Clinician's dilemma: Naproxen-induced liver injury

Naproxen is a widely used nonsteroidal anti-inflammatory drug (NSAID) in pediatric population, used for mild-to-moderate pains, arthritis, and other immune-mediated disorders. It rarely causes clinically apparent liver injury in the adult population taking high doses of the drug over a prolonged period and is reported even rarer in pediatric population. We present a case of drug-induced liver injury (DILI) in a 13-year-old girl taking naproxen in therapeutic doses for juvenile rheumatoid arthritis. There was a complete recovery of liver function following discontinuation of naproxen therapy.

Clinical efficacy of intra-articular injection with Triamcinolone acetonide in patients with juvenile idiopathic arthritis / 中华实用儿科临床杂志

Objective:To observe the clinical efficacy of intra-articular injection with Triamcinolone acetonide on the treatment of juvenile idiopathic arthritis (JIA).Methods:The clinical data of 26 children diagnosed with JIA undergoing the intra-articular injection of Triamcinolone acetonide for the joints with obvious swelling and pain at the Children′s Hospital Affiliated to Capital Institute of Pediatrics from October 2018 to December 2019 who were retrospectively analyzed.Erythrocyte sedimentation rate (ESR) and C-reactive protein(CRP) were tested before and after the application of Triamcinolone acetonide.Detailed clinical manifestations were recorded.The nonparametric Kruskal- Wallis test was used to compare the differences in clinical evaluation indicators and changes in laboratory tests at diffe-rent treatment times. Results:Among the 26 children, 8 were boys and 18 were girls.After the intra-articular injection of Triamcinolone acetonide, 9 cases (34.62%) achieved complete remission, 15 cases(57.69%) achieved partial remission, and 2 cases (7.69%) were not responsive to the intra-articular injection.The overall therapeutic efficacy was 92.31%.Compared with pre-treatment period, from 4 weeks after treatment, assessment of disease activity by the physicians and parents of the children was significantly improved after 4-week treatment, and the number of active joints, ESR and CRP and the Juvenile Arthritis Disease Activity Score with 27 joints (JADAS 27) gradually decreased, and the differences were statistically significant (all P<0.05). No adverse drug reactions were seen during the treatment and follow-up period. Conclusions:Intra-articular injection of Triamcinolone acetonide is effective in contro-lling joint symptoms of JIA with less adverse events.

Massa óssea e composição corporal em adultos jovens com artrite idiopática juvenil / Bone mass and body composition in young adults with juvenile idiophatic arthritis

Objetivo: Avaliar a massa óssea e a composição corporal em adultos com história de ARJ. Métodos: Estudo caso-controle em 6 pacientes adultos com ARJ e 24 controles com mesmo perfil demográfico. Resultados: Peso e o IMC foram maiores nos pacientes com ARJ. Todos os parâmetros da composição corporal foram diferentes. Pacientes com ARJ apresentaram maior taxa de gordura corporal e menor porcentagem de massa magra apesar de possuírem peso e IMC maiores. A baixa massa óssea foi observada em dois pacientes com ARJ (33,3%) e em nenhum controle (0%) (Teste Exato de Fisher p=0,034). Embora a DMO média de todos os sites esqueléticos avaliados tenha sido menor no grupo JRA, não houve diferença estatística. Três pacientes com JRA (50,0%) usaram prednisona até a idade adulta com uma dose cumulativa média de 14,600 mg (± 9341,4 DP). Conclusões: Pacientes adultos com JRA ativo apresentaram maior frequência de baixa massa óssea e composição corporal diferente em relação ao grupo controle. Eles apresentaram mais massa gorda e menos massa magra.

Objective: To evaluate bone mass and body composition in adults with a history of JRA. Methods: A case-control study in 6 adult patients with JRA and 24 controls with same demographic profile. Results: Weight and BMI were higher in patients with JRA. All body composition parameters were different. JRA patients showed higher percentage of body fat mass and lower lean mass despite having higher weight and BMI. Low bone mass was observed in two patients with JRA (33.3%) and was not present in control group (0%) (Fisher's exact test p = 0.034). Although mean BMD of all evaluated skeletal sites was lower in JRA group there was no statistically difference. Three JRA patients (50.0%) used prednisone until adulthood with mean cumulative dose of 14,600 mg (± 9341.4 DP). Conclusions: Adult patients with active JRA showed higher frequency of low bone mass and different body composition in relation to control group. They presented more fat mass and less lean mass.

Características clínicas de niños y niñas con artritis idiopática juvenil del Instituto Teletón Santiago-Chile, año 2012-2013 / Clinical characteristics of children with juvenile idiopatic arthritis at Instituto Teletón Santiago-Chile, 2012-2013

Introducción: La artritis idiopática juvenil (AIJ) es un grupo heterogéneo de trastornos inflamatorios que se expresan con inflamación, dolor e impotencia funcional en una o más articulaciones en niños menores de 16 años. Las características clínicas y epidemiológicas planteadas por la literatura nacional e internacional son diversas. Objetivos: Describir las características clínicas de los pacientes con diagnóstico de AIJ, subtipos de clasificación, articulación más afectada, relación con género y edad promedio de incorporación al Instituto Teletón Santiago. Pacientes y Método: Estudio descriptivo, transversal. Se identificaron 217 pacientes en cubo OLAP con diagnóstico de AIJ, de éstos se revisaron 174 fichas clínicas (80,1 por ciento) de pacientes activos, con edades entre 7 y 18 años, que fueron atendidos en el Instituto Teletón Santiago durante los años 2012-2013. Los datos se procesaron mediante SPSS versión 17.0. Se calcularon medidas de resumen y la relación entre variables se estableció mediante test de asociación, con p < 0,05. Resultados: Según subtipo de artritis, las mayores frecuencias correspondieron a oligoarticular (31 por ciento) y poliarticular (22,4 por ciento). Las articulaciones más afectadas fueron rodilla (70 por ciento), tobillo (57,6 por ciento) y muñeca (45,9 por ciento). Un 70,1 por ciento del total de pacientes correspondieron al género femenino. Se verifica asociación al género femenino estadísticamente significativa (p = 0,002) en los subtipos poliarticular (razón femenino/masculino = 5,50), oligoarticular (razón femenino/masculino= 3,15) y sistémica (razón femenino/masculino = 1,83). La edad de incorporación al Instituto se registró en promedio a los 9,22 +/- 3,96 años. Conclusión: Las características clínicas generales, tuvieron similitud a lo descrito en literatura, sin embargo, sería conveniente realizar estudios posteriores a nivel nacional.

Introduction: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of inflammatory disorders, which manifest themselves with swelling, pain and loss of function in one or more joints, in children under 16 years of age. Clinical and epidemiological characteristics raised by the national and international literature are varied. Objectives: To describe the general clinical characteristics of patients diagnosed with JIA, regarding subtype classifications, most affected joint, gender and average age of admission at Instituto Teletón Santiago. Patients and Method: A descriptive cross-sectional study with 217 patients diagnosed with JIA identified through OLAP Cubes, was performed. Medical records for 174 (80.1 percent) active patients were reviewed, whose ages ranged from 7 to 18 years of age, and who were treated at the Instituto Teletón Santiago, between the years 2012- 2013. Data was processed using SPSS version 17.0. Summary measures and the association between variables were calculated using c2 Test of Association, with p < 0.05. Results: According to subtype of arthritis, olygoarticular obtained the higher frequencies (31 percent) and polyarticular (22.4 percent). The most commonly affected joints were, knee (70 percent), ankle (57.6 percent) and wrist (45.9 percent). A 70.1 percent of all patients, were female. Association to the female gender was statistically significant (p = 0.002) for the polyarticular subtypes (reason female/male = 5.50), oligoarticular (reason female/male = 3.15 ) and systemic (right female/male = 1.83). The age for admission to the Institute, was recorded at an average of 9.22 +/- 3.96 years. Conclusion: The general clinical characteristics of the population, were similar to those described in literature, however further studies would be useful at the national level.

Human Leukocyte Antigen-DRB1 Associations in Korean Juvenile Rheumatoid Arthritis

OBJECTIVE: The aim of this study is to investigate the association of different subgroups of juvenile rheumatoid arthritis (JRA) with human leukocyte antigen (HLA) class II DR alleles. METHODS: One hundred and nineteen Korean juvenile rheumatoid arthritis patients were classified as HLA-DRB1 allele. To assess the frequency, phenotype frequencies of all JRA cases and each subtypes were compared to those of 485 adult controls. RESULTS: HLA-DRB1*01 was associated with increased risk of JRA. Furthermore, DRB1*01 was associated with polyarticular JRA and pauciarticular JRA. The frequencies of DRB1*14 and DRB1*15 were higher in systemic JRA patients than the controls. CONCLUSION: The data of this study on Korean children with JRA suggests that HLA-DRB1*01 was associated with the susceptibility of JRA. The study should be extended to include larger numbers of patients.

The Clinical Significance of Anti-cyclic Citrullinated Peptide Antibodies in Juvenile Rheumatoid Arthritis

OBJECTIVE: The aim of this study was to determine the clinical significance of anti-cyclic citrullinated peptide (anti-CCP) antibody in juvenile rheumatoid arthritis (JRA). METHODS: Sera for anti-CCP assay were obtained from 142 patients with arthralgia in our hospital during the period between November 2010 and October 2011. On the basis of medical records, 83 patients with JRA were designated to the study group, and 59 patients with arthralgia but were treated as transient arthritis to the control group. The values of anti-CCP were then analyzed retrospectively. We used the enzyme linked immunosorbent assay (ELISA) for detecting anti-CCP. RESULTS: Positive anti-CCP values were found in the sera of 11 patients with JRA (13.3%), one patient presented with pauciarticular JRA and the others with polyarticular JRA. There was a statistically significant difference in the anti-CCP values between the JRA group and the control group. However, patients with systemic JRA and arthralgia without JRA had no anti-CCP. Especially, 10 out of 14 (71.4%) patients with RF-positive polyarticular JRA had anti-CCP. In addition, the rate of positive anti-CCP was significantly higher in patients with polyarticular JRA than in the control group. CONCLUSION: We expect that the presence of anti-CCP may help to evaluate the severity of diseases in patients with JRA. Anti-CCP can be an important biomarker for making early intensive therapeutic decision in patients with polyarticular JRA, which might have more severe course and longer disease duration.

A case of fat embolism syndrome in juvenile rheumatoid arthritis patient

Fat embolism syndrome is a serious complication that can occur after trauma or operation of the limbs. Clinical criteria are used for the diagnosis of fat embolism syndrome and sometimes radiologic findings are helpful. Fat embolism syndrome is known to occur less frequently in children than in adults, but there is an increased risk in children with connective tissue disease. However, there are only a few reported cases of fat embolism syndrome in juvenile rheumatoid arthritis which is the most common connective tissue disease in children. We report a case of fat embolism syndrome diagnosed in a 13-year-old boy with juvenile rheumatoid arthritis, which was treated with corticosteroid.

Alterações da articulação temporomandibular e suas repercussões orofaciais em pacientes portadores de artrite idiopática juvenil / Temporomandibular joint alterations and their orofacial complications in patients with juvenile idiopathic arthritis

Os pacientes com artrite idiopática juvenil (AIJ) podem apresentar alterações no metabolismo ósseo e no crescimento esqueletal, além de danos na articulação temporomandibular (ATM), o que pode gerar alterações extraorais e/ou intraorais, acarretando distúrbios craniofaciais. Nosso objetivo é promover uma revisão da literatura sobre as alterações orofaciais em pacientes com AIJ. Dentre os distúrbios orofaciais em pacientes com AIJ, as mudanças no crescimento mandibular, geradas por disfunções na região da ATM, parecem de grande prevalência nesses pacientes. As alterações mais encontradas são: retrognatismo, micrognatia, mordida aberta anterior, apinhamento dentário, assimetria facial e limitação de abertura de boca. Assim, o reumatologista torna-se uma peça-chave na detecção precoce dessas disfunções, ajudando no encaminhamento do paciente ao dentista. O diagnóstico, por sua vez, deve ser realizado pelo ortodontista, utilizando exames clínico e de imagem, o que possibilita tratamento precoce e prognóstico favorável. Distúrbios na ATM devem ser tratados de forma multidisciplinar, incluindo tratamento farmacológico para o controle da dor e tratamento odontológico por meio de aparelhagem funcional e/ou ortodôntica, fisioterapêutico e por vezes fonoaudiológico. Concluímos que dentre os distúrbios orofaciais em pacientes com AIJ, as mudanças no crescimento mandibular geradas por disfunções na região da ATM parecem de grande prevalência. Tais disfunções podem promover, principalmente, mordida aberta, retrusão mandibular, micrognatia, apinhamento dentário e assimetria facial. O reumatologista pode detectar tais alterações precocemente, encaminhando o paciente de imediato a uma equipe que deve ser, preferencialmente, multidisciplinar, composta por ortodontista, fisioterapeuta e fonoaudiólogo, visando reduzir futuras complicações oclusais e de crescimento mandibular.

Patients with juvenile idiopathic arthritis (JIA) can have alterations in bone metabolism and skeletal growth, as well as damage to the temporomandibular joint (TMJ), which can generate extra and/or intraoral alterations, resulting in craniofacial disorders. Our goal is to carry out a review of the literature on orofacial alterations in patients with JIA. Among the orofacial disorders in patients with JIA, alterations in mandibular growth, caused by dysfunctions in the TMJ region, seem highly prevalent in these patients. The most often found alterations are: retrognathia, micrognathia, anterior open bite, dental crowding, facial asymmetry and mouth opening limitation. Thus, the rheumatologist becomes a key agent in the early detection of these disorders, helping with patient referral to a dentist. The diagnosis, in turn, should be performed by the orthodontist, using clinical examination and imaging methods, allowing early treatment and a favorable prognosis. TMJ disorders should be treated by a multidisciplinary team, including pharmacological treatment for pain control and dental care through functional appliance and/or orthodontic therapy, physical therapy and sometimes, speech therapy. We conclude that among the orofacial disorders in patients with JIA, alterations in mandibular growth generated by dysfunctions in the TMJ region seem highly prevalent. Such dysfunctions can cause mainly open bite, mandibular retrusion, micrognathia, dental crowding and facial asymmetry. The rheumatologist can detect these alterations at an early stage, with immediate patient referral to a team that should preferably be a multidisciplinary one, consisting of an orthodontist, physical therapist and speech therapist, to reduce future occlusal and mandibular growth complications.

Capilaroscopia periungueal em crianças e adolescentes com doenças reumáticas / Nailfold capillaroscopy in children and adolescents with rheumatic diseases

OBJETIVO: Avaliar a capilaroscopia periungueal de crianças e adolescentes com doenças reumáticas autoimunes (artrite idiopática juvenil, lúpus eritematoso sistêmico, dermatomiosite juvenil, esclerodermia e doença mista do tecido conjuntivo) e relacioná-la com comprometimentos clínico e laboratorial e atividade de doença. MÉTODOS: Estudo transversal no qual foram avaliados 147 pacientes por meio de capilaroscopia periungueal: 60 com artrite idiopática juvenil, 30 com lúpus eritematoso sistêmico, 30 com dermatomiosite juvenil, 20 com esclerodermia localizada, quatro com esclerodermia sistêmica e três com doença mista do tecido conjuntivo. Exames clínico, laboratorial e de capilaroscopia periungueal foram realizados em todos os pacientes. A capilaroscopia periungueal foi realizada com microscópio óptico com aumentos de 10 e 16 vezes pelo mesmo observador. RESULTADOS: A maioria dos pacientes avaliados (76,2%) apresentou capilaroscopia periungueal normal. As maiores alterações na capilaroscopia periungueal foram observadas nos pacientes com dermatomiosite juvenil, esclerodermia sistêmica e doença mista do tecido conjuntivo, e caracterizaram o padrão escleroderma. Não houve associação entre capilaroscopia periungueal e atividade de doença nos pacientes com artrite idiopática juvenil, lúpus eritematoso sistêmico e esclerodermia localizada. Houve associação entre atividade da doença e alterações capilaroscópicas nos pacientes com dermatomiosite juvenil. CONCLUSÃO: A capilaroscopia periungueal é um método útil para o diagnóstico das doenças reumáticas autoimunes e para o acompanhamento de atividade de doença.

OBJECTIVE: To assess nailfold capillaroscopy in children and adolescents with autoimmune rheumatic diseases (juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, scleroderma and mixed connective tissue disease) and relate it to clinical and laboratory findings and disease activity. METHODS: Cross-sectional study assessing 147 patients by use of nailfold capillaroscopy as follows: 60 with juvenile idiopathic arthritis; 30 with systemic lupus erythematosus; 30 with juvenile dermatomyositis; 20 with localized scleroderma; four with systemic sclerosis; and three with mixed connective tissue disease. Clinical and laboratory tests and nailfold capillaroscopy were performed in all patients. The nailfold capillaroscopy was performed with an optical microscope (at 10- and 16-time magnifications) by the same observer. RESULTS: Most patients (76.2%) had normal nailfold capillaroscopy. The major changes in nailfold capillaroscopy, characterizing the scleroderma pattern, were observed in patients with juvenile dermatomyositis, systemic scleroderma and mixed connective tissue disease. There was no association between nailfold capillaroscopy and disease activity in patients with juvenile idiopathic arthritis, systemic lupus erythematosus and localized scleroderma. Disease activity and capillaroscopy were associated in patients with juvenile dermatomyositis. CONCLUSION: Nailfold capillaroscopy is a useful method to diagnose autoimmune rheumatic diseases and monitor disease activity.

La artritis idiopática juvenil y su relevancia dentro de las enfermedades reumatológicas de la infancia / Juvenile idiopathic arthritis and its relevance in rheumatological diseases of children

El dolor y la inflamación que afecta a las articulaciones o tejidos periarticulares son motivo frecuente de consulta a nivel pediátrico. Dentro de los diagnósticos diferenciales se encuentran las enfermedades reumatológicas. En los últimos años ha cambiado el pronóstico y visión que se tenía de estas enfermedades gracias a los nuevos conocimientos sobre la patogenia y a la incorporación de nuevas terapias con agentes biológicos, basados en anticuerpos monoclonales y que se ha traducido en un cambio en los tratamientos convencionales de estas enfermedades. La Artritis Reumatoidea Juvenil, actualmente denominada Artritis Idiopática Juvenil (AIJ), constituye la enfermedad más frecuente dentro del espectro de estos cuadros autoinmunes. El objetivo de esta presentación es dar a conocer las diversas formas de AIJ y los principales hallazgos clínicos y de laboratorio que pueden orientar al clínico acerca de estas enfermedades y así iniciar un tratamiento oportuno que asegure un buen pronóstico de la enfermedad.

Musculoskeletal pain, joint pain and arthritis are a common complaint in pediatric practice. Among the differential diagnosis for these conditions are rheumatic diseases. Treatment and outcome of these conditions has greatly improved in recent years due to advances in the knowledge of the underlying mechanisms and the development of new therapies with biologic agents, based on monoclonal antibodies. These new therapies have changed the outcome and vision of these diseases. Among the different rheumatologic diseases described in children, Juvenile Rheumatoid Arthritis, now called Juvenile Idiopathic Arthritis (JIA), is the most common disease within the spectrum of autoimmune conditions. The aim of this presentation is to show the different forms of JIA and the main clinical and laboratory findings that can guide the clinician to an early diagnosis and initiate a timely treatment that can guarantee a better prognosis.

Osteoporosis en enfermedades reumáticas / Osteoporosis in rheumatic diseases

La osteoporosis es una enfermedad compleja que resulta de la interacción de células del sistema óseo, principalmente osteoblastos, osteoclastos y osteocitos, que está regulada por varias hormonas y citokinas que interactúan sobre un variado sistema de transducción de señales que derivan en reabsorción o formación de hueso. En varias enfermedades reumáticas este delicado balance se pierde por efecto de la enfermedad misma, aunque también influye poderosamente el tratamiento empleado. En este artículo se revisa el mecanismo general de la fisiología del tejido óseo y su alteración por la inflamación, que son la base de las enfermedades reumáticas. También se revisa la pérdida de masa ósea inducida por esteroides y su tratamiento.

Osteoporosis is a complex disease that results from the interaction of cells in the bone marrow, mainly osteoblasts, osteoclasts and osteocytes, which is regulated by several hormones and cytokines that interact on a variety of signal transduction system that lead to bone resorption or formation. In several rheumatic diseases this delicate balance is lost due to the disease itself, but also strongly influenced by the treatment used. This article reviews the general mechanism of bone physiology and its alteration by inflammation, which are the basis of rheumatic diseases. We also review the bone loss induced by steroid and its treatment.

Juvenile Rheumatoid Arthritis in Children with Ebstein-barr Virus Infection

OBJECTIVE: Juvenile rheumatoid arthritis (JRA) may occur in the wake of infection with several viruses including Ebstein-barr virus (EBV). EBV remains an interesting target. To determine the possible role of EBV infections in the clinical course of JRA, we attempt to demonstrate the radiologic changes and the frequency prescription of etanercept rather than classic therapy. METHODS: Total of 87 patients with JRA, who were hospitalized in Hangang Sacred Hospital and Kangnam Sacred Hospital in Seoul from 2002 to 2010, were assessed serologically for EBV infection (anti EBV VCA IgM and IgG) at admission. Patients with JRA were devided 2 groups, one is EBV VCA IgG (+) JRA patients who had been infected before and another is EBV VCA IgG (-) JRA patients who had not. RESULTS: EBV VCA IgG (+) were seen in 55 patients (63.2%). 31 boys (76%) and 24 girls (52%) were infected with EBV. The mean age of patients of EBV (+) JRA was 8.2+/-3.6 years and that of EBV (-) JRA was 5.3+/-3.4 years. 7 of EBV (+) JRA (13%) developed radiologic change within 2 years, compare with none of EBV (-) JRA. 22 of EBV (+) JRA (49%) with JRA did not respond to the classic therapy, compare with 7 of EBV (-) JRA (22%). CONCLUSION: JRA patients with past EBV infection were older in ages, more in male, more radiologic changes, needed more biologic treatment than those without past EBV infection.

Fiberoptic intubation through a laryngeal mask airway as a management of difficult airwary due to the fusion of the entire cervical spine: A report of two cases / 대한마취과학회지

Patients with cervical spine instability and limited range of motion are challenge to anesthesiologists. It is important to consider alternatetive methods for securing the airway while maintaining neutral position and minimizing neck motion, because these patients are at increased risk for tracheal intubation failure and neurologic injury during airway management or position change. We experienced two cases that patients had cervical spine instability and severe limited range of motion due to the fusion of the entire cervical spine. One patient was a 6-year-old girl weighing 12.7 kg and had Klippel-Feil syndrome with Arnold-Chiari malformation, the other was a 24-year-old female weighing 31 kg and had juvenile rheumatoid arthritis. We successfully performed the intubation by using the fiberoptic intubation though a laryngeal mask airway in these two cases.

Childhood granulomatous periorificial dermatitis in children with extra-facial involvement.

Childhood granulomatous periorificial dermatitis (CGPD) is a self-limiting and well-recognized entity. A six-year-old male child, a known case of juvenile rheumatoid arthritis (JRA) presented with multiple red raised and yellowish lesions over the face, neck, trunk and upper extremities since one month with occasional itching. Cutaneous examination revealed multiple erythematous scaly papules of size up to 5 mm around the mouth, nose and periorbital areas, neck, trunk and upper extremities with few excoriations. Lesional skin biopsy was pathognomic of CGPD. We report a six-year-old Indian male child with extra-facial involvement and healing with small atrophic pigmented scars in a known case of JRA.

The clinical features and quality of life study of juvenile idiopathic arthritis / 国际儿科学杂志

Objective To explore the clinical features and long-term prognosis of juvenile idiopathic arthritis (JIA)in children, as well as particular quality of life associated with the clinical features and therapeutic options. Methods Seventy patients from August 1997 to August 2007 were retrospectively reviewed and survey was conducted using a questionnaire indicating quality of life(CHAQ questionnaires and CHQ questionnaire, adjusted appropriately) either by phone,letter or out-patient follow-up approach.The manner of correlation and logistic regression was used to analyze quality of life associated with the clinical features and therapeutic options. Results (l)The majority (74.3%) of patients were diagnosed as systemic onset JIA. The Cronbach Alpha coefficient of questionnaire is 0.9599, with a higher inernal consistency reliability. The scales validity also had the higher construction. Sixty nine cases with 76.8 percent of the score showed good quality of life. Quality of life in children less than 5 years old was significantly different from those more than 5-year old( P < 0.05). The rank sum test of single-use NSAIDs group and NSAIDs adding other medicine group suggested that the two groups had differences in quality of life( P = 0.026) .The correlation coefficient between the variables of time from onset to formal treatment and quality of life scores was 0.329( P < 0.05), and "walking"as the dependent variable for the regression analysis showed regression coefficient was 0.15( P = 0.016). The variable grip had the significant relationship with the "disease onset to regular treatment, the initial number of joint involvement" respectively. Conclusion Systemic oneset JIA is the most common type of JIA. Most of JIA patients have a good prognosis.The major factors which may cause the decline in the quality of life are age, duration without therapy after the disease onset,and the compliance to medication.To achieve a better prognosis,more aggressive therapy may be needed and individulized.

A case of encephalitis in a juvenile rheumatoid arthritis patient treated with etanercept / 소아과

Tumor necrosis factor-alpha (TNF-alpha) is a major proinflammatory cytokine involved in the pathophysiology of juvenile rheumatoid arthritis. Etanercept is an effective inhibitor of TNF-alpha and has shown a beneficial effect in patients with JRA. However, the most important cause of concern related to etanercept administration is infection. We report a case of encephalitis in a JRA patient receiving long-term treatment with etanercept. The patient was a 4-year-old boy with refractory JRA, and he received etanercept subcutaneously at a dose of 0.4 mg kg(-1) day(-1) twice a week for 14 months, along with non-steroidal anti-inflammatory drugs, methotrexate, oral steroids, and sulfasalazine. The patient presented with sudden fever, headache, vomiting, a generalized tonic seizure, and changes in mental status. We suspected a central nervous system infection, and simultaneously administered antibiotics, an antiviral agent, and steroids. After 2 days of hospitalization, his mental function returned to normal, and he showed no further seizure-like movements. Brain magnetic resonance imaging scan of the patient showed a multifocal cortical lesion on both sides of the temporoparietooccipital lobe, which indicated encephalitis. Although we were unable to identify the causative organism of encephalitis, we think that the encephalitis may be attributed to infection, and the use of etanercept may have increased the risk of severe infection. Therefore, etanercept was discontinued and the patient recovered shortly after. To the best of our knowledge, this is the first case of encephalitis in a juvenile rheumatoid arthritis patient treated with etanercept.

Development of juvenile rheumatoid arthritis in a patient with multiple sclerosis / 대한내과학회지

Multiple sclerosis (MS) is an immune-mediated, inflammatory demyelinating disease of the central nervous system. The coexistence of multiple sclerosis and rheumatoid arthritis is very rare. This rare association is interesting due to the overlapping pathophysiological similarities of T-cells and tumor necrosis factor-alpha (TNF-alpha) in both diseases. We report a case of a Korean 10 year old girl with multiple sclerosis, who after 3 years developed a clinical and serological manifestation of juvenile rheumatoid arthritis.

Síndrome de Evans na evolução da doença de Still / Evans syndrome occurring in Still's disease

Objetivo: Mostrar a importância do diagnóstico precoce e tratamento da síndrome de Evans, ocorrendo como complicação de uma doença sistêmica, a doença de Still. Descrição: Relato do caso da paciente A.L.F.T., 4 anos e 1 mês, com diagnóstico de doença de Still há um ano e cuja evolução, durante sua hospitalização, complicou-se com a síndrome de Evans. Comentário: A síndrome de Evans, ocorrendo no curso da doença de Still, é uma entidade clínica infreqüente, mas importante em termos do prognóstico do paciente. É difícil a distinção dos sintomas dessas enfermidades e a estratégia de tratamento constitui uma urgência.

Juvenile rheumatoid arthritis / 소아과

The diagnosis of juvenile rheumatoid arthritis (JRA) is based on patient's age at disease onset, symptom duration, gender, and clinical manifestations. JRA is of unknown origin, begins under the age of 16, and persists for a minimum of 6 weeks. JRA is categorized into three principal types, systemic, oligoarticular and polyarticular. Infection, other connective tissue diseases, malignancy, trauma, and immunodeficiency are discussed as differential diagnoses for JRA. Because of joint damage, focusing on early diagnosis and intervention, a vigorous initial therapeutic approach must be taken in patients who have poor prognostic factors. A multidisciplinary team approach is also important for the care of patients with JRA.