Enfermedad de Crohn en paciente con artritis idiopática juvenil de inicio sistémico: ¿asociación o complicación asociada al tratamiento? / Crohn's Disease in a Patient with Systemic Onset Juvenile Idiopathic Arthritis. Association or Associated Side Effect of Treatment?

La evolución de la artritis idiopática juvenil de inicio sistémico (AIJs) hacia las diferentes formas de presentación de enfermedad inflamatoria intestinal es extremadamente infrecuente. Presentamos la que, hasta ahora, es la primera comunicación de un paciente con AIJs con evolución a enfermedad de Crohn en el que se han detectado mutaciones en genes responsables de la adecuada regulación del sistema inmune innato.(AU)

The progression of systemic-onset juvenile idiopathic arthritis (JIAs) to the different forms of presentation of inflammatory bowel disease is extremely rare. We present the first report of a patient with SJIA that progressed to Crohn's disease in which mutations have been detected in genes responsible for the adequate regulation of the innate immune system.(AU)

Efectividad y seguridad de los bisfosfonatos en el tratamiento de la osteoporosis infantil secundaria / Effectiveness and safety of bisphosphonates therapy in secondary osteoporosis in children

Introducción: Los estudios sobre efectividad y seguridad de los bisfosfonatos en osteoporosis infantil secundaria (OIS) son escasos. El objetivo fue analizar efectividad y seguridad de los bisfosfonatos en OIS. Pacientes y métodos: Estudio multicéntrico retrospectivo en <18 años afectos de OIS tratados con bisfosfonatos. Se recogieron variables clínicas. Se valoró densidad mineral ósea mediante el Z-score de densidad mineral ósea en columna lumbar (ZDMOcl) medido por absorciometría de rayos X de doble energía (DXA). Valoramos efectividad en función del cambio del ZDMOcl al año y a los dos años de su inicio y del descenso del número de fracturas/año. Los eventos adversos reportados fueron recogidos. Se realizó análisis descriptivo y bivariante. Resultados: Se reclutaron 32 pacientes. El ZDMOcl se incrementó al año del inicio del tratamiento ([-2,46±0,96] vs. [-1,54±1,38]; p<0,001). El número de fracturas/año disminuyó significativamente (1 [1-2] vs. 0 [0-0,61]; p<0,001). El cambio en el ZDMOcl fue mayor en los pacientes deambulantes (1,88+/- 0,72 vs. 0,55+/-0,82; p=0,07) y se correlacionó positivamente con el percentil del IMC (rho:0,564; p<0,001). El descenso del número de fracturas/año fue mayor en los pacientes con menor tasa inicial de fracturas (rho:-0,47; p=0,006) y cuanto mayor era el Z-score inicial (rho:-0,47; p=0,07). Se reportaron 10 eventos adversos leves en 7 pacientes (22%), todos con bisfosfonatos intravenosos. No se halló relación entre eventos adversos y las variables estudiadas. Conclusiones: Los bisfosfonatos son efectivos en OIS. La respuesta parece ser mejor en pacientes deambulantes, bien nutridos y en estadios precoces de la enfermedad. Resultan seguros, siendo los efectos adversos leves, aunque frecuentes. (AU)

Introduction: There are few studies on effectiveness and safety of bisphosphonate therapy in secondary osteoporosis in children. The aim of this research was to analyse effectiveness and safety of bisphosphonates in secondary osteoporosis in children. Patients and methods: Multicentre retrospective study in patients younger than 18 suffering from secondary osteoporosis and who had received bisphosphonates. Clinical data were recorded. Bone mineral density was assessed in terms of bone mineral density Z-score in lumbar spine (ZBMDls) measured by dual-energy X-ray absorptiometry (DXA). Effectiveness was valued at changes in ZBMDls one and two years after the onset of bisphosphonates and at the decrease in the number of fractures a year. Adverse events reported were recorded. Descriptive and bivariant analysis were performed. Results: 32 patients were recruited. ZBMDls increased one year after the onset of treatment ([−2.46±0.96] vs. [−1.54±1.38]; p<.001). Fractures a year decreased significantly (1 [1–2] vs. 0 [0–0.61]; p<,001). ZBMDls increase was higher in patients who were able to walk (1.88±0.72 vs. 0.55±0.82; p=.07) and correlated positively with body mass index (BMI) for age percentile (rho: 0.564; p<.001). The decrease in the number of fractures a year was higher in patients with lower initial fracture rate (rho: −0.47; p=.006) and with higher initial ZBMDls (rho: −0.47; p=.07). 10 adverse events were reported in 7 patients (22%), all of them intravenous bisphosphonates related. No association was found between adverse events and studied variables. Conclusions: Bisphosphonates are effective in secondary osteoporosis in children. Response seems to be better in patients who are able to walk, well-nourished and in the early stages of the disease. Adverse events were frequent but mild. (AU)

Effectiveness and safety of bisphosphonates therapy in secondary osteoporosis in children.

INTRODUCTION: There are few studies on effectiveness and safety of bisphosphonate therapy in secondary osteoporosis in children. The aim of this research was to analyse effectiveness and safety of bisphosphonates in secondary osteoporosis in children. PATIENTS AND METHODS: Multicentre retrospective study in patients younger than 18 suffering from secondary osteoporosis and who have received bisphosphonates. Clinical data were recorded. Bone mineral density (BMD) was assessed in terms of BMD Z-score in lumbar spine (ZBMDls) measured by dual-energy X-ray absorptiometry (DXA). Effectiveness was valued at changes in ZBMDls one and two years after the onset of bisphosphonates and at the decrese in the number of fractures a year. Adverse events reported were recorded. Descriptive and bivariant analysis was performed. RESULTS: 32 patients were recruited. ZBMDls increased one year after the onset of treatment ((-2.46 ± 0.96) vs. (-1.54 ± 1.38); p < .001). Fractures a year dicreased significantly (1 (1-2) vs. 0 (0-0.61); p < .001). ZBMDls increase was higher in patients who were able to walk (1.88 ± 0.72 vs. 0.55 ± 0.82; p = .07) and correlated positively with body mass index (BMI)- for- age percentile (rho: 0.564; p < .001). The decrease in the number of fractures a year was higher in patients with lower initial fracture rate (rho: -0,47; p = .006) and with higher initial ZBMDls (rho: -0.47; p = .07). 10 adverse events were reported in 7 patients (22%), all of them intravenous bisphosphonates related. No association was found between adverse events and studied variables. CONCLUSIONS: Bisphosphonates are effective in secondary osteoporosis in children. Response seems to be better in patients who are able to walk, well-nourished and in the early stages of the disease. Adverse events were frequent but mild.

Longitudinal Study of Cognitive Functioning in Adults with Juvenile Idiopathic Arthritis.

OBJECTIVE: To prospectively evaluate possible decline of cognitive functions in adult patients with juvenile idiopathic arthritis (JIA) and identify associated factors. PATIENTS AND METHODS: We performed a 24-month prospective observational study of adults (≥16 years) with JIA. The primary outcome measure was decline in cognitive function defined as a worsening of ≥2 points on the scales of the subsets administered to evaluate the different cognitive areas using the Wechsler Adult Intelligence Scale (WAIS) after 24 months: attention/concentration (digit span); verbal function (vocabulary); visual-spatial organization (block design); working memory (letter-number sequencing); and problem solving (similarities). Other variables included average inflammatory activity using C-reactive protein and composite activity indexes, comorbidity, and treatment. Logistic regression was performed to identify factors associated with cognitive decline. RESULTS: The study population comprised 52 patients with JIA. Of these, 15 (28.8%) had cognitive decline at V24. The most affected functions were working memory (17.3%), attention/concentration (9.6%), verbal function (7.7%), visual-spatial organization (7.7%), and problem solving (3.8%). There were no significant differences in the median direct or scale scores for the cognitive functions evaluated between V0 and V24 for the whole sample. The factors associated with cognitive decline in patients with JIA were average C-reactive protein (OR [95% CI], 1.377 [1.060-1.921]; p = 0.039), depression (OR [95% CI], 3.691 [1.294-10.534]; p = 0.015), and treatment with biologics (OR [95% CI], 0.188 [0.039-0.998]; p = 0.046). CONCLUSION: Cognitive decline was detected in almost one third of adults with JIA after 24 months of follow-up. Systemic inflammatory activity in JIA patients was related to cognitive decline. Patients treated with biologics had a lower risk of decline in cognitive functions.

Recomendaciones para el diagnóstico y tratamiento de la baja masa ósea para la edad cronológica y la osteoporosis infantil en Atención Primaria / Recommendations for the diagnosis and treatment of low bone mass for chronological age and childhood osteoporosis in primary care

Introducción: la ausencia en nuestro medio de protocolos de manejo y de derivación de los pacientes de riesgo hace que exista una gran variabilidad en la actividad preventiva y en el manejo clínico respecto a la osteoporosis infantil en los pediatras de nuestro país. Método: recientemente, el Grupo de Trabajo de Osteogénesis Imperfecta y Osteoporosis Infantil, de la Sociedad Española de Reumatología Pediátrica (SERPE) ha publicado un documento de consenso con recomendaciones sobre el diagnóstico y tratamiento de la osteoporosis secundaria infantil. En este artículo, resumimos aquellas más relevantes en el ámbito de Atención Primaria. Un panel de expertos, compuesto por pediatras y reumatólogos, elaboró una serie de recomendaciones basadas en la evidencia tras realizar una revisión cualitativa de la literatura. El nivel de evidencia se determinó para cada sección utilizando el sistema del Centro de Medicina basada en la Evidencia de Oxford (CEBM). Se realizó una encuesta Delphi para aquellas recomendaciones con un nivel de evidencia de IV o V. Se incluyeron todas las recomendaciones que tuvieron un nivel de concordancia superior o igual al 70%. Esta encuesta se envió a todos los miembros de la Sociedad Española de Reumatología Pediátrica. Resultados: se obtuvieron 51 recomendaciones, categorizadas en ocho secciones. Las recomendaciones resultantes son: cuándo sospechar y cómo prevenir la osteoporosis infantil y la baja masa ósea según la edad cronológica; qué métodos de detección y diagnóstico utilizar; cuáles son los tratamientos actuales y cómo prevenir la osteoporosis inducida por los corticoesteroides. Conclusión: la detección precoz y un enfoque terapéutico adecuado de la baja masa mineral ósea desde Atención Primaria (AP) son fundamentales para mejorar la salud ósea de nuestra población infantil. Las recomendaciones expuestas pueden ayudar a tomar las medidas de prevención y tratamiento correctas en la población infantil de riesgo (AU)

Introduction: due to the lack of standardised protocols for the management and referral of at-risk patients, there is substantial variability in the prevention and clinical management of childhood osteoporosis among paediatricians in Spain.Methods: the Working Group on Osteogenesis Imperfecta and Childhood Osteoporosis of the Sociedad Española de Reumatología Pediátrica (SERPE) recently published a consensus document with recommendations on the diagnosis and management of secondary childhood osteoporosis. An expert panel comprised of paediatricians and rheumatologists carried out a qualitative literature review and developed evidence-based recommendations.For each section, the level of evidence was determined using the Oxford Centre for Evidence-based Medicine (CEBM) system. A Delphi survey was conducted for those recommendations with a level of evidence of IV or V. All recommendations for which the level of agreement was 70% or greater were included. This survey was sent to all members of the SERPE.Results: the process yielded 51 recommendations categorized into 8 sections. The resulting recommendations concern when to suspect and how to prevent childhood osteoporosis and low bone mass according to chronological age; which screening and diagnosis methods to use; the current treatments and how to prevent corticosteroid-induced osteoporosis.Conclusions: early detection and an adequate approach to the treatment of low bone mass at the primary care (PC) level are essential to improve bone health in our paediatric population. These recommendations could contribute to improving prevention and treatment measures in at-risk children. (AU)

Afectación pulmonar rápidamente progresiva secundaria a dermatomiositis juvenil anti-MDA5(+) con evolución devastadora / Interstitial lung disease in anti-MDA5 positive dermatomyositis with devastating evolution

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Digital ischemia as the initial presentation of catastrophic antiphospholipid syndrome.

Catastrophic antiphospholipid syndrome is an infrequent disease in children, but of major relevance because of its high morbidity and mortality. We report the case of a child with digital ischaemia in whom, after aetiological screening, the diagnosis of catastrophic antiphospholipid syndrome was made.

Isquemia digital como forma de presentación de síndrome antifosfolípido catastrófico pediátrico / Digital Ischemia as the Initial Presentation of Catastrophic Antiphospholipid Syndrome

El síndrome antifosfolípido catastrófico es una entidad infrecuente en Pediatría, pero con importante relevancia dada la elevada morbimortalidad. Se expone el caso de un niño con isquemia digital en el que, tras realizar despistaje etiológico de diferentes entidades infecciosas e inflamatorias, se llegó al diagnóstico de síndrome antifosfolípido catastrófico primario.(AU)

Catastrophic antiphospholipid syndrome is an infrequent disease in children, but of major relevance because of its high morbidity and mortality. We report the case of a child with digital ischaemia in whom, after aetiological screening, the diagnosis of catastrophic antiphospholipid syndrome was made.(AU)

Crohn's disease in a patient with systemic onset juvenile idiopathic arthritis. Association or associated side effect of treatment?

The progression of systemic-onset juvenile idiopathic arthritis (JIAs) to the different forms of presentation of inflammatory bowel disease is extremely rare. We present the first report of a patient with SJIA that progressed to Crohn's disease in which mutations have been detected in genes responsible for the adequate regulation of the innate immune system.

Miopatía necrosante inmunomediada seronegativa con afectación miocárdica / Seronegative immune-mediated necrotising myopathy with myocardial involvement

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Adiposity Is Related to Inflammatory Disease Activity in Juvenile Idiopathic Arthritis.

OBJECTIVE: To identify factors associated with the higher proportion of fatty tissue and overweight/obesity observed in patients with juvenile idiopathic arthritis (JIA). PATIENTS AND METHODS: We performed a cross-sectional study of 80 JIA patients aged 4-15 years with 80 age- and sex-matched healthy controls. Body composition was assessed using dual-energy x-ray absorptiometry. The 27-joint Juvenile Arthritis Disease Activity score (JADAS27) was calculated. Two multivariate models were constructed to identify factors associated with overweight/obesity and fat mass index (FMI). RESULTS: No differences were found between cases and controls in body mass index (BMI) or body composition. However, compared with controls, patients with a high inflammatory activity (JADAS27 > 4.2 for oligoarticular JIA or >8.5 for polyarticular disease) had higher values for BMI (p = 0.006); total fat mass (p = 0.003); FMI (p = 0.001); and fat in the legs (p = 0.001), trunk (p = 0.001), and arms (p = 0.002). The factors associated with overweight/obesity in patients were the duration of therapy with biological drugs, measured in months (OR [95% CI] = 1.12 [1.02-1.04]; p = 0.037), and physical activity (OR [95% CI] = 0.214 [0.07-0.68]; p = 0.010), while the factors associated with FMI were age (ß [95% CI] = 0.30 [0.17-1.41]; p = 0.014), JADAS27 (ß [95% CI] = 0.45 [0.16-1.08]; p = 0.009), and physical activity (ß [95% CI] = -0.22 [-5.76 to 0.29]; p = 0.031). CONCLUSION: Our study revealed no differences between JIA patients with well-controlled disease and low disability and the healthy population in BMI or body composition. Furthermore, the association observed between inflammatory activity and adiposity could be responsible for poorer clinical course.

Lesiones cutáneas en niños durante la primera ola de la pandemia por SARS-CoV-2 / Skin lesions in children during the first wave of the SARS-CoV-2 pandemic

Introducción: Las manifestaciones cutáneas se han incluido en el espectro clínico de los pacientes con COVID-19. Nuestro objetivo fue determinar la asociación entre las lesiones cutáneas observadas en niños durante la primera ola de la pandemia y la infección por SARS-CoV-2, analizando otras posibles etiologías infecciosas o autoinmunes.Material y métodosEstudio observacional, multicéntrico, de corte transversal, desarrollado en niños con manifestaciones cutáneas desde abril hasta mayo de 2020. La determinación de SARS-CoV-2 se realizó mediante PCR en exudado nasofaríngeo y/o serología.ResultadosSe seleccionó a 62 niños; 9 (14,5%) presentaron serología positiva para SARS-CoV-2, siendo la PCR negativa en todos los casos en los que se realizó. Los pacientes con serología positiva para SARS-CoV-2 presentaron con más frecuencia lesiones pernióticas y/o vesiculosas (66,7 vs. 24,5%; p=0,019). El exantema generalizado, urticarial y maculopapuloso fue más habitual en el grupo de pacientes con serología negativa (37,7 vs. 0%; p=0,047); se aislaron otros patógenos en el 41,5%. No hubo diferencias significativas en cuanto a la positividad de autoanticuerpos entre ambos grupos.ConclusiónEn nuestro estudio, las lesiones de tipo perniosis o vesiculosas se relacionaron significativamente con el contacto previo con SARS-CoV-2. (AU)

Background: Cutaneous manifestations have been included in COVID-19 patients’ clinical spectrum. Our objective was to determine the association between skin lesions in children and SARS-CoV-2 infection, analyzing others possible infectious/autoimmune etiologies.Material and methodsObservational, multicenter, cross-sectional study, about children with skin manifestations from April to May 2020. The diagnosis of SARS-CoV-2 was performed by PCR in nasopharyngeal exudate and/or presence of antibodies by serology.ResultsSixty-two children were included, 9 (14.5%) presented positive antibodies to SARS-CoV-2, with no positive PCR to SARS-Cov-2 in those patients in whom it was made. Patients with positive serology to SARS-CoV-2 presented chilblains and/or vesicular-bullous skin lesions more frequently (66.7% vs. 24.5%, p=0.019). Generalized, urticarial and maculopapular rash was more common in patients with negative antibodies (37.7 vs. 0%, p=0.047), others pathogens were isolated in 41.5% of these patients. There were no significant differences in the positivity for autoantibodies between both groups.ConclusionIn our study, the presence of chilblains-like and/or vesicular lesions were significantly related to SARS-CoV-2 previous contact. (AU)

Skin lesions in children during the first wave of the SARS-CoV-2 pandemic.

BACKGROUND: Cutaneous manifestations have been included in COVID-19 patients' clinical spectrum. Our objective was to determine the association between skin lesions in children and SARS-CoV2 infection, analyzing others possible infectious/autoimmune etiologies. MATERIAL AND METHODS: Observational, multicenter, cross-sectional study, about children with skin manifestations from April to May 2020. The diagnosis of SARS-CoV2 was performed by PCR in nasopharyngeal exudate and/or presence of antibodies by serology. RESULTS: Sixty-two children were included, 9 (14.5%) presented positive antibodies to SARS-CoV-2, with no positive PCR to SARS-Cov-2 in those patients in whom it was made. Patients with positive serology to SARS-CoV-2 presented chilblains and/or vesicular-bullous skin lesions more frequently (66.7% vs. 24.5%, p = 0.019). Generalized, urticarial and maculopapular rash was more common in patients with negative antibodies (37.7 vs. 0%, p = 0.047), others pathogens were isolated in 41.5% of these patients. There were no significant differences in the positivity for autoantibodies between both groups. CONCLUSION: In our study, the presence of chilblains-like and/or vesicular lesions were significantly related to SARS-CoV2 previous contact.

INTRODUCCIÓN: Las manifestaciones cutáneas se han incluido en el espectro clínico de los pacientes con COVID-19. Nuestro objetivo fue determinar la asociación entre las lesiones cutáneas observadas en niños durante la primera ola de la pandemia y la infección por SARS-CoV-2, analizando otras posibles etiologías infecciosas o autoinmunes. MATERIAL Y MÉTODOS: Estudio observacional, multicéntrico, de corte transversal, desarrollado en niños con manifestaciones cutáneas desde abril hasta mayo de 2020. La determinación de SARS-CoV-2 se realizó mediante PCR en exudado nasofaríngeo y/o serología. RESULTADOS: Se seleccionó a 62 niños; 9 (14,5%) presentaron serología positiva para SARS-CoV-2, siendo la PCR negativa en todos los casos en los que se realizó. Los pacientes con serología positiva para SARS-CoV-2 presentaron con más frecuencia lesiones pernióticas y/o vesiculosas (66,7 vs. 24,5%; p = 0,019). El exantema generalizado, urticarial y maculopapuloso fue más habitual en el grupo de pacientes con serología negativa (37,7 vs. 0%; p = 0,047); se aislaron otros patógenos en el 41,5%. No hubo diferencias significativas en cuanto a la positividad de autoanticuerpos entre ambos grupos. CONCLUSIÓN: En nuestro estudio, las lesiones de tipo perniosis o vesiculosas se relacionaron significativamente con el contacto previo con SARS-CoV-2.

Panarteritis nudosa cutánea: no todo es eritema nudoso / Polyarteritis nodosa: not all is erythema nodosum

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[Skin lesions in children during the first wave of the SARS-CoV-2 pandemic]. / Lesiones cutáneas en niños durante la primera ola de la pandemia por SARS-CoV-2.

BACKGROUND: Cutaneous manifestations have been included in COVID-19 patients' clinical spectrum. Our objective was to determine the association between skin lesions in children and SARS-CoV-2 infection, analyzing others possible infectious/autoimmune etiologies. MATERIAL AND METHODS: Observational, multicenter, cross-sectional study, about children with skin manifestations from April to May 2020. The diagnosis of SARS-CoV-2 was performed by PCR in nasopharyngeal exudate and/or presence of antibodies by serology. RESULTS: Sixty-two children were included, 9 (14.5%) presented positive antibodies to SARS-CoV-2, with no positive PCR to SARS-Cov-2 in those patients in whom it was made. Patients with positive serology to SARS-CoV-2 presented chilblains and/or vesicular-bullous skin lesions more frequently (66.7% vs. 24.5%, p=0.019). Generalized, urticarial and maculopapular rash was more common in patients with negative antibodies (37.7 vs. 0%, p=0.047), others pathogens were isolated in 41.5% of these patients. There were no significant differences in the positivity for autoantibodies between both groups. CONCLUSION: In our study, the presence of chilblains-like and/or vesicular lesions were significantly related to SARS-CoV-2 previous contact.