwards in patients under regular penicillin prophylaxis. The levels of ASO in ARF were also significantly higher than in patients with isolated chorea, recurrent oropharyngeal infections or juvenile idiopathic arthritis (P=0.0025), when age-matched samples of these groups were compared. The testacute;s sensitivity was 73.3% and the specificity was 57.6%, and it was calculated taking into account the upper limit of normality at 320 IU/ml, as well as the established diagnosis of ARF. The testacute;s specificity and positive predictive value increased with rising or higher titers, being higher with titers above 960 UI/ml. CONCLUSION: This reappraisal of ASO profile in ARF patients indicates a remarkable response during the acute phase, and that points to the extent to which ASO levels may differentiate ARF from other diseases with high levels of ASO antibodies, as coincidental but unrelated streptococcal infection or chronic arthritis flareup

13. MAGNI-MANZONI S, ROSSI F, PISTORIO A, TEMPORINI F, VIOLA S, BELUFFI G, MARTINI A, RAVELLI A: Prognostic factors for radiographic progression, radiographic damage, and disability in juvenile idiopathic arthritis. Arthritis Rheum 48:12 3509-3517, 2003
    Organism: Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S Matteo, Pavia, ItalyFAU - Magni-Manzoni, Silvia
    Abstract: OBJECTIVE: To investigate the rate of radiographic progression and identify prognostic factors of radiographic progression, radiographic damage, and physical disability in juvenile idiopathic arthritis (JIA). METHODS: Ninety-four JIA patients with a median disease duration of 1.1 years were followed up prospectively for a median of 4.5 years. Bilateral wrist radiographs were obtained at baseline, at 1 year, and at the last followup visit. Radiographic damage was assessed by the carpal length (Poznanski score), and physical disability by the Childhood Health Assessment Questionnaire (C-HAQ). Yearly radiographic progression, the Poznanski score at the final visit, and the C-HAQ score at the final visit were used as outcome measures. Baseline parameters included demographic, clinical, laboratory, and radiographic data. RESULTS: The mean +/- SD Poznanski score was -1.2 +/- 1.3 at baseline, -1.7 +/- 1.8 at the 1-year visit, and -1.9 +/- 2.2 at the final visit (P < 0.0001). Radiographic progression was greater during the first year (mean +/- SD -0.5 +/- 1.1) than between the 1-year visit and the final visit (-0.2 +/- 1.3). The mean yearly radiographic progression during the entire study period was -0.1 +/- 0.4. Logistic regression analysis revealed that radiographic progression during the first year was the only baseline parameter that was predictive of all 3 study outcomes. The final Poznanski score was also predicted by the baseline Poznanski score, whereas female sex was protective against radiographic progression. CONCLUSION: We identified the prognostic factors for poorer outcome in polyarticular-course JIA. The changes in the early Poznanski score can be used to predict long-term joint damage and physical disability

14. MANGGE H, GINDL S, KENZIAN H, SCHAUENSTEIN K: Atopic dermatitis as a side effect of anti-tumor necrosis factor-alpha therapy. J Rheumatol 30:11 2506-2507, 2003
    Organism: Clinical Institute of Medical and Chemical Laboratory Diagnosis, University of Graz, A-8036 Graz, Austria haraldmangge@uni-grazatFAU - Mangge, Harald

15. MANGGE H, HEINZL B, HANS M, EL SHABRAWI Y, SCHAUENSTEIN K: Therapeutic experience with infliximab in a patient with polyarticular juvenile idiopathic arthritis and uveitis. Rheumatol Int Berlin 23:5 258-261, 2003
    Abstract: A pediatric patient with prolonged seronegative polyarticular juvenile idiopathic arthritis (JIA) and concomitant aggressive, anterior uveitis refractory to any conventional antirheumatic therapy was treated with infliximab. Arthritis and C-reactive protein (CRP) values showed prompt positive effects but, after 6 weeks, returned gradually to initial values despite ongoing therapy. In contrast, a more sustained therapeutic effect was observed on the uveitis, with increased visual acuity and reduced inflammatory signs of the affected eye. However, this benefit was also lost at week 30, after which infliximab had to be discontinued due to side effects. To conclude, in polyarticular seronegative JIA, infliximab showed a transient beneficial effect which was more pronounced on uveitis than arthritis

16. OLSON JC: Juvenile Idiopathic Arthritis: An Update. Wisconsin Medical Journal (United States ) 102:7 45-50, 2003
    Abstract: Juvenile idiopathic arthritis (JIA) is the most common chronic arthropathy of childhood. Previous terminology identified this entity as juvenile rheumatoid arthritis. The 7 subsets of JIA identified under the new classification system are discussed, as are current treatments. A differential diagnosis of JIA is included as this condition continues to be diagnosed by exclusion. Recent studies, which discuss the outcome of adults with previous childhood arthritis, are reviewed

17. OSONE S, MORIMOTO A, TSUTSUI J, KANO G, TODO S, SUGIMOTO T: Systemic juvenile idiopathic arthritis mimics multicentric Castleman's disease. Clin Rheumatol 22:6 484-486, 2003
    Organism: Department of Pediatrics, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kamigyo-ku, 602-8566, Kyoto, Japan, shinn-o@kotokpu-macjpFAU - Osone, Shinya
    Abstract: An 11-year-old girl presented with fever and a large cervical lymphadenopathy. Indicators of inflammation were remarkable: she had extremely high levels of serum interleukin-6 (IL-6) (398 pg/ml) in addition to hypergammaglobulinemia and hypoalbuminemia. Computed tomography (CT) revealed swollen systemic lymph nodes. Two weeks after the onset of symptoms she developed polyarthralgia. Biopsy of the cervical lymph node revealed massive infiltration of plasma cells without hyaline vascular changes. She was diagnosed with systemic juvenile idiopathic arthritis (JIA). The patient's symptoms and hypercytokinemia disappeared soon after corticosteroid treatment was started. This case demonstrates that overproduction of IL-6 is common to systemic JIA and multicentric Castleman's disease

18. OZCAKAR L, OZCAKAR ZB: Exercise in juvenile idiopathic arthritis: promise or passe. Ann Rheum Dis 63:1 112, 2004

19. PALAZZI C, D'AMICO E, CACCIATORE P, PENNESE E, PETRICCA A, OLIVIERI I: Juvenile onset psoriatic arthritis in a patient with X-linked agammaglobulinemia (Bruton's disease). Scand J Rheumatol 32:5 309-311, 2003
    Organism: Division of Rheumatology, Villa Pini Clinic, Chieti, Italy kaps57@virgilioitFAU - Palazzi, C
    Abstract: We describe a 47-year-old male patient suffering from X-linked (or Bruton's) agammaglobulinemia with severe psoriatic arthritis (PsA), which started in childhood. PsA has been previously described in T-cell defective disorders, such as HIV infection, but our observation demonstrates that this rheumatic disease can also occur in subjects with B-lymphocyte cell functional impairment. Chronic inflammatory (bacterial?) involvement of the bowel could represent a pathogenetic connection between X-linked agammaglobulinemia and PsA

20. PARK Y, PARK H, YOO E, KIM D: SOX13 autoantibodies are likely to be a supplementary marker for type 1 diabetes in Korea. Ann N Y Acad Sci 1005:253-8.: 253-258, 2003
    Organism: Division of Endocrinology and Metabolism, Department of Internal Medicine and Pediatrics, Hanyang and Yonsei University, Seoul, Korea parkys@hanyangackrFAU - Park, Yongsoo
    Abstract: The SOX13, one of the family of transcription factors that play key roles in organ development, is reported to be a diabetes autoantigen, islet cell antigen 12 (ICA12). Recently, a study of antibodies to SOX13 was conducted in patients with type 1 diabetes mellitus (T1DM) indicating that these antibodies potentially identified patients without antibodies to the major T1DM-associated autoantigens, insulin, GAD, or IA-2. We know that the prevalence of islet-specific autoantibodies (GAD, IA-2) in Korean patients is much lower than that in white patients. It may be possible that other autoantibodies that could be directed to as yet unknown antigen may play a role in Korean T1DM patients. To investigate this, we measured SOX13 autoantibodies applying a radioligand binding assay using in vitro transcribed and translated antigen in 188 T1DM patients (mean duration, 4.2 years) and 64 T2DM patients and compared the results with those of 101 healthy control subjects. SOX13 autoantibodies occurred at a significantly higher frequency among T1DM patients (55/188, 29.3%) than among T2DM patients (4/64, 6.2%) or healthy adult controls (1/101, 1%). The 55 patients with positive SOX13 antibodies had significantly shorter duration of diabetes than SOX13 antibody-negative patients (3.6 +/- 2.8 vs. 4.5 +/- 3.9 years; p < 0.05). We could detect a prevalence similar to control in patients with Hashimoto's thyroiditis (4.9%, n = 101) and rheumatoid arthritis (6.7%, n = 89). As a whole, 44 of the 55 patients with SOX13 antibodies had at least one or more other autoantibodies to the major T1DM-associated autoantigens. However, SOX13 antibodies were the only antibodies detected as positive in 1 of the 11 new-onset patients. We conclude, therefore, that these antibodies are likely to be one of several epitope-spreading responses to islet- or nonislet-specific autoantigens seen in the development of T1DM, and they may be used as a supplementary marker for investigating T1DM in Korea

21. PEREIRA BA, SILVA RR: [Chronic arthritis in a child with primary agammaglobulinemia]. J Pediatr (Rio J) 75:6 467-469, 1999
    Organism: Faculdade de Medicina da Universidade Federal de Goias (UFG), Jatai, GO, BrazilFAU - Pereira, B A
    Abstract: OBJECTIVES: To report a case of a child with Bruton's disease and the unusual association with chronic aseptic arthritis resembling juvenile rheumatoid arthritis. METHODS: A 16 month-old boy was seen at the Pediatric Rheumatology unit of HC-UFG and Hospital da Crianca (Goiania- GO). The authors evaluated relevant clinical and laboratorial features, including follow-up and response to therapy. The data were then compared to previous reports published in the world literature based on a Medline search of the subject. RESULTS: Since the age of 6 months, the patient had recurrent episodes of infection that responded poorly to antibiotics. Forty days before admission, he had onset of arthritis in his left knee. The diagnosis of Bruton's disease was based on the seric levels of immunoglobulins and the response to intravenous gammaglobulin infusions. Besides improvement with this therapy, clinical characteristics and other indirect laboratorial tests highly suggested the diagnosis of chronic, aseptic arthritis. CONCLUSION: We present a case of a rare association between Bruton's disease and chronic, aseptic arthritis, very similar to juvenile rheumatoid arthritis. Early recognition of this rare aseptic articular involvement is important for early and efficient therapy with intravenous gammaglobulin infusions, avoiding unnecessary hospital admissions and inappropriate use of antibiotics

22. PETRA M, DIAZ J, MCLARDY-SMITH P, MURRAY D, GUNDLE R, ATHANASOU NA: A correlative study of clinical and histological findings of revision hip arthroplasty for rheumatoid arthritis and inflammatory joint disease. Scand J Rheumatol 32:5 281-286, 2003
    Organism: Department of Surgery, University of Oxford, Nuffield Orthopaedic Centre, Oxford UKFAU - Petra, M
    Abstract: OBJECTIVE: Primary hip arthroplasty in rheumatoid arthritis (RA) and other forms of inflammatory joint disease (IJD) is generally thought to be associated with a less favourable outcome in terms of implant survival and other complications. Whether the duration of implant survival correlates with the degree of rheumatoid-like inflammatory changes in periprosthetic tissues is uncertain. METHODS: Histopathological changes in periprosthetic tissues obtained following revision surgery of 34 total hip replacements on 27 patients with IJD (RA 18 cases: ankylosing spondylitis three cases; juvenile chronic arthritis six cases) were examined. RESULTS: A heavy diffuse lymphocyte and plasma cell infiltrate +/- lymphoid aggregates was noted in 29% of cases in whom the mean implant survival was 5.6 years (range: 2-8 years). Where little or no lymphocytic infiltrate was noted in periprosthetic tissues, the mean implant survival was 8.6 years (range: 1-17 years). Revision arthroplasty was not undertaken for early or late infection of a primary hip replacement. CONCLUSIONS: Our findings indicate that implant survival is less in those cases where there is a heavy lymphocyte and plasma-cell infiltrate in periprosthetic tissues. These findings suggest that the presence of a heavy chronic inflammatory-cell infiltrate in periprosthetic tissues is likely to be a contributory factor in causing early implant failure in RA

23. RAMOS VA, RAMOS PA, DOMINGUEZ MC: [Role of oxidative stress in the maintenance of inflamation in patients with juvenile rheumatoid arthritis]. J Pediatr (Rio J) 76:2 125-132, 2000
    Organism: Instituto Candida Vargas, Joao Pessoa, PB, BrazilFAU - Ramos, V A
    Abstract: OBJECTIVE: To determine the level of cellular oxidative stress blood markers and the enzymatic system of antioxidant defense establishing the oxidative profile in patients with Juvenile Rheumatoid Arthritis. METHODS: Case-control study that included 64 patients (46 of female sex) with Juvenile Rheumatoid Arthritis (JRA) following clinical control in the Pediatric Rheumatology Service of the Vall dacute;Hebron Hospital, Barcelona, Spain. The patients were separated in three subtypes based on the pattern of onset within the first six months of disease: polyarticular, pauciarticular and systemic. The control group included 60 patients (38 of female sex) following clinical control to diseases of non inflammatory nature, in the same hospital. The plasmatic levels of malondialdehyde (MDA), lipoperoxide (LPO), hydroperoxide (HPX), carbonile groups (CG) of proteins and gluthathione and the enzymatic activities of Superoxide dismutase (SOD), gluthathione peroxidase (GSH-Px) and gluthathione reductase were determined. RESULTS: The group of patients with JRA presented high concentrations of lipid peroxidation products, evaluated by determining the plasmatic levels of MDA, LPO, and HPX; oxidative damage of the circulate protein, determined by CG contents of plasma proteins; elevation of enzymatic activity of SOD and GSH-Red; decrease of GSH-Px activity and GSH levels. CONCLUSIONS: Our results show the presence of molecular damage determined by oxygen free radicals in the JRA patients. The SOD activity and the changes of gluthathione redox enzymatic cycle confirm the decrease of capacity of cellular defense system against the induced toxicity of oxidative stress in these patients

24. ROBERTO AM, TERRERI MT, LEN C, MUCCIOLI C, HILARIO MO: [Uveitis in juvenile idiopathic arthritis]. J Pediatr (Rio J) 78:1 62-66, 2002
    Organism: Universidade Federal de Sao Paulo (UNIFESP-EPM), SP, BrazilFAU - Roberto, Adriana M
    Abstract: OBJECTIVE: To evaluate the frequency of chronic anterior uveitis in patients with juvenile idiopathic arthritis and its association with the presence of antinuclear antibodies. PATIENTS AND METHODS: We retrospectively studied 72 patients with juvenile idiopathic arthritis. All of them were submitted to slit-lamp examination of the anterior chamber at diagnosis. Both antinuclear antibodies and rheumatoid factor were determined. Patients with positive results for antinuclear antibodies were evaluated every three months and those with negative results were assessed every six months. RESULTS: Forty patients were male (55.5%) and 36 were Caucasoid (50%). The mean age at the onset of juvenile idiopathic arthritis was 6.4 years (range = 1 to 14 years) and the mean age at the beginning of the study was 10.4 years (1 to 19 years). According to the type of disease at onset, 32 were pauciarticular (44.4%) (17 boys and 15 girls), 30 were polyarticular (41.6%) (17 boys and 13 girls) and 10 were systemic (14%) (6 boys and 4 girls). We observed chronic anterior uveitis in five patients (6.5%) (mean age = 11.4 years). Among them, four (80%) had pauciarticular juvenile idiopathic arthritis at disease onset (three girls with type I juvenile idiopathic arthritis and positive antinuclear antibodies and one boy with type I juvenile idiopathic arthritis and negative antinuclear antibodies) and one girl with polyarticular juvenile idiopathic arthritis (negative antinuclear antibodies and rheumatoid factor). In this group, the mean age at the onset of juvenile idiopathic arthritis was 5.1 years and the mean age of uveitis onset was 9 years. Antinuclear antibodies were positive in 3/5 patients (60%) with uveitis. Antinuclear antibodies were positive in 12% of the patients without uveitis (n = 67). Among the patients with uveitis, three had only one flare and the other two had four flares with cataract. The frequency of antinuclear antibodies was statistically higher in the patients with uveitis (P< 0.05). CONCLUSION: Although the incidence of uveitis in our study was lower than that reported in the literature, the frequency of uveitis was higher in females, in those with pauciarticular juvenile idiopathic arthritis and in patients with positive antinuclear antibodies

25. ROSENBAUM JT, PLANCK SR, DAVEY M, IWANAGA Y, KURZ DE, MARTIN TM: With a mere nod, uveitis enters a new era. Am J Ophthalmol 136:4 729-732, 2003
    Abstract: PURPOSE: To advance the knowledge of the ophthalmologist with regard to new developments in the genetics and pathologic mechanisms of uveitis. DESIGN: A review of recently published literature exploring the relationship between the nucleotide oligomerization domain (NOD2) gene and uveitis. RESULTS: Mutations in the nucleotide-binding region of NOD2 were found to be responsible for familial juvenile systemic granulomatosis (Blau syndrome or Jabs disease), a rare form of uveitis, arthritis, and dermatitis. The NOD2 gene is thought to be involved in the innate immune response to pathogens. Currently, the pathologic mechanisms behind Blau syndrome in familial juvenile systemic granulomatosis are unknown, but the interactions of NOD2 with caspases, nuclear factor kappaB, and other pathways are slowly being revealed. CONCLUSIONS: A single amino acid change in NOD2 can lead to a chronic granulomatous uveitis. By studying NOD2 and the proteins that interact with NOD2, we should gain a better understanding of the pathogenic mechanisms of uveitis and identify novel ways to halt its destructive consequences

26. SAVOLAINEN E, KAIPIAINEN-SEPPANEN O, KROGER L, LUOSUJARVI R: Total incidence and distribution of inflammatory joint diseases in a defined population: results from the Kuopio 2000 arthritis survey. J Rheumatol 30:11 2460-2468, 2003
    Organism: Kuopio Municipal Hospital, 70211 Kuopio, FinlandFAU - Savolainen, Elina
    Abstract: OBJECTIVE: To study the incidence of inflammatory joint diseases in a defined population in Finland. METHODS: We collected data for the year 2000 on a population of 87,000 inhabitants of Kuopio, Finland, of whom 20% were < 16 years of age. Information about the study was given through a local newspaper, and subjects attended one health center and 2 local hospitals for study. Inclusion criteria were that subjects have at least one peripheral joint with synovitis or signs of inflammation in sacroiliac, glenohumeral, or hip joints on the first visit. Incidence rates were calculated according to the diagnosis on the first visit, except for children, for whom diagnoses were established after 3 months' followup. RESULTS: A total of 188 adult incident cases (138 women, 50 men) and 11 children (8 girls, 3 boys) satisfied the inclusion criteria. The incidence of all arthritides was 230/100,000 (95% confidence interval 198.9-263.9) for the whole population; 271/100,000 (95% CI 233.7-312.7) for adults and 64/100,000 (95% CI 31.7-113.8) for children. Among adults the annual incidence of rheumatoid arthritis (RA), ankylosing spondylitis (AS), psoriatic arthritis (PsA), reactive arthritis (ReA), other spondyloarthropathies (SpA), connective tissue disease (CTD), crystalline arthritis, viral arthritis, and undifferentiated arthritis were 36, 7, 23, 10, 13, 9, 19, 7, and 149/100,000, respectively. The mean age at diagnosis was 49.4 +/- 16.3 years for all cases of arthritis among adults, about the same for both women and men. The mean age at diagnosis was 59.7 years in RA, 31.5 years in AS, 48.7 years in PsA, 38.0 years in ReA, 36.5 years in other SpA, 36.1 years in CTD, 65.0 years in crystalline arthritis, 53.3 years in viral arthritis, and 48.3 years in undifferentiated arthritis. Four of 11 children had juvenile idiopathic arthritis (JIA). The incidence of JIA was 23/100,000 in the population < 16 years of age. Of the remaining cases, 3 children had antibodies against Sindbis (Pogosta) virus and 4 had a transient monoarthritis. CONCLUSION: The overall incidence of arthritides among adults was slightly higher than previously reported from Finland. The incidence rates in the child population are in agreement with previous figures. These data are useful in planning the provision of health care

27. SIMON D, LUCIDARME N, PRIEUR AM, RUIZ JC, CZERNICHOW P: Effects on growth and body composition of growth hormone treatment in children with juvenile idiopathic arthritis requiring steroid therapy. J Rheumatol 30:11 2492-2499, 2003
    Organism: Department of Endocrinology and Diabetology, Robert Debre Teaching Hospital, 48 Boulevard Serurier, 75019 Paris, France dominiquesimon@rdbap-hop-parisfrFAU - Simon, Dominique
    Abstract: OBJECTIVE: Decreased growth velocity and abnormal body composition including severe osteoporosis are common in glucocorticoid-treated patients with juvenile idiopathic arthritis (JIA). We evaluated the effects of recombinant human growth hormone (GH) given for 3 years on growth velocity, height standard deviation score (SDS), and body composition, together with potential adverse effects on glucose tolerance. METHODS: Thirteen patients received GH (0.46 mg/kg/week) for 3 years. Body composition was assessed by dual-energy x-ray absorptiometry and glucose tolerance by annual oral glucose tolerance tests. RESULTS: Median growth velocity increased from 2.1 to 6.0 cm/year (p = 0.002) in the first year and remained higher than baseline in the second year of treatment. Height SDS did not change significantly (-4.6 SDS at baseline vs -4.3 SDS at study completion), but the growth response varied markedly across patients. Compared with baseline, lean mass increased by 33%, fat mass remained stable, and lumbar bone mineral density increased by 36.6%. Transient glucose intolerance developed in 6 patients, but glycosylated hemoglobin concentrations did not change significantly and diabetes mellitus did not occur. CONCLUSION: Treatment with GH restored linear growth without inducing catch-up growth, significantly improved body composition, and prevented further bone loss. Prolonged followup is needed to assess the benefits of GH and longterm consequences of hyperinsulinism

28. SZTAJNBOK FR, SERRA CR, RODRIGUES MC, MENDOZA E: [Rheumatic diseases in adolescence]. J Pediatr (Rio J) 77 Suppl 2:S234-44.: S234-S244, 2001
    Organism: Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, BrazilFAU - Sztajnbok, F R
    Abstract: OBJECTIVE: To present an updated review concerning the most prevalent diseases with musculoskeletal signs and symptoms that make adolescents seek medical care, giving special emphasis to rheumatic diseases. Our aim is to offer physicians and health care providers the possibility of distinct differential diagnoses, thus allowing them to establish a therapeutic approach and, if necessary, refer the patient to a specialist METHODS: Review of literature using Medline database, data obtained at our department, and the authors personal experience. RESULTS: Musculoskeletal pain is characteristic of several diseases and usually urges adolescents to seek medical care. Rheumatic diseases, especially rheumatic fever, account for nearly fifty percent of the cases. In adolescents, it is also important that the aspects regarding the diagnosis and treatment of idiopathic juvenile arthritis, arthritis associated with enthesitis, systemic lupus erythematosus, and vasculitis be considered. Fibromyalgia, reflex sympathetic dystrophy, growing pains, hypermobility syndrome, and psychogenic rheumatism are noninflammatory conditions that frequently mimic rheumatic diseases. CONCLUSIONS: Inflammatory and noninflammatory conditions, and diseases of different etiology (infectious, neoplastic, and orthopedic) are frequently associated with musculoskeletal pain. It is important that health professionals diagnose these diseases as early as possible so that prompt action can be taken and prognosis can be improved

29. TWILT M, VAN DEN BERG MARIETTA SWART, VAN MEURS JC, CATE R, SUIJLEKOM-SMIT LISETTE WA: Persisting uveitis antedating psoriasis in two boys. European Journal Of Pediatrics 162:9 607-609, 2003
    Abstract: Two young boys had a severe uveitis which was difficult to treat. They both developed psoriasis located in the pubic area almost 1 year and several years later respectively. One boy also developed arthritis of an intermittent character. Both boys were ANA and HLA-B27 negative. Conclusion: in children, the possible association between uveitis and psoriasis with or without arthritis has to be kept in mind

30. UNAL O, OZCAKAR L, CETIN A, KAYMAK B: Severe bilateral carpal tunnel syndrome in juvenile chronic arthritis. Pediatr Neurol 29:4 345-348, 2003
    Organism: Hacettepe University Medical School, Department of Physical Medicine and Rehabilitation, Ankara, TurkeyFAU - Unal, Oya
    Abstract: Carpal tunnel syndrome, although rare, is known to occur in children mainly because of genetic or metabolic disorders. The clinical findings are variable and include symptoms of burning pain, tingling, numbness, and weakness or atrophy in the hands of the patients. It is usually diagnosed by demonstration of prolonged distal latency times during the electrodiagnostic studies. Reported here is a patient with juvenile chronic arthritis and a diagnosis of severe bilateral carpal tunnel syndrome, the first patient reported in the literature, to the best of the authors' knowledge

31. VARBANOVA B, NIKOLOV K, BALEV S: Clinical associations of antibodies to individual histones in juvenile chronic arthritis. Rheumatology (Bulgaria ) 11:1-2 53-58, 2003
    Abstract: This study was aimed to investigate the prevalence and the clinical associations of antibodies to individual histones in juvenile chronic arthritis (JCA). We studied 50 children with pauciarticular and polyarticular JCA and control groups for IgG- and IgM-antibodies to histone 1, histone 2 and histone 3 by ELISA. Our results demonstrated a prevalence of those antibodies in 1/4-1/ 3 of the children with JCA. We found a significant clinical association with uveitis of the IgM-antibodies to histone 1 and histone 3 and IgG-antibodies to histone 2. The IgM-antibodies to histone 2 correlated with polyarthritis and active disease

32. WELBURY RR, THOMASON JM, FITZGERALD JL, STEEN IN, MARSHALL NJ, FOSTER HE: Increased prevalence of dental caries and poor oral hygiene in juvenile idiopathic arthritis. Rheumatology (United Kingdom ) 42:12 1445-1451, 2003
    Abstract: Objectives. Recent decades have seen a trend to treat juvenile idiopathic arthritis (JIA) with increasing immunosuppression to improve the long-term outcome. Poor oral hygiene and dental decay cause significant morbidity, and patients with chronic disease (who may be further immunocompromised by treatment) are at greater risk. This study investigated patients with JIA using standard measures of oral health. Methods. One hundred and forty-nine patients with JIA were included. The children were attending a regional paediatric rheumatology service and the adults were attending an adult rheumatology clinic. Random age- and sex-matched healthy controls were recruited from a dental teaching hospital. The structured dental examination included standard epidemiological indices of oral hygiene (gingival index, plaque index, oral cleanliness index) and dental decay [DMFT (decayed, missing or filled teeth) index]. Results. JIA patients, at all ages, had increased levels of dental decay and poor oral hygiene. This increased level of decay was statistically significant in the patients aged 0-11 yr. Significant levels of untreated caries and increased levels of missing teeth were found in JIA, suggesting that patients with JIA had less restorative dental treatment, with tooth extraction often the chosen option for the treatment of dental decay. Conclusions. This is the largest study of oral health in JIA and is cross-sectional with non-diseased controls. It shows significantly increased levels of poor oral hygiene and dental decay in patients with JIA. The high levels of untreated dental decay suggest barriers to dental care. These results emphasize the role of regular dental care in the multidisciplinary management of JIA

33. WENDLING D, BERTRAND MA: Hemarthrosis in acquired hemophilia. Two case-reports. Joint Bone Spine 70:6 521-523, 2003
    Organism: Rheumatology Department, Jean Minjoz Teaching Hospital, 25030 Besancon, FranceFAU - Wendling, Daniel
    Abstract: The many causes of hemarthrosis include acquired hemophilia due to production of autoantibodies to factor VIII. We report two very different cases. Case 1: This woman experienced onset of juvenile idiopathic arthritis at 8 years of age. Her first child was born when she was 28-years-old. Three months after delivery, vaginal bleeding and recurrent hemarthrosis led to a diagnosis of acquired hemophilia (isolated APTT prolongation, 1% VIIIc activity, and 58 U of anti-factor VIII antibody). Treatment included glucocorticoid therapy, prothrombin complex, and intravenous immunoglobulins. She achieved a full recovery within a year. Case 2: In this 84-year-old woman, spontaneous recurrent hemarthrosis with hematomas revealed idiopathic acquired hemophilia. Treatment included prothrombin complex, factor VIII concentrates, and intravenous immunoglobulins, followed by cyclophosphamide and glucocorticoid therapy. Recovery was complete within a year. The diagnosis, etiology, prognosis, and treatment of acquired hemophilia are discussed. CONCLUSION: Although rare, acquired hemophilia should be considered among the causes of hemarthrosis, particularly as a favorable outcome can be expected with early diagnosis and appropriate treatment