Long-term outcome of children with pediatric-onset cutaneous and visceral polyarteritis nodosa.

OBJECTIVE: To assess the prognostic impact of clinical presentation in children with polyarteritis nodosa (PAN). METHODS: Children diagnosed between 1986 and 2006 in a tertiary care pediatric rheumatology center were classified as "cutaneous PAN" (group 1), "cutaneous PAN with significant extra-cutaneous features" (group 2) or "visceral childhood PAN" (group 3). OUTCOME MEASURES: (1) clinical remission off-therapy at last follow-up, (2) requirement and length of glucocorticoid therapy, (3) presence of disease-related sequelae. RESULTS: Twenty-nine children were included. Sixteen met the Ankara criteria for PAN. Nine patients were qualified as group 1, 11 as group 2, and 9 as group 3. At last follow-up, 15 children were in clinical remission off-therapy: 4 from group 1 (44%), 4 from group 2 (36%) and 7 from group 3 (78%). Glucocorticoid therapy was required for 8 (89%), 7 (64%) and 7 (78%) patients from groups 1, 2 and 3, respectively. Seven children did not require any glucocorticoid therapy. Time-dependent probability of achieving glucocorticoid-free clinical remission was similar between the three groups. Three patients (one from each group) had digital ischemia leading to amputation. There were no significant between-group differences in outcome based on the three outcome measures addressed. CONCLUSION: Outcome was not strikingly predictable from initial presentation in children with PAN. The organ distribution-based distinction between cutaneous and visceral PAN had little prognostic power in this series.

Effect of biologic treatments on growth in children with juvenile idiopathic arthritis.

OBJECTIVE: Growth retardation is a frequent complication of severe juvenile idiopathic arthritis (JIA). Biologic treatments may improve growth velocity by controlling systemic inflammation and reducing corticosteroids. Our goals were to compare growth velocity before and after the onset of biologic therapy and to determine whether the JIA subtype, the use of steroids, the requirement of one or several biologic agents, or the disease activity influenced growth velocity. METHODS: We retrospectively analyzed the growth of children with JIA who never received growth hormone treatment, who started biologic treatment before puberty, and who were followed for at least 6 months afterward. RESULTS: We included 100 children (33 boys). Median patient age was 7.1 years (range: 1.6-15.7) at the onset of biologic treatment and 11.0 years (range: 2.3-19.5) at the latest followup. Forty-six patients had received corticosteroid and 34 had received more than 1 biologic agent. Patient median height expressed as SD score (SDS) was 0.31 (range: -2.47 to 5.46) at disease onset, -0.24 (-3.63 to 2.90) at biologic therapy onset (p < 0.0001), and -0.15 (-4.95 to 3.52) at the latest followup (p = 0.171 compared to biologic treatment onset). Patients who required several biologics and systemic patients had a significantly lower growth velocity after the onset of biologic treatment. At the latest followup, 18% of our study group had low growth velocities and 19% were below -2SD or shorter than genetically programmed. CONCLUSION: In a subset of patients, particularly systemic JIA patients and patients who required more than 1 biologic, biologic therapy may be insufficient to restore normal growth velocity.

ANCA-associated glomerulonephritis in systemic-onset juvenile idiopathic arthritis.

Systemic-onset juvenile idiopathic arthritis is an inflammatory disease of unknown cause and is not commonly associated with kidney involvement. We describe 3 patients with systemic-onset juvenile idiopathic arthritis with high disease activity who developed antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis 1-6 years after the onset of systemic-onset juvenile idiopathic arthritis. Renal and systemic-onset juvenile idiopathic arthritis remission occurred in one patient under anti-interleukin 1 (anti-IL-1) treatment associated with immunosuppressive drugs. The other 2 patients developed end-stage renal disease, and one of those patients died. This report suggests that the diagnosis of ANCA-associated glomerulonephritis must be considered in patients with systemic-onset juvenile idiopathic arthritis with persistently active systemic disease who present with proteinuria. Furthermore, use of an anti-IL-1 agent might be an effective therapeutic option.

Cataract surgery with primary intraocular lens implantation in children with uveitis: long-term outcomes.

PURPOSE: To report long-term outcomes of cataract surgery with primary posterior chamber intraocular lens (IOL) implantation in children with chronic uveitis. SETTING: Department of Ophthalmology, Pitié-Salpêtrière Hospital, Paris, France. DESIGN: Case series. METHODS: This case series comprised patients younger than 16 years with chronic uveitis who underwent phacoemulsification with primary implantation of a heparin surface-modified poly(methyl methacrylate) posterior chamber IOL in the capsular bag. The intraocular inflammation was fully controlled for at least 3 consecutive months before surgery in all cases. The main outcome measures were final corrected distance visual acuity (CDVA), postoperative inflammation, complications, and level of immunosuppressive treatment. RESULTS: Twenty-two eyes of 16 children (7 girls, 9 boys; median age at surgery 9.5 years old) were included. Underlying uveitic entities were juvenile idiopathic arthritis in 9 patients; idiopathic uveitis in 4; and Behçet disease, sarcoidosis, and varicella zoster-associated uveitis in 1 patient each. The final CDVA was 0.3 logMAR or better in all cases. Postoperative complications included posterior capsule opacification requiring laser capsulotomy in 2 eyes, glaucoma in 4 eyes, and cystoid macular edema/macular dysfunction in 3 eyes. The mean dose of oral prednisone was 29.5 mg/day preoperatively and 8.13 mg/day at the last follow-up. The median follow-up was 6 years (range 5 to 19 years). CONCLUSION: The results indicate that uveitis is not a formal contraindication to primary IOL implantation in the management of pediatric cataract surgery in cases with full control of intraocular inflammation. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

Mevalonate kinase deficiency: a survey of 50 patients.

OBJECTIVE: The goal of this study was to describe the spectrum of clinical signs of mevalonate kinase deficiency (MKD). METHODS: This was a retrospective French and Belgian study of patients identified on the basis of MKD gene mutations. RESULTS: Fifty patients from 38 different families were identified, including 1 asymptomatic patient. Symptoms began during the first 6 months of life in 30 patients (60%) and before the age of 5 years in 46 patients (92%). Symptoms consisted of febrile diarrhea and/or rash in 23 of 35 patients (66%). Febrile attacks were mostly associated with lymphadenopathy (71%), diarrhea (69%), joint pain (67%), skin lesions (67%), abdominal pain (63%), and splenomegaly (63%). In addition to febrile attacks, 27 patients presented with inflammatory bowel disease, erosive polyarthritis, Sjögren syndrome, and other chronic neurologic, renal, pulmonary, endocrine, cutaneous, hematologic, or ocular symptoms. Recurrent and/or severe infections were observed in 13 patients, hypogammaglobulinemia in 3 patients, and renal angiomyolipoma in 3 patients. Twenty-nine genomic mutations were identified; the p.Val377Ile mutation was the most frequently found (29 of 38 families). Three patients died of causes related to MKD. The disease remained highly active in 17 of the 31 surviving symptomatic patients followed up for >5 years, whereas disease activity decreased over time in the other 14 patients. Interleukin 1 antagonists were the most effective biological agents tested, leading to complete or partial remission in 9 of 11 patients. CONCLUSION: MKD is not only an autoinflammatory syndrome but also a multisystemic inflammatory disorder, a possible immunodeficiency disorder, and a condition that predisposes patients to the development of renal angiomyolipoma.

Ocular modifications in a young girl with cryopyrin-associated periodic syndromes responding to interleukin-1 receptor antagonist anakinra.

An 8-year-old patient with genetically confirmed chronic infantile neurological cutaneous and articular syndrome was treated with interleukin-1 receptor antagonist, anakinra. She initially presented with recurrent episodes of fever, rash, chronic fatigue, frequent headaches, ocular involvement (corneal infiltrate and papillary edema), and permanent increased biologic inflammatory markers. Following treatment with anakinra, all symptoms and inflammation resolved. Ophthalmologic signs normalized. This ophthalmologic description (optic nerve and cornea) has never been illustrated, even if ocular affections are classic in the cryopyrin-associated periodic syndromes.

Sicca syndrome and salivary gland infiltration in children with autoimmune disorders: when can we diagnose Sjögren syndrome?

OBJECTIVES: To analyse the initial presentation and outcome of children with a diagnosis of childhood-onset Sjögren's syndrome (SS) in a paediatric referral care center. To study whether the diagnosis was made in accordance with the most recent criteria of paediatric SS and to compare our patients to those reported in the literature. METHODS: We retrospectively analysed the clinical, histological and laboratory features of patients seen over a period of 15 years and diagnosed with SS before the age of 16. RESULTS: Eight patients had a diagnosis of SS in childhood and were followed for up to 14 years. Diagnosis of SS was based on histological evidence of salivary gland involvement in all patients with or without presence of specific autoantibodies (anti-SSA and -SSB). Sicca syndrome as a presenting symptom occurred in only 2/8 of children, recurrent parotid swelling in 3/8; whereas anti-SSA/SSB antibodies and typical salivary-gland histology were found in 6/8 patients. Five children fulfilled the proposed paediatric criteria for SS. Three patients did not fulfill the paediatric criteria but disclosed typical histology findings. Two patients developed overlapping lupus nephritis or autoimmune hepatitis years following diagnosis of SS. CONCLUSIONS: Childhood-onset SS is an heterogeneous disease in its presentation and outcome. The diagnosis may be discussed in some patients who do not fulfill the proposed diagnosis criteria, even though they disclose sicca syndrome and typical immunologic and histological findings. Some patients with typical SS may develop overlapping lupus disease over time.

Long-term efficacy of the interleukin-1 receptor antagonist anakinra in ten patients with neonatal-onset multisystem inflammatory disease/chronic infantile neurologic, cutaneous, articular syndrome.

OBJECTIVE: Cryopyrin-associated periodic syndromes (CAPS) are a group of rare autoinflammatory diseases. Neonatal-onset multisystem inflammatory disease (NOMID)/chronic infantile neurologic, cutaneous, articular syndrome (CINCA syndrome) is the most severe phenotype, with fever, rash, articular manifestations, and neurologic and neurosensory involvement. CAPS are caused by mutations in CIAS1, the gene encoding NLRP3, which plays a critical role in interleukin-1 (IL-1) processing. Anakinra, an IL-1 receptor antagonist, has been shown to be an effective treatment; however, data on long-term efficacy and safety have been sparse. This study was undertaken to assess the long-term efficacy and safety of anakinra treatment in patients with NOMID/CINCA syndrome. METHODS: We retrospectively analyzed the medical records of NOMID/CINCA syndrome patients referred to 2 centers, who had started anakinra treatment before June 2007. RESULTS: There were 10 patients with NOMID/CINCA syndrome who had been treated with anakinra. The patients' ages at the time anakinra treatment was initiated ranged from 3 months to 20 years. They had been followed up for 26-42 months. Sustained efficacy in the treatment of systemic inflammation and, in some cases, neurologic involvement and growth parameters, was achieved. The dosage of anakinra required for efficacy ranged from 1 to 3 mg/kg/day in the 8 oldest patients and from 6 to 10 mg/kg/day in the 2 youngest. Residual central nervous system inflammation and deafness persisted in some patients, especially if there had been a delay in diagnosis and treatment. Secondary amyloidosis persisted in cases in which it was present at treatment initiation, but no new lesions developed. No effect on overgrowth arthropathy was observed. Adverse events consisted of mild injection-site reactions. CONCLUSION: The present results indicate that anakinra treatment is effective over the long term in NOMID/CINCA syndrome. However, treatment has to be initiated before irreversible lesions develop, and, particularly in very young patients, dosage adjustment is required.

Ocular threat in juvenile idiopathic arthritis.

OBJECTIVES: Uveitis is the most severe complication of juvenile idiopathic arthritis (JIA). The diagnosis may be delayed, as visual symptoms are usually absent, which provides time for insidious complications. The course is chronic and long-term treatment is therefore required. METHODS: We retrospectively reviewed the medical records of 75 children with uveitis who accounted for 10.5% of all patients with JIA seen at our pediatric rheumatology outpatient clinic between July 1997 and July 2007. RESULTS: Uveitis occurred chiefly in patients with pauciarticular JIA. At last follow-up, in 69 patients in whom the files could be satisfactorily reviewed, only the anterior compartment was involved in 59.4% and both the anterior and the posterior compartments in the remainder. Mean age at the uveitis diagnosis was 4.5 years. In 9 (13.0%) patients, uveitis antedated joint manifestations; in 17 (24.7%) both were diagnosed simultaneously; and in 43 (62.3%) arthritis antedated uveitis. In 42 (61%) patients, complications occurred (synechiae, papillary block, cataract, hyalitis, papilledema, glaucoma, macular edema, elevated intraocular pressure, vision loss, and hypotonia). Topical medications were used in all patients for at least 3 months. Severe ocular involvement required systemic glucocorticoid therapy in 29 (42.0%) patients. Among immunomodulating agents, methotrexate and cyclosporine were used in 41 patients and TNFalpha antagonists in 15 patients. Surgery was performed in 21 (30.4%) patients. Uveitis completely resolved in 12 (17.4%) patients, a relapsing course occurred in 14 (20.3%), and became chronic with relapses as soon as the topical treatment was decreased in 23 (33.3%). A severe course was observed in 21 (30.4%) patients of whom 3 became blind and 4 lost vision in one eye. CONCLUSION: Uveitis is a severe complication of JIA. Patients with JIA should receive routine ophthalmological follow-up at regular intervals, even is their joint disease is quiescent.

Cryopyrinopathies: update on pathogenesis and treatment.

Cryopyrinopathies are a group of rare autoinflammatory diseases that includes familial cold autoinflammatory syndrome, Muckle-Wells syndrome and chronic infantile neurologic cutaneous articular syndrome (also termed neonatal-onset multisystemic inflammatory disease). These syndromes were initially considered to be distinct disease entities despite some clinical similarities; however, mutations of the same gene have since been found in all three cryopyrinopathies. These diseases, therefore, are not separate but represent a continuum of subphenotypes. The gene in question, CIAS1 (now renamed NLRP3) encodes NALP3 (also known as cryopyrin). NALP3 is an important mediator of inflammation and interleukin 1beta processing. New therapies based on biologic agents that specifically target interleukin 1beta are currently being developed. These new agents have provided very encouraging results for patients with these long-lasting inflammatory conditions--which used to be considered refractory to treatment. The development of therapeutic options for these cryopyrinopathies illustrates effective translation of basic science to clinical practice and the convergence of human genetics and targeted therapies.

A randomized, placebo-controlled trial of infliximab plus methotrexate for the treatment of polyarticular-course juvenile rheumatoid arthritis.

OBJECTIVE: To evaluate the safety and efficacy of infliximab in the treatment of juvenile rheumatoid arthritis (JRA). METHODS: This was an international, multicenter, randomized, placebo-controlled, double-blind study. One hundred twenty-two children with persistent polyarticular JRA despite prior methotrexate (MTX) therapy were randomized to receive infliximab or placebo for 14 weeks, after which all children received infliximab through week 44. Patients received MTX plus infliximab 3 mg/kg through week 44, or MTX plus placebo for 14 weeks followed by MTX plus infliximab 6 mg/kg through week 44. RESULTS: Although a higher proportion of patients in the 3 mg/kg infliximab group than in the placebo group had achieved responses according to the American College of Rheumatology (ACR) Pediatric 30 (Pedi 30) criteria for improvement at week 14 (63.8% and 49.2%, respectively), the between-group difference in this primary efficacy end point was not statistically significant (P = 0.12). By week 16, after the crossover from placebo to infliximab 6 mg/kg when all patients were receiving infliximab, an ACR Pedi 30 response was achieved in 73.2% of all patients. By week 52, ACR Pedi 50 and ACR Pedi 70 responses had been reached in 69.6% and 51.8%, respectively, of patients. Infliximab was generally well tolerated, but the safety profile of infliximab 3 mg/kg appeared less favorable than that of infliximab 6 mg/kg, with more frequent occurrences of serious adverse events, infusion reactions, antibodies to infliximab, and newly induced antinuclear antibodies and antibodies to double-stranded DNA observed with the 3 mg/kg dose. CONCLUSION: While infliximab at 3 mg/kg and 6 mg/kg showed durable efficacy at 1 year, achievement of the primary efficacy end point at 3 months did not differ significantly between infliximab-treated and placebo-treated patients. Safety data indicated that the 6-mg/kg dose may provide a more favorable risk/benefit profile. These results warrant further investigation in children with JRA.

[Juvenile idiopathic arthritis. II. Treatment and prognosis]. / Arthrites juvéniles idiopathiques. II. Traitement et pronostic.

Immunosuppressants, including methotrexate and more recently anti-TNFalpha, have modified the prognosis of the most severe forms juvenile idiopathic arthritis. However, the therapeutic management is still challenging and adjuvant therapies, including physiotherapy, surgery, or even bisphosphonates and growth hormone, are still widely used to limit the negative impact of an extended general corticotherapy.

[Juvenile idiopathic arthritis. (I) Clinical aspects]. / Arthrites juvéniles idiopathiques. I. Les différentes formes cliniques.

Juvenile idiopathic arthritis (JIA), formerly know as juvenile chronic arthritis, is a broad term encompassing several disorders starting before the age of 16. It is characterized by arthritis lasting more than 6 weeks, of unknown etiology, usually persisting for six month initially. Approximately 1 in 5 000 children are affected in France. Of the various distinguishable clinical forms, oligoarticular JIA is the most frequent one. It is characterized by an involvement of up to 4 joints during the first 6 months and is mostly observed in females. The prognosis may be further complicated by the presence of uveitis, associated with an insidious progression. In systemic JIA (also called Still's disease) as well as in some polyarticular forms, with or without rheumatoid factor, inflammation may continue in adulthood. Severe polyarticular involvement or hip involvement may be associated with a poor functional prognosis.

Allogeneic bone marrow transplantation in mevalonic aciduria.

Mevalonic aciduria is a rare, inborn error of isoprene biosynthesis characterized by severe, periodic attacks of fever and inflammation, developmental delay, ataxia, and dysmorphic features. This autosomal recessive disease is caused by a mutation in the mevalonate kinase gene that severely reduces mevalonate kinase activity. A 3-year-old boy with mevalonic aciduria whose condition had failed to improve with antiinflammatory treatment underwent allogeneic bone marrow transplantation from an HLA-identical sister who was a heterozygous carrier of the mutant gene. We observed sustained remission of febrile attacks and inflammation during a 15-month follow-up period.

Early recombinant human growth hormone treatment in glucocorticoid-treated children with juvenile idiopathic arthritis: a 3-year randomized study.

CONTEXT: Long-term glucocorticoid therapy adversely affects growth and body composition in children with juvenile idiopathic arthritis (JIA). In previous studies, recombinant human GH (rhGH) halted the progression of these complications without inducing catch-up growth. OBJECTIVES: The objective of the study was to evaluate the impact on growth and body composition of rhGH started early after glucocorticoid initiation and to record adverse effects in children with JIA. DESIGN: This is a 3-yr randomized controlled study. SETTING: This study was conducted in a teaching hospital. PATIENTS: Thirty children, 12-15 months into glucocorticoid therapy for severe JIA, were enrolled. INTERVENTION: Patients received rhGH (0.46 mg/kg.wk) in daily sc injections (n = 15) or no rhGH therapy (n = 15) for 3 yr. MAIN OUTCOME MEASURE: Difference in height sd score (SDS) change between the two groups was assessed. Height velocity, body composition, and oral glucose tolerance were evaluated yearly. RESULTS: Mean height SDS increase was larger with rhGH (+0.37 +/- 1.5 SDS) than without (-0.96 +/- 1.2 SDS) (P = 0.04). Mean height velocity returned to normal within the first year of rhGH treatment and remained normal thereafter. Mean lean mass increase was greater with rhGH treatment (+7.3 +/- 2.9 kg vs. +4.4 +/- 2.8 kg; P = 0.03). Fat mass and bone mineralization were not significantly different in the two groups. Fasting serum insulin increased significantly in rhGH-treated patients (5.2 +/- 16 mIU/liter) compared with untreated controls (-2.3 +/- 5 mIU/liter) (P = 0.04); fasting glycemia was unchanged. CONCLUSIONS: rhGH started early in the course of JIA preserved normal growth velocity and height. Although rhGH was well tolerated, carbohydrate metabolism should be monitored closely.

Proxy-reported health-related quality of life of patients with juvenile idiopathic arthritis: the Pediatric Rheumatology International Trials Organization multinational quality of life cohort study.

OBJECTIVE: To investigate the proxy-reported health-related quality of life (HRQOL) and its determinants in patients with juvenile idiopathic arthritis (JIA). METHODS: In this multinational, multicenter, cross-sectional study, HRQOL of patients with JIA was assessed through the Child Health Questionnaire (CHQ) and was compared with that of healthy children of similar age from the same geographic area. Potential determinants of HRQOL included demographic data, physician's and parent's global assessments, measures of joint inflammation, Childhood Health Assessment Questionnaire (CHAQ), and erythrocyte sedimentation rate. RESULTS: A total of 6,639 participants (3,324 with JIA and 3,315 healthy) were enrolled from 32 countries. The mean +/- SD physical and psychosocial summary scores of the CHQ were significantly lower in patients with JIA than in healthy children (physical: 44.5 +/- 10.6 versus 54.6 +/- 4.0, P < 0.0001; psychosocial: 47.6 +/- 8.7 versus 51.9 +/- 7.5, P < 0.0001), with the physical well-being domain being most impaired. Patients with persistent oligoarthritis had better HRQOL compared with other subtypes, whereas HRQOL was similar across patients with systemic arthritis, polyarthritis, and extended oligoarthritis. A CHAQ score >1 and a pain intensity rating >3.4 cm on a 10-cm visual analog scale were the strongest determinants of poorer HRQOL in the physical and psychosocial domains, respectively. CONCLUSION: We found that patients with JIA have a significant impairment of their HRQOL compared with healthy peers, particularly in the physical domain. Physical well-being was mostly affected by the level of functional impairment, whereas the intensity of pain had the greatest influence on psychosocial health.

A child with a systemic febrile illness - differential diagnosis and management.

Fever is a common symptom in children and may sometimes be prolonged or recurrent. There are many differential diagnoses, which may lead to significant diagnostic delay. Diagnosis is based on the clinical presentation as well as a widespread panel of investigations that are necessary in order to exclude the many potential causes of fever before reaching a definite diagnosis. In particular, the physician will look for infections and malignancies before considering the disease as inflammatory. This chapter reviews the differential diagnosis of prolonged or recurrent fever, and discusses most of the inflammatory syndromes presenting with fever.

Open label phase II trial of single, ascending doses of MRA in Caucasian children with severe systemic juvenile idiopathic arthritis: proof of principle of the efficacy of IL-6 receptor blockade in this type of arthritis and demonstration of prolonged clinical improvement.

Eighteen Caucasian (white, Middle East and Asian) children diagnosed by paediatric rheumatologists in the UK and France as having systemic juvenile idiopathic arthritis (sJIA) were enrolled in this open label, single dose trial. All patients had evidence of continued symptoms and disease activity for at least three months while receiving >0.2 mg/kg/day of prednisolone, or its equivalent, prior to recruitment. Twelve patients also received methotrexate (< or =20 mg/m2/week). The patients were divided into three groups receiving 2, 4 or 8 mg/kg of MRA (tocilizumab) by intravenous infusion. No evidence of dose-limiting toxicity was observed and there were no dose-limiting safety issues. MRA appeared to be dramatically effective, with clinical and laboratory responses observed by 48 h post infusion, and these improvements continued well after serum MRA was undetectable. Eleven patients achieved the JIA definition of improvement (at least 3 of 6 core set criteria with a 30% improvement and no more than one worsened by 30%) and eight achieved > or =50% improvement. There were no observable differences with age. Clinical improvement in these children was observed for up to eight weeks, supporting the hypothesis that IL-6 is a key cytokine in the upregulation of genes crucial in the inflammation processes of sJIA, and the possibility of sequestration of MRA in the extra-vascular compartment needs to be considered.

Improvements in growth parameters in children with juvenile idiopathic arthritis associated with the effect of methotrexate on disease activity.

OBJECTIVE: To assess changes in growth parameters associated with the response to methotrexate (MTX) therapy in pre-pubertal children with juvenile idiopathic arthritis (JIA) and who had not been treated with steroids. METHODS: We enrolled 27 pre-pubertal children with JIA who had been treated with MTX but not with steroids. The children were considered to have responded to treatment if the number of joints with active disease decreased by at least 50% 1 year after treatment initiation. We compared growth parameters (height, growth rate, weight and body mass index (BMI)) in responders and non-responders. RESULTS: Twenty-one children (77.8%) responded to MTX therapy. The growth parameters were similar in the responders and non-responders before the onset of treatment. After 1 year, height (P=0.025), growth rate (P=0.03), weight (P=0.007) and BMI (P=0.05) increased significantly in the responder group, but not in the non-responder group. This increase was maintained for growth rate and weight after 2 and 3 years of treatment. After 1 year, height (P=0.023) and growth rate (P=0.0009) were significantly higher in the responders than in the non-responders, and these differences were still significant after 3 years (P=0.01 and P=0.033, respectively). CONCLUSION: In pre-pubertal children with JIA, a clinical response to MTX therapy is associated with a significant increase in growth parameters.

Camptodactyly-arthropathy-coxa vara-pericarditis syndrome versus juvenile idiopathic arthropathy.

OBJECTIVE: The objective of our article was to highlight the important clinical and radiographic features of camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome. In particular, we emphasize those features that allow differentiation of CACP syndrome from juvenile idiopathic arthropathy. CONCLUSION: CACP syndrome should be considered in all patients who present with a noninflammatory arthropathy or with "atypical juvenile idiopathic arthritis," particularly if radiographs reveal an absence of erosions. In the correct clinical setting, large acetabular cysts on pelvic radiographs may be considered pathognomonic of CACP syndrome.