Presentation of enthesitis-related arthritis and juvenile-onset spondyloarthritis: a cross-sectional study in a pediatric and adult clinic.

BACKGROUND: Juvenile idiopathic arthritis (JIA) comprises a whole spectrum of chronic arthritis starting before 16 years of age. The study aims to explore the clinical and demographic descriptors, treatment, and disease progression of enthesitis-related arthritis (ERA) in comparison with juvenile-onset spondyloarthritis (SpA). METHODS: Cross-sectional analysis of consecutive patients in two dedicated clinics, with a single visit and retrospective case-notes review. Arthritis, enthesitis and sacroiliitis were evaluated by scoring disease activity and damage. Continuous variables were reported by median, interquartile range; categorical variables were reported by the frequency comparison of the two groups. RESULTS: Thirty-three cases were included, being 23 (69.7%) with ERA. The median age at diagnosis was 12.5 y (SpA) vs. 9 y (ERA) (p < 0.01); the time from symptom onset to diagnosis was 5.5 y (SpA) vs. 1.5 y (ERA) (p < 0.03). In both groups, the predominant presentation was a single joint or < 5 lower limb joints and asymmetric involvement, with a high frequency of enthesitis. There was a higher frequency of mid-tarsal and ankle synovitis in the ERA group and hip involvement in those with SpA. The comparison of the frequency of spine symptoms at presentation, 30% SpA vs. 21.7% ERA (p = 0.7), was not significant, and radiographic progression to spinal involvement occurred in 43.5% of ERA patients. The median time for spinal progression and age at onset was 2.2 and 12 y for ERA, and 4 and 16.5 y for SpA, respectively. Activity and damage scores were not significantly different between the groups. Treatment comparison resulted in 91.3% of ERA and 100% SpA being treated, predominantly with NSAIDs in both groups, followed by DMARDs and biologics, with a higher frequency of biologics in SpA. CONCLUSION: The main differences were the late diagnoses of SpA, and the hip and spine involvement, with higher frequency of biologic treatment in juvenile-onset SpA compared to ERA.

Rhupus syndrome in the pediatric population: A comprehensive systematic literature review.

INTRODUCTION: The term "Rhupus" was employed to descriptively illustrate the overlap observed in some pediatric patients displaying features of both juvenile idiopathic arthritis (JIA) and systemic lupus erythematosus (SLE). Although "Rhupus" is traditionally used in adults, we applied it broadly to emphasize this clinical overlap. METHODS: We sought to identify studies that registered signs, symptoms, imaging characteristics, and treatments given to patients with JIA and SLE. We searched four databases using a Boolean search string, resulting in 231 articles after duplicate removal. Title and abstract screening yielded 57 articles for full-text assessment. Full reviewed 13 extracted data regarding sex, age of onset, serologic and imaging findings, and management strategies. The NIH quality assessment tool was applied to ensure the internal validity of the articles. RESULTS: From the 13 articles evaluated that meet inclusion criteria, none had standardized diagnostic algorithms. The total number of patients in those articles is 26, without discussing treatment guidelines. DISCUSSION: Clinical presentation, diagnostic parameters, and treatment of pediatric Rhupus were synthesized in this review. Fundamental keys help distinguish the joint presentation when Juvenile Idiopathic Arthritis or Lupus is present, compared with the signs and symptoms when developing the overlapping syndrome. We highlight the importance of physicians knowing about this rare condition and call all specialists to report new cases of the disease so a consensus can be reached to establish standardized guidelines for diagnosing and treating Rhupus syndrome.

Inflammatory Biomarkers Can Differentiate Acute Lymphoblastic Leukemia with Arthropathy from Juvenile Idiopathic Arthritis Better Than Standard Blood Tests.

OBJECTIVE: To evaluate the predictive value of biomarkers of inflammation like phagocyte-related S100 proteins and a panel of inflammatory cytokines in order to differentiate the child with acute lymphoblastic leukemia (ALL) from juvenile idiopathic arthritis (JIA). STUDY DESIGN: In this cross-sectional study, we measured S100A9, S100A12, and 14 cytokines in serum from children with ALL (n = 150, including 27 with arthropathy) and JIA (n = 236). We constructed predictive models computing areas under the curve (AUC) as well as predicted probabilities in order to differentiate ALL from JIA. Logistic regression was used for predictions of ALL risk, considering the markers as the respective exposures. We performed internal validation using repeated 10-fold cross-validation and recalibration, adjusted for age. RESULTS: In ALL, the levels of S100A9, S100A12, interleukin (IL)-1 beta, IL-4, IL-13, IL-17, matrix metalloproteinase-3, and myeloperoxidase were low compared with JIA (P < .001). IL-13 had an AUC of 100% (95% CI 100%-100%) due to no overlap between the serum levels in the 2 groups. Further, IL-4 and S100A9 had high predictive performance with AUCs of 99% (95% CI 97%-100%) and 98% (95% CI 94%-99%), respectively, exceeding both hemoglobin, platelets, C-reactive protein, and erythrocyte sedimentation rate. CONCLUSIONS: The biomarkers S100A9, IL-4, and IL-13 might be valuable markers to differentiate ALL from JIA.

Diagnosis of juvenile idiopathic arthritis with asymptomatic involvement of the temporomandibular joint: A case report.

Juvenile idiopathic arthritis (JIA) is a broad term that describes a group of heterogeneous rheumatologic diseases, mainly characterized by inflammation in the joints of children and young people up to 16 years of age. Its etiology is still not well understood and the diagnosis, essentially clinical, begins with the exclusion of other joint diseases. When the disease affects the temporomandibular joint, diagnosis is a challenge, as many patients are asymptomatic. The objective of this paper is to present a case of JIA with severe involvement of the temporomandibular joint and to discuss the clinical, radiographic, laboratory findings and the importance of early diagnosis. The lack of diagnosis of active arthritis in the temporomandibular joint in patients with JIA can cause irreversible effects such as micrognathia, malocclusion and reduced maximum mouth opening. Early diagnosis of temporomandibular joint involvement in JIA is important and needs to be investigated early in the clinical manifestation of systemic disease. Laboratory tests and clinical history are important to define treatment and prognosis, but not to predict temporomandibular joint arthritis. Imaging exams are important diagnostic tools to identify morphological changes in soft and hard tissues of the temporomandibular joint.

Development and Initial Validation of the ONCOREUM Score to Differentiate Childhood Cancer with Arthropathy from Juvenile Idiopathic Arthritis.

OBJECTIVE: To develop and validate a weighted score, the ONCOREUM score, that aids physicians in differentiation of cancer with arthropathy from juvenile idiopathic arthritis (JIA). STUDY DESIGN: Data were extracted from the ONCOREUM Study, a multicenter, cross-sectional investigation aimed at comparing children with cancer and arthropathy to children with JIA. Three statistical approaches were applied to develop the ONCOREUM score and assess the role of each variable in the diagnosis of cancer with arthropathy, including 2 approaches based on multivariable stepwise selection (models 1 and 2) and 1 approach on a Bayesian model averaging method (model 3). The ß coefficients estimated in the models were used to assign score points. Considering that not missing a child with cancer is a mandatory clinical objective, discriminating performance was assessed by fixing sensitivity at 100%. Score performance was evaluated in both developmental and validation samples (representing 80% and 20% of the study population, respectively). RESULTS: Patients with cancer and arthropathy (49 with solid tumors and 46 with hematologic malignancies without peripheral blasts) and 677 patients with JIA were included. The highest area under the receiver operating characteristic (ROC) curve (AUC) in the validation data set was yielded by model 1, which was selected to constitute the ONCOREUM score. The score ranged from -18 to 21.8, and the optimal cutoff obtained through ROC analysis was -6. The sensitivity, specificity, and AUC of the cutoff in the validation sample were 100%, 70%, and 0.85, respectively. CONCLUSIONS: The ONCOREUM score is a powerful and easily applicable tool that may facilitate early differentiation of malignancies with articular complaints from JIA.

Therapeutic plasma exchange in refractory macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a case-based review.

Juvenile idiopathic arthritis (JIA) is a heterogeneous group of arthritis of autoimmune aetiology. Systemic-onset juvenile idiopathic arthritis (soJIA) presents with fever, transient erythematous rash, hepatomegaly, splenomegaly, lymphadenopathy, and serositis. SoJIA presents multiple complications, and the most severe is the macrophage activation syndrome (MAS); the timely treatment of MAS must be established early and aggressively to avoid a fatal outcome. Therapeutic plasma exchange has anecdotally been used in refractory cases. A 66-month-old male with a 1-year illness characterized by evening-predominant, intermittent fever, adenomegalies, urticarial-like rash, arthralgia, and arthritis. Biochemical analysis revealed anaemia, leukocytosis, neutrophilia, hypertriglyceridemia, hyperferritinemia, and hypofibrinogenemia; bone marrow aspirate showed hemophagocytosis. He was diagnosed with SoJIA complicated with MAS. He received multiple treatments with IV human gammaglobulin, cyclosporine, dexamethasone, and tocilizumab without improvement. Plasma replacement treatment was performed. Afterwards, he presented significant improvement. After 3-year-follow-up, he remains in good general condition. We present a refractory case of soJIA complicated with MAS successfully treated with plasma exchange.

Multiple Sclerosis in a Patient with Intermediate Uveitis and Juvenile Idiopathic Arthritis Treated with Adalimumab: A Case Report.

PURPOSE: To report a case of multiple sclerosis (MS) development in a patient with Juvenile Idiopathic Arthritis (JIA) and bilateral intermediate uveitis (IU) treated with Adalimumab. CASE REPORT: A 21-year-old Colombian woman diagnosed with JIA and bilateral refractory IU treated with methotrexate and Adalimumab with difficult control of the disease and multiple ocular complications. Eight years after starting Adalimumab, the patient presented paresthesia in the left upper limb. Radiologic findings in the brain and cervical spine MRI confirmed the diagnosis of MS. CONCLUSIONS: We reported the first case of MS development in a patient with JIA treated with Adalimumab and the third in a patient with noninfectious uveitis treated with anti-TNFα. It remains uncertain whether MS is secondary to anti-TNFα therapy or is linked to a polyautoimmunity phenomenon.

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)

Afectación articular en adolescente con artritis idiopática juvenil / Joint involvement in an adolescent with juvenile idiopathic arthritis

La artritis idiopática juvenil es una enfermedad inflamatoria sistémica y crónica que se caracteriza por el daño articular y la presencia de manifestaciones extraarticulares que afectan distintos órganos y sistemas de órganos del cuerpo humano. Como enfermedad tiene varias formas clínicas de presentación que se corresponden con posibles enfermedades en la edad adulta. El objetivo de la presente investigación es presentar el caso de un adolescente de 14 años de edad con historia de cuadro inflamatorio poliarticular de más de 3 años de duración con deformidad articular en ambas rodillas, lo cual es poco frecuente y que es expresión del proceso inflamatorio mantenido. Después del tratamiento fue dado de alta con una mejoría notable de los rangos de movimiento articular. En la actualidad evoluciona satisfactoriamente y lleva alrededor de un año en seguimiento en consulta externa sin exacerbaciones de la actividad clínica de la enfermedad. Se considera importante el reporte del caso para concientizar a la comunidad médica en relación con el diagnóstico precoz de esta enfermedad para minimizar el riesgo de aparición de complicaciones articulares y sistémicas(AU)

Juvenile idiopathic arthritis is a systemic and chronic inflammatory disease characterized by joint involvement and the presence of extra-articular manifestations that occur in different organs and organ systems of the human body. As a disease, it includes a series of clinical forms of presentation that correspond to possible diseases in adulthood. The objective of this research is to present the case of a 14-year-old adolescent with a history of polyarticular inflammatory symptoms lasting more than three years with the presence of rare joint deformity in both knees, which is an expression of the sustained inflammatory process. The case report is considered important to raise awareness in the medical community regarding the early diagnosis of this disease to minimize the risk of the appearance of joint and systemic complications(AU)

[Role of Hematological Parameters in the Diagnosis of Juvenile Idiopathic Arthritis in Children with Arthritis]. / Papel de los parámetros hematológicos en el diagnóstico de Artritis Idiopatica Juvenil en niños con artritis.

INTRODUCTION: Early diagnosis and treatment of arthritis are essential for the prognosis of the disease. Especially during the active phase of juvenile idiopathic arthritis (JIA), a prompt diagnosis is necessary to ma nage the disease properly. New inflammation markers such as neutrophil-lymphocyte ratio (NLR), monocyte-lymphocyte ratio (MLR), mean platelet volume (MPV), and platelet distribution width (PDW) have been investigated in various inflammatory disorders. This study aimed at the diagnostic value of NLR, MLR, MPV, and PDW in differentiating JIA in children with arthritis. PATIENTS AND METHOD: Case-control study with 324 children with arthritis (case group) and 324 healthy children (control group). Additionally, children with arthritis were grouped into JIA and non-JIA. Medical records of children aged 0-18 were retrospectively reviewed. Hematological parameters at the time of diagnosis were recorded. NLR, MLR, MPV, and PDW were analyzed in the study groups. RESULTS: In the case group, 52.8% were boys, and 47.2% were girls; the mean age was 7.7 ± 4.0 years. The NLR in the case group was significantly higher than the control one (p = 0.001). The mean MPV was lower in the case group than the control group (p = 0.001). There were no differences in NLR and MPV between JIA and non-JIA groups (p = 0.062, p = 0.689). The JIA group's mean PDW was lower than the non-JIA group (p = 0.001). CONCLUSION: The increase in NLR may indicate inflammation but has no superiority in distinguishing JIA from other arthritis causes. Platelet distribution width was lower in JIA patients, but its clinical utility is limited.

Spotlight on latent tuberculosis infection screening for juvenile idiopathic arthritis in two countries, comparing high and low risk patients.

BACKGROUND: Rheumatic diseases are associated with an increase in overall risks of tuberculosis (TB). The aim of this study was to evaluate the frequency of TB and the frequency of latent TB infection (LTBI), in clinical practice, for juvenile idiopathic arthritis (JIA) patients from high and low risk of TB incidence endemic countries. METHODS: This is an international, multicenter, cross-sectional, observational study of data collection from Brazil and Registry of Portugal at REUMA.PT. The inclusion criteria were patients with Juvenile Idiopathic Arthritis (JIA) with age ≤ 18 years who underwent screening for Mycobacterium tuberculosis infection [tuberculin skin test (TST) and/or interferon gamma release assay (IGRA)]. Chest X-rays and history of exposure to TB were also assessed. RESULTS: 292 JIA patients were included; mean age 14.3 years, mean disease duration 7.5 years, 194 patients (66.4%) performed only TST, 14 (4.8%) only IGRA and 84 (28.8%) both. The frequency of LTBI (10.6%) and TB was similar between the two countries. The reasons for TB screening were different; in Brazil it was performed more often at JIA onset while in Portugal it was performed when starting Disease Modified Anti-Rheumatic Drugs (DMARD) treatment (p < 0.001). Isoniazid therapy was prescribed in 40 (13.7%) patients (31 with LTBI and 9 with epidemiologic risks and/or due to contact with sick people). Only three patients (1%) developed active TB. CONCLUSION: We found nearly 10% of patients with LTBI, a small percentage of patients with treatment due to epidemiologic risks and only 1% with active TB. Distinct reasons and screening methods for LTBI were observed between the two countries.

A Diagnostic Prediction Model for Separating Juvenile Idiopathic Arthritis and Chronic Musculoskeletal Pain Syndrome.

OBJECTIVE: To develop and validate a diagnostic prediction model that can distinguish between juvenile idiopathic arthritis (JIA) and chronic musculoskeletal pain syndrome (CMPS) based on patient-reported outcomes. STUDY DESIGN: This retrospective cohort study evaluated whether the Juvenile Arthritis Multidimensional Assessment Report (JAMAR) performs well in distinguishing JIA from CMPS. We analyzed JAMARs completed by 287 patients at their first visit to the pediatric rheumatology department of Wilhelmina Children's Hospital in Utrecht, The Netherlands. Relevant JAMAR items for predicting a diagnosis of JIA were selected in a penalized multivariable model suitable for clinical application. The model was subsequently validated with new data from the same center. RESULTS: A total of 196 JAMARs (97 JIA, 99 CMPS) were collected in the model development data, and 91 JAMARs (48 JIA, 43 CMPS) were collected in the validation data. Variables in the prediction model that were strongest associated with a diagnosis of JIA instead of CMPS were asymmetric pain/swelling in the shoulder (OR, 2.34), difficulty with self-care (OR, 2.41), skin rash (OR, 2.07), and asymmetric/pain swelling in the knee (OR, 2.29). Calibration and discrimination (area under the receiver operating characteristic curve, 0.83; 95% CI, 0.74-0.92) of the model in the validation data were good. CONCLUSIONS: Several items from the JAMAR questionnaire can potentially distinguish JIA from CMPS in patients with corresponding symptoms. We present an easy-to-use, adjusted, and validated model to separate these 2 diagnoses early at presentation based on patient-reported outcomes to facilitate proper referral and treatment.

Monitoring and Treatment of Juvenile Idiopathic Arthritis-associated Uveitis: Brazilian Evidence-based Practice Guidelines.

PURPOSE: To present a national guideline for ophthalmologic care and surveillance of juvenile idiopathic arthritis-associated uveitis (JIA-uveitis). METHODS: Review article based on medical literature and the experience of an Expert Committee composed of members of the Brazilian Society of Pediatric Ophthalmology/Brazilian Council of Ophthalmology and the Brazilian Society of Pediatrics/Brazilian Society of Rheumatology. Studies with a high level of evidence were selected by searching the PubMed/Medline database. The final document was approved by the experts. RESULTS: The main recommendations are that children/adolescents with JIA should undergo screening according to their risk factors. Ophthalmological checkups should also consider ocular inflammation and therapy. Topical glucocorticoids should be the first line of therapy, with systemic glucocorticoids acting as bridge treatments in severe uveitis. Methotrexate should be the first-line systemic therapy and anti-tumor necrosis factor (anti-TNF alpha) the second for uncontrolled uveitis. CONCLUSIONS: This evidence-based guideline for JIA-uveitis will be useful for both ophthalmology and rheumatology practice.

Enthesitis-related arthritis: monitoring and specific tools.

OBJECTIVES: In this article, the authors aimed to review the different tools used in the monitoring of enthesitis-related arthritis. SOURCES: The authors performed a literature review on PubMed, Google Scholar, and Scopus databases. The dataset included the original research and the reviews including patients with enthesitis-related arthritis or juvenile spondylarthritis up to October 2020. SUMMARY OF FINDING: Enthesitis-related arthritis is a category of juvenile idiopathic arthritis. It is characterized by the presence of enthesitis, peripheral arthritis, as well as axial involvement. The only validated tool for disease activity measurement in juvenile idiopathic arthritis is the Disease Activity Score: It has proven its reliability and sensitivity. Nevertheless, due to an absence of validated evaluation tools, the extent of functional impairment, as well as the children and parents' perception of the disease, could not be objectively perceived. Despite the great progress in the field of imaging modalities, the role they play in the evaluation of disease activity is still controversial. This is partially due to the lack of validated scoring systems. CONCLUSIONS: Further work is still required to standardize the monitoring strategy and validate the outcome measures in enthesitis-related arthritis.

Ferritin levels in children with juvenile idiopathic arthritis of systemic onset and children with other causes of fever of unknown origin: A multicenter study of diagnostic tests / Niveles de ferritina en artritis idiopática juvenil de inicio sistémico comparada con otras fiebres de origen desconocido: estudio multicéntrico de pruebas diagnósticas

Introduction: There are no sensitive or specific tests available to diagnose systemic juvenile idiopathic arthritis (sJIA). Objective: To assess the utility as diagnostic tests of total ferritin (TF) levels greater than 5 times the normal value (TF>5N) and the decreased percentage (less than or equal to 20% of TF) of glycosylated ferritin (GF≤20%) for the diagnosis of sJIA in patients with fever of unknown origin evaluated by pediatric rheumatology. Materials and methods: We conducted an observational, cross-sectional study of diagnostic tests in children under 16 years of age hospitalized between 2010 and 2014. The reference diagnostic standard was the fulfillment of the classification criteria or confirmed diagnosis at follow-up. We determined the measures of utility of the tests. Results: We included 40 patients with fever of unknown origin, 11 with sJIA, and 29 with other diagnoses. The median TF was higher in sAIJ (3992 ng/ml) versus other causes of fever of unknown origin (155 ng/ml) (p=0.0027), as well as TF>5N (90.91% versus 51.72%) (p=0.023). The percentage of GF≤20% was higher in patients with other causes of fever of unknown origin (96.5%) compared to sJIA (81.8%) (p=0.178). TF>5N had a sensitivity of 91%, specificity of 48%, positive likelihood ratio (LR) of 1.76, and negative LR of 0.19 demonstrating greater utility for the diagnosis of sJIA than the combination of FT> 5N with GFR <20%, with a sensitivity of 81.8%, specificity of 48.3%, positive LR of 1.58, and negative LR of 0.38. Conclusion: In patients with FUO evaluated by pediatric rheumatology, TF> 5N proved useful as a screening test for the diagnosis of sJIA.

Introducción. No se dispone de pruebas sensibles ni específicas para diagnosticar la artritis idiopática juvenil sistémica. Objetivo. Evaluar la utilidad diagnóstica de niveles de ferritina total cinco veces por encima del valor normal (ferritina total>5N) y el porcentaje disminuido (menor de o igual a 20 % de la ferritina total) de la ferritina glucosilada (ferritina glucosilada<20 %) para el diagnóstico de artritis idiopática juvenil sistémica en pacientes con fiebre de origen desconocido evaluados por reumatología pediátrica. Materiales y métodos. Se hizo un estudio observacional de pruebas diagnósticas de corte transversal en menores de 16 años hospitalizados entre el 2010 y el 2014. El patrón diagnóstico de referencia fue el cumplimiento de los criterios de clasificación o diagnóstico confirmado en el seguimiento. Se determinaron las medidas de utilidad de las pruebas. Resultados. Se incluyeron 40 pacientes con fiebre de origen desconocido: 11 con artritis idiopática juvenil sistémica y 29 con otros diagnósticos. La mediana de la ferritina total fue mayor en la artritis idiopática juvenil sistémica (3.992 ng/ml) comparada con otras causas de fiebre de origen desconocido (155 ng/ml) (p=0,0027), así como la ferritina total>5N (90,91 % Vs. 51,72 %) (p=0,023). El porcentaje de ferritina glucosilada≤20 % fue de 96,5 % en otras fiebres de origen desconocido en comparación con la artritis idiopática juvenil sistémica (81,8 %) (p=0,178). La ferritina total>5N tuvo una sensibilidad del 91 %, una especificidad del 48 %; un cociente de probabilidades (Likelihood Ratio, LR) positivo de 1,76 y uno negativo de 0,19, demostrando mayor utilidad para el diagnóstico que la combinación de la ferritina total>5N y ferritina glucosilada≤20 %, cuya sensibilidad fue del 81,8 %, la especificidad del 48,3 %, un cociente de probabilidades LR positivo de 1,58 y un LR negativo de 0,38. Conclusión. En pacientes con fiebre de origen desconocido evaluados por reumatología pediátrica, la ferritina total>5N demostró ser útil como prueba de tamización para el diagnóstico de artritis idiopática juvenil sistémica.

Niveles de ferritina en artritis idiopática juvenil de inicio sistémico comparada con otras fiebres de origen desconocido: estudio multicéntrico de pruebas diagnósticas / Ferritin levels in children with juvenile idiopathic arthritis of systemic onset and children with other causes of fever of unknown origin: A multicenter study of diagnostic tests

Resumen | Introducción. No se dispone de pruebas sensibles ni específicas para diagnosticar la artritis idiopática juvenil sistémica. Objetivo. Evaluar la utilidad diagnóstica de niveles de ferritina total cinco veces por encima del valor normal (ferritina total>5N) y el porcentaje disminuido (menor de o igual a 20 % de la ferritina total) de la ferritina glucosilada (ferritina glucosilada<20 %) para el diagnóstico de artritis idiopática juvenil sistémica en pacientes con fiebre de origen desconocido evaluados por reumatología pediátrica. Materiales y métodos. Se hizo un estudio observacional de pruebas diagnósticas de corte transversal en menores de 16 años hospitalizados entre el 2010 y el 2014. El patrón diagnóstico de referencia fue el cumplimiento de los criterios de clasificación o diagnóstico confirmado en el seguimiento. Se determinaron las medidas de utilidad de las pruebas. Resultados. Se incluyeron 40 pacientes con fiebre de origen desconocido: 11 con artritis idiopática juvenil sistémica y 29 con otros diagnósticos. La mediana de la ferritina total fue mayor en la artritis idiopática juvenil sistémica (3.992 ng/ml) comparada con otras causas de fiebre de origen desconocido (155 ng/ml) (p=0,0027), así como la ferritina total>5N (90,91 % Vs. 51,72 %) (p=0,023). El porcentaje de ferritina glucosilada≤20 % fue de 96,5 % en otras fiebres de origen desconocido en comparación con la artritis idiopática juvenil sistémica (81,8 %) (p=0,178). La ferritina total>5N tuvo una sensibilidad del 91 %, una especificidad del 48 %; un cociente de probabilidades (Likelihood Ratio, LR) positivo de 1,76 y uno negativo de 0,19, demostrando mayor utilidad para el diagnóstico que la combinación de la ferritina total>5N y ferritina glucosilada≤20 %, cuya sensibilidad fue del 81,8 %, la especificidad del 48,3 %, un cociente de probabilidades LR positivo de 1,58 y un LR negativo de 0,38. Conclusión. En pacientes con fiebre de origen desconocido evaluados por reumatología pediátrica, la ferritina total>5N demostró ser útil como prueba de tamización para el diagnóstico de artritis idiopática juvenil sistémica.

Abstract | Introduction: There are no sensitive or specific tests available to diagnose systemic juvenile idiopathic arthritis (sJIA). Objective: To assess the utility as diagnostic tests of total ferritin (TF) levels greater than 5 times the normal value (TF>5N) and the decreased percentage (less than or equal to 20% of TF) of glycosylated ferritin (GF≤20%) for the diagnosis of sJIA in patients with fever of unknown origin evaluated by pediatric rheumatology. Materials and methods: We conducted an observational, cross-sectional study of diagnostic tests in children under 16 years of age hospitalized between 2010 and 2014. The reference diagnostic standard was the fulfillment of the classification criteria or confirmed diagnosis at follow-up. We determined the measures of utility of the tests. Results: We included 40 patients with fever of unknown origin, 11 with sJIA, and 29 with other diagnoses. The median TF was higher in sAIJ (3992 ng/ml) versus other causes of fever of unknown origin (155 ng/ml) (p=0.0027), as well as TF>5N (90.91% versus 51.72%) (p=0.023). The percentage of GF≤20% was higher in patients with other causes of fever of unknown origin (96.5%) compared to sJIA (81.8%) (p=0.178). TF>5N had a sensitivity of 91%, specificity of 48%, positive likelihood ratio (LR) of 1.76, and negative LR of 0.19 demonstrating greater utility for the diagnosis of sJIA than the combination of FT> 5N with GFR <20%, with a sensitivity of 81.8%, specificity of 48.3%, positive LR of 1.58, and negative LR of 0.38. Conclusion: In patients with FUO evaluated by pediatric rheumatology, TF> 5N proved useful as a screening test for the diagnosis of sJIA.

[Quality of life and disease activity assessment in juvenile idiopathic arthritis]. / Evaluación de la calidad de vida y la actividad de la enfermedad en artritis idiopática juvenil.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatological disease in children. It is a multisystemic, dynamic pathophysiology of unknown cause and genetically heterogeneous. OBJECTIVE: To compare the quality of life and illness' activity in patients with juvenile idiopathic arthritis, from urban, suburban and rural areas. MATERIAL AND METHODS: Comparative, observational, and cross-sectional study in pediatric patients treated in a second-level medical care hospital, during the period from August to October 2015. The following questionnaires were applied: The Childhood Health Assessment Questionnaire for Quality of Life and The Disease Activity Score-28, in addition to the facial pain and verbal numerical scales for disease activity, both for patients and parents. The Kruskal-Wallis test was used for data analysis. RESULTS: 42 patients of three geostatistical areas were included: urban, suburban and rural; the middle age was 12.7 years, with predominance of the feminine sex (3:1). The polyarticulate type is more prevalent in the rural area. The pain is similar in the three regions. The population of the areas did not find significant difference for activity of the illness and quality of life between geostatistical areas (p ≤ 0.05). CONCLUSIONS: The patients with juvenile idiopathic arthritis are usually older of eight years. The quality of life is better in the patients of the urban area, compared to those of other areas.

INTRODUCCIÓN: la artritis idiopática juvenil (AIJ) es la enfermedad reumatológica crónica más frecuente en niños. Es una fisiopatología multisistémica, dinámica, de causa desconocida y genéticamente heterogénea. OBJETIVO: comparar la calidad de vida y la actividad de la enfermedad en pacientes con artritis idiopática juvenil, originarios de áreas urbanas, suburbanas y rurales. MATERIAL Y MÉTODOS: estudio comparativo, observacional y transversal, en pacientes pediátricos atendidos en un hospital de segundo nivel de atención médica, durante el periodo de agosto a octubre de 2015. Se aplicaron los cuestionarios: Childhood Health Assessment Questionnaire para calidad de vida y Disease Activity Score-28, además de las escalas facial del dolor y verbal numérica para actividad de la enfermedad, tanto a los pacientes como a los padres. Se utilizó la prueba de Kruskal-Wallis para el análisis de los datos. RESULTADOS: se incluyeron 42 pacientes de tres áreas geoestadísticas: urbana, suburbana y rural; la edad media fue de 12.7 años, con predominio del sexo femenino (3:1). El tipo de clasificación poliarticular es el más prevalente en el área rural. El dolor es similar en las tres regiones. No se encontró diferencia significativa para actividad de la enfermedad y calidad de vida entre la población de las áreas geoestadísticas (p ≤ 0.05). CONCLUSIONES: los pacientes con artritis idiopática juvenil suelen ser mayores de ocho años. La calidad de vida es mejor en los pacientes del área urbana, en comparación con los de las otras áreas.