Valores de referencia para el 1-minute sit-to-stand test en pacientes pediátricos con artritis idiopática juvenil / Reference values for the 1-minute sit-to-stand test in pediatric patients with juvenile idiopathic arthritis

Introducción: los niños con artritis idiopática juvenil (AIJ) experimentan períodos de dolor e inmovilidad que afectan sus capacidades condicionales. Objetivos: describir los valores de referencia para el 1-minute Sit-to-Stand Test(1-STS; test de 1 minuto de sentarse y pararse) en niños con AIJ como evaluación de la capacidad aeróbico-funcional y de la fuerza muscular de los miembros inferiores (MMII).Materiales y métodos: se realizó un estudio observacional que incluyó a 15 niños con AIJ de entre 5 y 16 años. Se evaluó su rendimiento en el 1-STS. Resultados: se encontró una correlación positiva significativa entre el 1-STS y el test de la marcha de 6 minutos (r=0,56; p=0,03), como con el índice de capacidad funcional (CAPFUN) (r=0,54; p=0,03). No se observaron correlaciones significati-vas entre el Childhood Health Assessment Questionnaire (CHAQ) y el 1-STS (r=-0,21; p=0,44), tampoco con el Juvenile Arthritis Disease Activity Score (JADAS-10) (p=0,83). Conclusiones: el 1-STS parece prometedor para medir la capacidad aeróbi-co-funcional y la fuerza muscular de los miembros inferiores en niños con AIJ oligoarticular.

Introduction: children with juvenile idiopathic arthritis (JIA) experience periods of pain and immobility that affect their physical capacities. Objectives: to describe reference values for the 1-minute sit to stand test (1-STS) in children with JIA as an assessment of aerobic-functional capacity and lower limb muscle strength.Materials and methods: an observational study was conducted, including 15 children with JIA aged between 5 and 16 years. Their performance in the 1-STS was assessed. Results: a significant positive correlation was found between the 1-STS and the 6-Minute Walk Test (r=0,56; p=0,03), as well as with the Functional Capacity Index (CAPFUN) (r=0,54; p=0,03). No significant correlations were observed between the Childhood Health Assessment Questionnaire (CHAQ) and the 1-STS (r=-0,21; p=0,44), nor with the Juvenile Arthritis Disease Activity Score (JADAS-10) (p=0,83). Conclusions: the 1-STS appears promising for assessing aerobic-functional capacity and lower limb muscle strength in children with oligoarticular JIA.

Valores de referencia para el 1-minute sit-to-stand test en pacientes pediátricos con artritis idiopática juvenil / Reference values for the 1-minute sit-to-stand test in pediatric patients with juvenile idiopathic arthritis

Resumen Introducción: los niños con artritis idiopática juvenil (AIJ) experimentan períodos de dolor e inmovilidad que afectan sus capacidades condicionales. Objetivos: describir los valores de referencia para el 1-minute Sit-to-Stand Test (1-STS; test de 1 minuto de sentarse y pararse) en niños con AIJ como evaluación de la capacidad aeróbico-funcional y de la fuerza muscular de los miembros inferiores (MMII). Materiales y métodos: se realizó un estudio observacional que incluyó a 15 niños con AIJ de entre 5 y 16 años. Se evaluó su rendimiento en el 1-STS. Resultados: se encontró una correlación positiva significativa entre el 1-STS y el test de la marcha de 6 minutos (r=0,56; p=0,03), como con el índice de capacidad funcional (CAPFUN) (r=0,54; p=0,03). No se observaron correlaciones significativas entre el Childhood Health Assessment Questionnaire (CHAQ) y el 1-STS (r=-0,21; p=0,44), tampoco con el Juvenile Arthritis Disease Activity Score (JADAS-10) (p=0,83). Conclusiones: el 1-STS parece prometedor para medir la capacidad aeróbico-funcional y la fuerza muscular de los miembros inferiores en niños con AIJ oligoarticular.

Abstract Introduction: children with juvenile idiopathic arthritis (JIA) experience periods of pain and immobility that affect their physical capacities. Objectives: to describe reference values for the 1-minute sit to stand test (1-STS) in children with JIA as an assessment of aerobic-functional capacity and lower limb muscle strength. Materials and methods: an observational study was conducted, including 15 children with JIA aged between 5 and 16 years. Their performance in the 1-STS was assessed. Results: a significant positive correlation was found between the 1-STS and the 6-Minute Walk Test (r=0,56; p=0,03), as well as with the Functional Capacity Index (CAPFUN) (r=0,54; p=0,03). No significant correlations were observed between the Childhood Health Assessment Questionnaire (CHAQ) and the 1-STS (r=-0,21; p=0,44), nor with the Juvenile Arthritis Disease Activity Score (JADAS-10) (p=0,83). Conclusions: the 1-STS appears promising for assessing aerobic-functional capacity and lower limb muscle strength in children with oligoarticular JIA.

Advances in enthesitis-related arthritis / 国际儿科学杂志

Enthesitis related arthritis（ERA）is one of the subtypes of juvenile idiopathic arthritis（JIA）. It is a group of diseases characterized by arthritis，inflammation，axial lesions，and human leucocyte antigen-B27 positive. In the previous classification criteria，ERA does not include all of the subsets of juvenile spondyloarthritis（JSpA）. Based on an evidence-based approach，the Pediatric Rheumatology International trials Organization developed the latest classification criteria in 2019，and proposed the classification criteria of ERA/SpA，to distinguish it from other subtypes of JIA. The new definition added the term spondylitis，included imaging criteria，and adopted a new definition of back pain. The pathogenesis，evolution of classification criteria and prognosis of spondyloarthritis are described in this paper，in order to improve the level of diagnosis and treatment of patients with axial joint involvement，so as to improve the prognosis.

Analysis of therapeutic effect of thalidomide on refractory systemic onset juvenile idiopathic arthritis / 国际儿科学杂志

Objective:To analyze and summarize the efficacy and safety of thalidomide in the treatment of refractory systemic juvenile idiopathic arthritis（sJIA）.Methods:The clinical data of ten patients with refractory sJIA admitted to Department of Nephrology and Immunology in Children's Hospital of Hebei Province from January 2015 to March 2022 were collected，and the clinical manifestations，efficacy and safety of thalidomide in the treatment of refractory sJIA were analyzed retrospectively. Systemic juvenile arthritis disease activity score（sJADAS）was used to evaluate the efficacy of the treatment. Statistical analysis was performed by repeated measurements using general linear models.Results:Among the 10 children（4 males and 6 females）with refractory sJIA，the average age of onset was（7.5±3.3）years. Seven patients were complicated with macrophage activation syndrome at an early stage of disease.The average course of disease was（4.4±1.7）years，and the longest course of disease was 8.3 years. Before the application of thalidomide，all the 10 children experienced relapses（ranging from 2 to 10 times）. The indices of 10 children treated with thalidomide at 6 months and 12 months were compared with those before treatment. Peripheral blood leukocytes［（10.19±3.67）×10 9/L，（8.53±2.83）×10 9/L vs.（16.11±7.81）×10 9/L， F=7.918，11.084， P=0.020，0.009］，C-reactive protein［19.13（0.38，35.21）mg/L，8.05（0.10，18.00）mg/L vs. 59.34（24.20，131.90）mg/L， F=7.030，12.731， P=0.026，0.006］，sJADAS scores［6.00（1.50，12.50）scores，3.00（0，12.50）scores vs. 20.00（11.50，28.00）scores， F=14.710，17.870， P=0.004，0.002］were decreased significantly. The doses of prednisone［0.13（0，0.45）mg/（kg·d），0.02（0，0.06）mg/（kg·d）vs. 0.42（0.16，1.47）mg/（kg·d）， F=5.890，7.623， P=0.041，0.022］were significantly decreased.All the differences were statistically significant. Prednisone was successfully discontinued in 7 cases. Tocilizumab was gradually withdrawn in 3 cases，and tocilizumab administration interval was prolonged in 1 case. None of the 10 children had serious adverse reactions. Conclusion:Thalidomide is clinically effective in the treatment of sJIA，and can reduce the required dose of prednisone and prolong the tocilizumab free remission.

Professor FAN Yongsheng's Academic Experience in Treating Systemic Juvenile Idiopatic Arthritis from the Latent Pathogens Warm Disease / 浙江中医药大学学报

[Objective]To review and summarize the academic experience of Professor FAN Yongsheng,one of the first session national famous traditional Chinese medicine masters,in the treatment of systemic juvenile idiopathic arthritis.[Methods]Through follow-up study,sorting out and analyzing typical medical records,and referring to relevant literature,combined with Professor FAN Yongsheng's works and clinical practice,Professor FAN Yongsheng's academic experience in the treatment of systemic juvenile idiopathic arthritis was summarized from two aspects of etiology,pathogenesis and treatment ideas,and one proven case was attached for evidence.[Results]Professor FAN Yongsheng believes that the pathogenic process of systemic juvenile idiopathic arthritis conforms to the law of the transformation of warm diseases Wei Qi Ying Xue,which is similar to the pathogenic characteristics of latent pathogenic febrile diseases.It is mostly caused by the deficiency of healthy Qi in the interior,combined with the invasion of external pathogens,hidden in the interior,waiting for opportunities,or triggered by external pathogens.Clinical application of fire stagnation requires dissipation,dispersing and expelling the latent pathogens;protecting the healthy Qi,moderating exorcism;sequential therapy,toxicity reduction and efficacy enhancement and other treatment ideas have achieved satisfactory clinical results in the treatment of this disease.In the test case,the initial manifestation of the patient was the pathogen lurking in Yin phase,dual blaze of Qi and Ying,and the treatment was to clear heat toxin and harmonize Ying,dispel wind and dredge collaterals;after treatment,rash regression and fever relieve,latent pathogens were affected from Yin to Yang and cardinal dysfunction,and the treatment was to reconcile cardinal,clear and expel the stagnant heat;when the pathogenic Qi disappeared gradually,the healthy Qi declined,the treatment was given to disperse and expel the heat,combined with invigorating the spleen and harmonizing the stomach.The whole process of treatment was permeated throughout dispersing latent pathogens,and at the same time reflecting Professor FAN Yongsheng's treatment ideas of flexible drug use,taking care of healthy Qi.[Conclusion]Professor FAN Yongsheng has achieved good clinical results in the treatment of systemic juvenile idiopathic arthritis based on the theory of latent pathogenic warm disease,and his academic experience is worthy of reference and promotion.

Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin

Abstract Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes of secondary HLH, such as infections and malignancy. In this article, we reviewed the concepts, epidemiology, clinical and laboratory features, diagnosis, differential diagnosis, prognosis, and treatment of HLH and MAS. We also reviewed the presence of MAS in the most common autoimmune diseases that affect children. Both are severe diseases that require prompt diagnosis and treatment to avoid morbidity and mortality.

Artritis idiopática juvenil, forma sistémica. A propósito de un caso

La artritis idiopática juvenil es la enfermedad reumática crónica más frecuente en niños, y una de las enfermedades crónicas más comunes en la infancia. En Angola no se han realizado revisiones de casos de dicha enfermedad de inicio sistémico, y solo se han reportado escasas publicaciones en el continente africano, en países como Egipto y Sudáfrica. El objetivo de este trabajo es describir un caso de artritis idiopática juvenil sistémica en una paciente de dos años que presentó síntomas como poliartritis, eritema evanescente, adenopatías, fiebre prolongada y visceromegalias. Este es el primer caso de artritis idiopática juvenil reportado en dicho país.

Juvenile idiopathic arthritis is the most frequent rheumatic disease and one of the most common chronic diseases in childhood. In Angola, there are no reviews reported of this systemic onset disease, and only few publications have been reported on the African continent, in countries such as Egypt and South Africa. The objective of this work is to inform on a case of systemic juvenile idiopathic arthritis in a two-years-old patient who presented symptoms such as polyarthritis, evanescent erythema, lymphadenopathies, prolonged fever and visceromegaly. This is the first case of juvenile idiopathic arthritis reported in that country.

Visual outcome in pediatric uveitis: A retrospective data review in 277 children

Purpose: The research activity in pediatric glaucoma (PG) was qualitatively and quantitatively evaluated using a scientometric approach. Methods: The “Web of Science” database was accessed for primary bibliometric data regarding PG using search terms “pediatric glaucoma,” “paediatric glaucoma,” “congenital glaucoma,” and “childhood glaucoma.” The data was analyzed for total research productivity, citations, and scientific output in terms of journals, countries, institutions, and authors. The results were further characterized for coauthorship links and visualized by VOS viewer software. Also, the top 25 cited articles were reviewed with the above bibliometric characteristics. Results: One thousand two hundred and sixty?nine items were obtained from our search query from 1955 to 2022; these received 15,485 citations, originated from 78 countries. The top?3 contributing countries were the United States of America (n = 369), India (n = 134), and China (n = 127). LV Prasad Eye Institute (n = 58), Duke University (n = 44), and King Khalid Eye Specialist Hospital (n = 42) were the top?3 productive institutes. The top?3 prolific authors were Mandal AK (n = 53), Freedman, SF (n = 36), and Sarfarazi, M (n = 33). Journal wise, “Investigative Ophthalmology” (n = 187), “Journal of Glaucoma” (n = 92), and “Journal of AAPOS” (n = 68) were the journals in which the most articles were published. The top?25 cited documents received 3564 citations and were published between 1977 and 2016. The key areas of interest were basic sciences (genetics of childhood glaucoma) and surgical management. Conclusion: United States of America, LVPEI, Mandal AK, and “Investigative Ophthalmology” were the top rankers as far as the productivity and publications related to PG are concerned. Articles on molecular genetics in PG have received interest among the ophthalmology community.

Role of CD4+NKG2D+ T cells in the disease activity of juvenile idiopathic arthritis / 中国当代儿科杂志

OBJECTIVES@#To study the expression levels of CD4+NKG2D+ T cells and NKG2D soluble ligands, the soluble MHC class I chain-related molecules A and B (sMICA/sMICB) in the active stage and stable stage of juvenile idiopathic arthritis (JIA) and their role in the disease activity of JIA.@*METHODS@#Nineteen children with systemic JIA and 20 children with articular JIA who were diagnosed in Children's Hospital of Chongqing Medical University from November 2019 to December 2021 were enrolled in this prospective study. Six healthy children were enrolled as the control group. After peripheral blood samples were collected, ELISA was used to measure the levels of sMICA and sMICB, and flow cytometry was used to measure the percentage of CD4+NKG2D+ T cells. Systemic Juvenile Arthritis Disease Activity Score-27 (sJADAS-27)/Juvenile Arthritis Disease Activity Score-27 (JADAS-27) was used to evaluate the disease activity in children with JIA. The Pearson correlation analysis and the receiver operating characteristic (ROC) curve were used to assess the role of CD4+NKG2D+ T cells, sMICA and sMICB in the disease activity of JIA.@*RESULTS@#The active systemic JIA and active articular JIA groups had a significant increase in the percentage of CD4+NKG2D+ T cells compared with the control group and their corresponding inactive JIA group (P<0.05). The JIA groups had significantly higher levels of sMICA and sMICB than the control group (P<0.05), and the active articular JIA group had a significantly higher level of sMICB than the stable articular JIA group (P<0.05). In the children with JIA, the percentage of CD4+NKG2D+ T cells and the levels of sMICA and sMICB were positively correlated with sJADAS-27/JADAS-27 disease activity scores (P<0.05). The ROC curve analysis showed that sMICB had an area under the curve of 0.755 in evaluating the disease activity of JIA, with a specificity of 0.90 and a sensitivity of 0.64.@*CONCLUSIONS@#The percentage of CD4+NKG2D+ T cells and the levels of sMICA and sMICB increase in children with JIA compared with healthy children and are positively correlated with the disease activity of JIA, suggesting that CD4+NKG2D+ T cells and NKG2D ligands can be used as potential biomarkers for evaluating the disease activity of JIA.

A preliminary study on the role of V-domain Ig suppressor of T cell activation in juvenile idiopathic arthritis / 中国当代儿科杂志

OBJECTIVES@#To study the expression of V-domain Ig suppressor of T cell activation (VISTA) in peripheral blood of children with juvenile idiopathic arthritis (JIA) and its role in the pathogenesis of JIA.@*METHODS@#In this prospective study, peripheral blood was collected from 47 children with different subtypes of JIA and 10 healthy children. Flow cytometry was used to measure the expression levels of VISTA, interferon-γ (IFN-γ), and tumor necrosis factor-α (TNF-α) on CD14+ mononuclear cells, CD4+ T lymphocytes, and CD8+ T lymphocytes.@*RESULTS@#The children with JIA had a significantly lower expression level of VISTA than the healthy children (P<0.05). There was a significant difference in the expression of VISTA between the children with different subtypes of JIA, with the lowest expression level in those with systemic JIA (P<0.05). There was also a significant difference in the expression of VISTA between different immune cells, with a significantly higher expression level on the surface of monocytes (P<0.05). Correlation analysis showed that VISTA was negatively correlated with the expression of IFN-γ and TNF-α on CD4+ T cells (r=-0.436 and -0.382 respectively, P<0.05), CD8+ T cells (r=-0.348 and -0.487 respectively, P<0.05), and CD14+ mononuclear cells (r=-0.582 and -0.603 respectively, P<0.05).@*CONCLUSIONS@#The insufficient expression of VISTA may be associated with the pathogenesis of JIA, and enhancing the immunomodulatory effect of VISTA might be one option for the treatment of JIA in the future.

Advances in systemic juvenile idiopathic arthritis associated-lung disease / 中华实用儿科临床杂志

Systemic juvenile idiopathic arthritis(sJIA) is one of the most serious critical illnesses in childhood, characterized by high fever, recurrent rash, and arthritis, etc.Children with sJIA associated-lung disease(sJIA-LD) are more severely ill and have a worse prognosis, the correlation between the mechanism and age, disease activity, anti-rheumatic drug therapy, applications of biologics, infection and other factors is worth exploring.This article reviews the research progress on the mechanism, risk factors, treatment methods and prognosis of sJIA-LD, so as to provide a theoretical basis for improving the diagnosis and treatment of sJIA and improving the prognosis.

A case of systemic juvenile idiopathic arthritis with an early manifestation of pulmonary ground-glass opacities combined with macrophage activation syndrome / 中国综合临床

Systemic juvenile idiopathic arthritis is one of the common rheumatic and immune diseases in children. It has a sudden onset, obvious systemic symptoms, and lung involvement. However, systemic juvenile idiopathic arthritis with an early manifestation of pulmonary ground-glass opacities combined with macrophage activation syndrome is rare. The clinical data of a child with systemic juvenile idiopathic arthritis with pulmonary ground-glass shadow and macrophage activation syndrome who was admitted to Hubei Maternal and Child Health Care Hospital affiliated to Tongji Medical College of Huazhong University of Science and Technology in December 2021 were analyzed retrospectively in order to improve the understanding of rheumatic diseases and pulmonary lesions. The child was admitted to the hospital for 10 days due to rash and fever. Thoracic CT showed scattered ground glass like shadows in both lungs due to the prevention and control screening of COVID-19 pneumonia epidemic situation. After admission, the child was still repeatedly flaccid with high fever, accompanied by dysfunction of both lower limbs. The knee joint MRI found that there was synovitis in the knee joint, and various laboratory indicators suggested macrophage activation syndrome. After that, systemic juvenile idiopathic arthritis was diagnosed. After being treated with methylprednisolone, cyclosporine and topzumab, the clinical remission and the ground-glass shadow of the lung basically disappeared. Through the analysis of this case, it is suggested that clinicians should not ignore other diseases that cause ground glass shadow in the lung during the current epidemic of COVID-19.

Clinical observation of early-onset antinuclear antibody-positive juvenile idiopathic arthritis / 中华微生物学和免疫学杂志

Objective:To investigate the clinical features, treatment and follow-up of children with early-onset antinuclear antibody (ANA)-positive juvenile idiopathic arthritis (JIA).Methods:Eighty-six oligoarticular JIA patients with early-onset arthritis (≤6 years old) admitted to the Children′s Hospital Affiliated to Capital Institute of Pediatrics from January 2017 to December 2019 were included in this study. According to ANA titer, these patients were divided into two groups: ANA-positive group (44 cases) and ANA-negative group (42 cases). Clinical data including demographic data, clinical features, laboratory testing results, treatment and follow-up data were statistically analyzed.Results:The ratio of male to female was 7∶37 in the ANA-positive group and 15∶27 in the ANA-negative group and there was significant difference between the two groups ( P=0.035). The proportions of patients with increased C-reactive protein and erythrocyte sedimentation rate were higher in the ANA-positive group than in the ANA-negative group [18.18% (8/44) vs 16.67% (7/42) and 29.55% (13/44) vs 19.05% (8/42), both P>0.05]. The most commonly involved joints in the ANA-positive group were knee (95.45%, 42/44), ankle (20.45%, 9/44) and wrist (18.18%, 8/44), and unilateral asymmetric joint involvement accounted for 81.8% (36/44). In the ANA-negative group, the involved joints were knee (85.71%, 36/42), ankle (14.29%, 6/42), wrist (14.29%, 6/42) and hip (11.90%, 5/42), and 27 out of the 42 cases (64.29%) had unilateral asymmetric joint involvement. There was no significant difference in the above indexes between the two groups (all P>0.05). There were seven cases (15.91%) with uveitis in the ANA-positive group and two cases (4.76%) in the ANA-negative group, and the difference between the two groups was significant ( P=0.045). Before treatment, the ANA-positive group had a significantly higher disease activity score (JADAS27) than the ANA-negative group (14.43±2.87 vs 12.09±3.32, P=0.002). After treatment, the JADAS27 score in both groups decreased (both P<0.05). After six months of treatment, the two groups had similar clinical remission rates [70.45% (31/44) vs 76.19% (32/42), P>0.05]. Conclusions:Early-onset ANA-positive JIA was more common in female children, and asymmetric knee joint involvement was the most common clinical manifestation. The incidence of ophthalmic complications was high, and ophthalmological examination should be performed more frequently during follow-up. The prognosis of early-onset ANA-positive JIA was good with early treatment. Positive ANA was not a risk factor for poor prognosis.

Advances in cytokines in systemic juvenile idiopathic arthritis / 国际儿科学杂志

With the development of economy and medicine, chronic diseases have become a more and more prominent problem affecting children′s physical and mental development.Juvenile idiopathic arthritis(JIA)is a common rheumatic disease in children.As an important medium of immune response, cytokines play a role in systemic juvenile idiopathic arthritis(SJIA)and its complication such as macrophage activation syndrome(MAS), which has attracted more and more attention.Many studies have found that a variety of cytokines play an inflammatory or anti-inflammatory role in the occurrence and development of SJIA and MAS.Therefore, this paper summarizes the specific mechanism of action and clinical significance of related cytokines in SJIA and MAS, and reviews the progress of related drug therapy targeting cytokines.By exploring the pathogenesis of SJIA, we hope to provide theoretical basis for identifying new therapeutic targets.

Advances in neutrophil extracellular traps in systemic juvenile idiopathic arthritis / 中华实用儿科临床杂志

Systemic juvenile idiopathic arthritis (sJIA) is an autoinflammatory disease characterized by recurrent unprovoked systemic inflammation, activation of the innate immune system, without the generation of autoantibodies or autoreactive antigen-specific T cells.Its pathogenesis is still unclear, and clinical diagnosis and treatment are challenging.Neutrophil extracellular traps (NETs) derived from the innate immune system are a novel neutrophil defense mechanism, and their role in sJIA has been increasingly concerned.In order to further research the pathogenesis of sJIA and to find better clinical diagnosis and treatment methods, this article reviews the involvement of neutrophils and NETs in sJIA, the pathogenesis, relevant biomarkers, potential therapeutic targets and drugs.

Does the use of panoramic radiography add information in the temporomandibular joint evaluation in Juvenile Idiopathic Arthritis patients? A case control study

Abstract Objective To determine the frequency of radiographic changes in the temporomandibular joint, in a representative population of patients with Juvenile Idiopathic Arthritis (JIA) and to compare with findings in healthy controls matched by sex and age. Patients and Methods One hundred and thirty-seven panoramic radiographies (PR) from JIA patients of a pediatric rheumatology outpatient clinic were prospectively evaluated and compared to 137 PR from healthy individuals. Results 102 (74.5%) JIA patients and 47 (34.3%) controls showed at least one radiological alteration (p < 0.001). The following radiographic alterations were more frequently observed in JIA patients than in controls: erosion (p < 0.001), altered condylar morphology (p < 0.001), disproportion between condylar process and the coronoid process (p < 0.001) and accentuated curve in the antegonial notch (p = 0.002). Twenty patients (14.6%) presented the four radiographic alterations simultaneously compared to only two controls (1.5%) (p < 0.001). Conclusion Due to the difference in the frequency of findings in the PR of patients and controls, we concluded that PR has value as a screening tool. In the presence of major changes in the mandible head in the PR of patients with a confirmed diagnosis of JIA, MRI should be considered to detect an active inflammatory process in this joint.

Could ultrasound and muscle elastography be associated with clinical assessment, laboratory and nailfold capillaroscopy in juvenile dermatomyositis patients?

Abstract Background Juvenile Dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in children. Imaging exams are useful for muscle assessment, with ultrasonography (US) being a promising tool in detecting disease activity and tissue damage. There are few studies about muscle elastography. Objectives Our aim was to associate clinical, laboratory, and nailfold capillaroscopy (NC) assessments with US in JDM patients; and to compare the findings of US and Strain Elastography (SE) from patients and healthy controls. Methods An analytic cross-sectional study was performed with JDM patients and healthy controls. Patients underwent clinical exam to access muscle strength and completed questionnaires about global assessment of the disease and functional capacity. Patients were submitted to NC and measurement of muscle enzymes. All subjects underwent US assessment, using gray scale, Power Doppler (PD), and SE. Results Twenty-two JDM patients and fourteen controls, aged between 5 and 21 years, matched for age and sex were assessed. In qualitative and semi-quantitative gray scale, we observed a higher frequency of alterations in patients (p < 0.001), while in PD, there was a higher frequency of positivity in patients' deltoids and anterior tibialis (p < 0.001). Active disease was associated with an important change in the semi-quantitative gray scale in deltoids (p = 0.007), biceps brachii (p = 0.001) and quadriceps femoris (p = 0.005). The SE demonstrated a high negative predictive value of 87.2. Conclusion US was able, through gray scale, to differentiate JDM patients from controls, while PD achieved such differentiation only for deltoids and anterior tibialis. The semi-quantitative gray scale showed disease activity in proximal muscles. SE was not able to differentiate patients from controls.

Enfermedad de Scheuermann lumbar atípica en rara asociación con artritis idiopática juvenil / Atypical lumbar Scheuermann disease and juvenile idiopathic arthritis: a rarely association

El dolor lumbar en los adolescentes es causa frecuente de motivo de consulta en reumatología y obedece a diferentes causas. Se presenta un caso clínico de un adolescente de 14 años de edad, de procedencia rural que acudió a consulta refiriendo dolor y aumento de volumen de ambas rodillas de 3 meses de evolución, acompañado de dolor lumbar desde hacía más de 2 años y que había requerido tratamiento con antinflamatorios no esteroideos y reposo, sin otros síntomas sistémicos acompañantes. Al examen físico se encontró artritis de rodillas, aumento de la cifosis fisiológica en la columna dorsal y puntos sacroilíacos positivos. En los exámenes complementarios fue significativa la presencia del HLA-B27, sinovitis en bolsa subcuadricipital bilateral detectada mediante ultrasonido de rodillas, así como hallazgos en las radiografías a nivel de los cuerpos de las vértebras lumbares característicos de la enfermedad de Scheuermann, y esclerosis de ambas sacroilíacas, características de artritis idiopática juvenil. Se concluyó que el paciente padecía de dos afecciones que por mecanismos diferentes causan dolor lumbar(AU)

Low back pain in adolescents is a frequent reason for consultation in rheumatology and is due to different causes. A clinical case of a 14-year-old adolescent from rural origin who comes to the clinic reporting pain and volume increase in both knees of three months of evolution accompanied by low back pain of more than two years of evolution that had required treatment is presented. with non-steroidal anti-inflammatory drugs and rest, without other accompanying systemic symptoms, physical examination revealed knee arthritis, increased physiological kyphosis in the thoracic spine and positive sacroiliac points. In the complementary tests, the presence of HLA-B27, synovitis in the bilateral sub quadriceps bursa on ultrasound of the knees, findings in the radiographs at the level of the bodies of the lumbar vertebrae characteristic of Scheuermann's disease, and sclerosis of both sacroiliacs' characteristic of juvenile idiopathic arthritis, it is concluded that the patient suffers from two conditions, which by different mechanisms cause low back pain(AU)

Esclerodermia localizada en escolar de 10 años / Localized scleroderma in a 10-year-old schoolgirl

Dentro del grupo de enfermedades reumáticas la esclerodermia es una de las de menor frecuencia de presentación, por lo que muchos autores la consideran una enfermedad rara. Aunque afecta predominantemente a pacientes adultos, en ocasiones se presenta en edades pediátricas y sus formas localizadas son las manifestaciones más frecuentes a estas edades. El objetivo del presente reporte es presentar el caso de una escolar de 10 años de edad, con un cuadro de lesión en la piel de 3 años de evolución a la cual se le diagnostica, mediante las características clínicas y los resultados de estudios anatomopatológicos una esclerodermia localizada profunda. En la actualidad la paciente se mantiene en régimen de seguimiento multidisciplinario. Este reporte de caso es importante para compartir con la comunidad médica los elementos básicos relacionados con el diagnóstico y tratamiento de esta enfermedad, como alternativa a la reducción de las complicaciones que genera(AU)

Within the group of rheumatic diseases, scleroderma is one of those with the lowest frequency of presentation; being considered a rare disease by many authors. Although it has a predominance of affectation in adult patients, it sometimes occurs in pediatric ages, its localized forms being the most frequent forms of presentation. The objective of this report is to present the case of a 10-year-old schoolgirl, with a 3-year history of skin lesions, which was diagnosed, through clinical characteristics and results of pathological studies, as deep localized scleroderma. The case report is considered important to share with the medical community the basic elements related to the diagnosis and treatment of this disease, as an alternative to reducing the complications it generates(AU)

Optic neuromyelitis in relation with juvenile idiopathic arthritis: A case report

Optic neuromyelitis (ONM), also called neuromyelitis optica spectrum (Neuromyelitis Optica Spectrum Disorders, NMOSD) is recognized as an inflammatory autoimmune demyelinating disease of the central nervous system, mediated by autoantibodies against the aquaporin-4 receptor (AQP4-IgG). It predominantly affects the optic nerves and the spinal cord.1-3 It is known that patients with immune disorders are more likely to present other autoimmune diseases, but the relation between juvenile idiopathic arthritis and ONM has not been completely described.5 In this paper, we report a case of a patient with juvenile idiopathic arthritis, presenting with a rapidly progressive neurological condition, who is treated with biological drugs.1-4

La neuromielitis óptica (NMO), también llamada espectro de la neuromielitis óptica (neuromyelitis optica spectrum disorders) se reconoce como una enfermedad inflamatoria, autoinmune, desmielinizante del sistema nervioso central, mediada por autoanticuerpos contra el receptor de acuaporina 4 (AQP4-IgG) que afecta predominantemente a los nervios ópticos y la médula espinal1-3. Es conocido que los pacientes con trastornos inmunitarios tienen más probabilidades de presentar otras enfermedades autoinmunes; sin embargo, no está completamente descrita la asociación entre artritis idiopática juvenil y NMO5. En este escrito se reporta el caso de una paciente que cursa con artritis idiopática juvenil, inició con compromiso neurológico rápidamente progresivo, y es tratada con medicamentos biológicos1-4.