Prognostic significance of lymphocytic foci composition in minor salivary gland biopsies for severe disease flare and severity in Sjögren's syndrome: a 3-year follow-up cohort study.

Introduction: This was an ambispective cohort study evaluating the prognostic significance of lymphocytic foci and its lymphoid composition in minor salivary gland biopsy (MSGB) for short-term disease flare and severity in Sjögren's syndrome (SS). Methods: The inclusion criteria comprised individuals meeting the ACR/EULAR 2016 criteria who underwent MSGB with an infiltration of more than 50 lymphocytes and received clinical diagnosis between September 2017 and December 2018. Patients with inadequate biopsy samples were excluded. The number of lymphocytic foci and their lymphoid composition in MSGB were assessed using immunofluorescence staining. Major organ damage and improvements in the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) were measured. Statistical analyses, including Cox and linear regressions, were conducted. Results: A total of 78 patients with at least one lymphocytic focus were included in the study. The presence of higher T-cell counts in lymphocytic foci in MSGB was associated with severe disease flare, and a logarithmic transformation of T-cell count indicated increased risk (HR 1.96, 95% CI 0.91-4.21). Improvements in the ESSDAI were associated with higher total lymphocyte count and T- and B-cell numbers in the lymphoid composition of the lymphocytic foci. Seropositive patients exhibited higher T CD4+ cell numbers. Correlation analysis showed negative associations between age and lymphocytic foci and the T-cell count. Positive correlations were observed between antinuclear antibody (ANA) titers and total lymphocyte numbers. Discussion: Patients with a higher number of T cells in the lymphocytic infiltrates of lymphocytic foci may have a two-fold risk of severe disease flare. The number of B cells and T CD4+ cells in the lymphocytic infiltrates of lymphocytic foci showed a weak but positive relation with the ESSDAI improvement during follow-up. Age and seropositivity appeared to influence the lymphoid composition of the lymphocytic foci.

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.

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)

Interobserver reliability of Masei index validation by a multicenter collaborative group of rheumatologists.

MASEI is the main validated ultrasound score for the evaluation of enthesis. The lack of studies facing the agreement to achieve for the interpretation of the MAdrid Sonographic Enthesis Index (MASEI) among researchers from different centers in multicenter studies is of concern. The aim of this multicenter was to evaluate the interobserver reliability of MASEI. An experienced ultrasonographer-rheumatologist performed ultrasound scans of the areas included in MASEI index in three patients with Ankylosing Spondylitis and Psoriatic Arthritis. Videos were captured. The videos were then evaluated by 24 rheumatologists of the ultrasound working group of the Catalan Society of Rheumatology (EcoCAT). A face-to-face training meeting was held. Ten days after the workshop, the study participants evaluated the videos. A reliability assessment was performed. The ICC for the MASEI scores after the workshop was of 0.97 (95% CI 89-99). Reliability did not vary statistically with examiner experience. Globally, no problems of reliability by structures were seen, and all the ICCs were above 0.90 and improved slightly after the educational program. However, the correlation observed between examiners at plantar aponeursis and triceps tendon was weak. The small variability observed in the results of the index validation in our study, suggests that the MASEI index is reproducible by different observers when those are well trained and show awesome results of the enthesis when examined by ultrasound.

Expert panel consensus recommendations for diagnosis and treatment of secondary osteoporosis in children.

BACKGROUND: Osteoporosis incidence in children is increasing due to the increased survival rate of patients suffering from chronic diseases and the increased use of drugs that can damage bones. Recent changes made to the definition of childhood osteoporosis, along with the lack of guidelines or national consensuses regarding its diagnosis and treatment, have resulted in a wide variability in the approaches used to treat this disease. For these reasons, the Osteogenesis Imperfecta and Childhood Osteoporosis Working Group of the Spanish Society of Pediatric Rheumatology has sounded the need for developing guidelines to standardize clinical practice with regard to this pathology. METHODS: An expert panel comprised of 6 pediatricians and 5 rheumatologists carried out a qualitative literature review and provided recommendations based on evidence, when that was available, or on their own experience. The level of evidence was determined for each section using the Oxford Centre for Evidence-based Medicine (CEBM) system. A Delphi survey was conducted for those recommendations with an evidence level of IV or V. This survey was sent to all members of the SERPE. All recommendations that had a level of agreement higher or equal to 70% were included. RESULTS: Fifty-one recommendations, categorized into eight sections, were obtained. Twenty-four of them presented an evidence level 4 or 5, and therefore a Delphi survey was conducted. This was submitted electronically and received a response rate of 40%. All recommendations submitted to the Delphi round obtained a level of agreement of 70% or higher and were therefore accepted. CONCLUSION: In summary, we present herein guidelines for the prevention, diagnosis and treatment of secondary childhood osteoporosis based on the available evidence and expert clinical experience. We believe it can serve as a useful tool that will contribute to the standardization of clinical practice for this pathology. Prophylactic measures, early diagnosis and a proper therapeutic approach are essential to improving bone health, not only in children and adolescents, but also in the adults they will become in the future.

Vacunación antigripal y antineumocócica 23 valencias en pacientes reumáticos en tratamiento con inmunosupresores / Influenza and pneumococcal (23-valent) vaccination in rheumatic patients on treatment with immunosuppressants

Objetivo: determinar la evolución de la tasa de vacunación antigripal y antineumocócica (23 valencias) entre los años 2010 y 2014 en pacientes reumáticos en tratamiento con inmunosupresores y evaluar la magnitud de infecciones respiratorias documentadas en el año posterior a la vacunación. Método: estudio descriptivo longitudinal retrospectivo. Se reclutaron por conveniencia 200 pacientes con artritis reumatoide o espondiloartropatías en tratamiento con fármacos antirreumáticos modificadores de la enfermedad (FAME) sintético, FAME biológico o corticoides. Se conformaron dos grupos de 100 pacientes (2010 y 2014) en los que se revisaron historias clínicas para recoger variables demográficas, comorbilidades, hábitos tóxicos, diagnóstico reumatológico y años de evolución de este, tratamiento, administración de la vacuna antigripal y antineumocócica 23v y número de infecciones respiratorias en el año posterior. Resultados: en la muestra un 66% fue mujer, la edad media fue de 66 años y 12 de evolución de la enfermedad. El 43,5% había recibido vacunación antigripal y el 30% vacunación neumocócica 23v. El 21,5% presentó infección respiratoria en el tiempo de seguimiento. Hubo una mayor tasa de vacunación antigripal y antineumocócica en 2014 frente a 2010 (49 y 29% vs. 38% y 21%, respectivamente), siendo solo estadísticamente significativo el aumento en la cobertura antineumocócica. Los pacientes mayores de 65 años y alguna comorbilidad o hábito tóxico son más vacunados (p< 0,05). No hubo diferencias en las vacunaciones ni en el número de infecciones respiratorias, ni en función del diagnóstico reumatológico, ni del tratamiento recibido por el paciente. Conclusiones: la tasa de vacunación mejoró en 2014 respecto a 2010. No obstante, el profesional enfermero ha de intervenir en mejorar la tasa de vacunación en estos pacientes

Objective: to determine the evolution in the rate of influenza and pneumococcal (23-valent) vaccination between the years 2010 and 2014 in rheumatic patients on treatment with immunosuppressants, and to evaluate the extent of documented respiratory infections within the year after vaccination. Method: a retrospective cross-sectional descriptive study. Two hundred (200) patients were recruited by convenience sampling, with rheumatoid arthritis or spondyloarthropathy, on treatment with synthetic disease modifying antirheumatic drugs (DMARDs), biologic DMARDs or corticosteroids. Two arms of 100 patients were formed (2010 and 2014), and clinical records were reviewed in order to collect demographical variables, comorbidities, toxic habits, rheumatology diagnosis and years of disease evolution, treatment, administration of the influenza and the pneumococcal 23 vaccine, and number of respiratory infections during the following year. Results: in the sample, 66% were women, their mean age was 66 years, with 12 years of disease evolution; 43.5% had received influenza vaccination, and 30% pneumococcal 23v vaccination; 21.5% presented respiratory infection within the time of follow-up. There was a higher rate of influenza and pneumococcal vaccination in 2014 vs. 2010 (49 and 29% vs. 38% and 21%, respectively); only the increase in pneumococcal coverage was statistically significant. Patients >65-year-old and with some comorbidity or toxic habit were more vaccinated (p< 0.05). There were no differences in vaccinations or in the number of respiratory infections based on the rheumatology diagnosis or the treatment received by the patient. Conclusions: the vaccination rate improved in 2014 vs. 2010. However, the nursing professional must be involved in the improvement of the vaccination rate for these patients