Miositis aguda infantil en urgencias pediátricas / Acute myositis in the paediatric emergency department

Introducción: la miositis aguda benigna de la infancia (MAB) es una enfermedad benigna y autolimitada, considerada en la actualidad como una secuela de enfermedades víricas. Su manifestación clínica típica son las mialgias en las extremidades inferiores acompañadas de rechazo a caminar. Pacientes y métodos: realizamos una revisión retrospectiva de los casos de MAB atendidos en nuestro servicio de urgencias pediátricas durante un periodo de 25 años. Obtuvimos datos demográficos, clínicos y de laboratorio a través de la revisión sistemática de historias clínicas. Se elaboraron estadísticas descriptivas y se efectuaron comparaciones entre grupos a través del test U de Mann-Whitney. Las correlaciones entre variables cuantitativas se realizaron mediante el análisis de rangos de Spearman. Resultados: se identificaron 139 casos de MAB (74,8% varones). La mayor incidencia se produjo en los meses de invierno y en niños de 4-8 años. Las mialgias bilaterales en miembros inferiores fueron el principal motivo de consulta. El 86,3% referían fiebre acompañada de síntomas respiratorios (88,5%), confirmándose en urgencias la infección por virus influenza en 37 casos (75,6% del serotipo B). En el momento del diagnóstico, la mediana de creatincinasa (CK) fue 1794 U/l, no existiendo diferencias significativas en función del sexo (p = 0,687). La función renal fue normal en todos los pacientes, ninguno necesitó hospitalización. Conclusiones: la MAB es una entidad fácilmente reconocible teniendo en cuenta sus manifestaciones clínicas y analíticas. A pesar de las masivas elevaciones de CK, sus complicaciones son excepcionales y la mayoría de los pacientes pueden ser dados de alta con tratamiento conservador (AU)

Introduction: benign acute childhood myositis (BACM) is a benign, self-limiting disease currently believed to be a sequela of viral infections. The typical clinical presentation is myalgia in the lower extremities accompanied by refusal to walk.Patients and methods: we did a retrospective review of all the cases of BACM managed in our paediatric emergency department over a 25-year period. We collected data on demographic, clinical and laboratory variables through the systematic review of health records. We performed a descriptive statistical analysis, followed by comparison of groups with the Mann-Whitney U test. The correlation between quantitative variables was assessed by means of the Spearman rank coefficient.Results: we identified 139 cases of BACM (74.8% in male patients). The highest incidence corresponded to children aged 4 to 8 years and the winter months. Bilateral lower limb myalgia was the most frequent presenting complaint. Fever was reported in 86.3% of cases, accompanied by respiratory symptoms (88.5%); influenza virus infection was confirmed in 37 cases (75.6% serotype B). At diagnosis, the median creatine kinase (CK) level was 1794 U/l, with no significant differences between the sexes (p=0.687). All patients had normal renal function, and none required hospitalization.Conclusions: based on its clinical and analytical manifestations, BACM is easy to recognise. Although massive elevation of CK is common, complications are rare, and most patients can be discharged with conservative treatment. (AU)

Humoral and cellular immune response to mRNA SARS-CoV-2 BNT162b2 vaccine in adolescents with rheumatic diseases.

BACKGROUND: Data about safety and efficacy of the mRNA SARS-CoV-2 vaccine in adolescents with rheumatic diseases (RD) is scarce and whether these patients generate a sufficient immune response to the vaccine remains an outstanding question. OBJECTIVE: To evaluate safety and humoral and cellular immunity of the BNT162b2 vaccine in adolescents 12 to 18 years with RD and immunosuppressive treatment compared with a healthy control group. METHODS: Adolescents from 12 to 18 years with RD followed at Hospital La Paz in Madrid (n = 40) receiving the BNT162b2 mRNA vaccination were assessed 3 weeks after complete vaccination. Healthy adolescents served as controls (n = 24). Humoral response was measured by IgG antiSpike antibodies, and cellular response by the quantity of IFN-γ and IL-2 present in whole blood stimulated with SARS-CoV-2 Spike and M proteins. RESULTS: There were no differences in spike-specific humoral or cellular response between groups (median IFN-γ response to S specific protein; 528.80 pg/ml in controls vs. 398.44 in RD patients, p 0.78, and median IL-2 response in controls: 635.68 pg/ml vs. 497.30 in RD patients, p 0.22. The most frequent diagnosis was juvenile idiopathic arthritis (26/40, 65%) followed by Lupus (6/40, 15%). 60% of cases (23/40) received TNF inhibitors and 35% (14/40) methotrexate. 40% of patients (26/64) had previous SARS-CoV-2 infection, 9 in the control group and 17 in the RD patients without differences. Of note, 70% of infections were asymptomatic. A higher IFN-γ production was found in COVID-19 recovered individuals than in naive subjects in both groups (controls: median 859 pg/ml in recovered patients vs. 450 in naïve p 0.017, and RD patients: 850 in recovered vs. 278 in naïve p 0.024). No serious adverse events or flares were reported following vaccination. CONCLUSIONS: We conclude that standard of care treatment for adolescents with RD including TNF inhibitors and methotrexate did not affect the humoral and the cellular immunity to BNT162b2 mRNA vaccination compared to a healthy control group. The previous contact with SARS-CoV-2 was the most relevant factor in the immune response.

Clinical course and seroprevalence of COVID-19 in children with rheumatic diseases-cross-sectional study from a reference centre in Spain.

SARS-CoV-2 infections in children are frequently asymptomatic or mild and can go unnoticed. This study aimed to describe the seroprevalence and clinical course of SARS-CoV-2 in a cohort of children with rheumatic diseases in a real-life setting and assess possible risk factors. A cross-sectional study was performed in a paediatric rheumatology unit (September 2020 to February 2021). At inclusion, a specific questionnaire was completed and SARS-CoV-2 serology was performed. Demographics, treatment and disease activity of patients with and without laboratory-confirmed SARS-CoV-2 infection were compared. A total of 105 children were included. SARS-CoV-2 infection was demonstrated in 27 patients (25.7%). The mean age was 11.8 years, and most patients were females (72.4%). The most frequent underlying condition was juvenile idiopathic arthritis (70.3%; 19/27). Patients received immunosuppressive treatment in 78% of cases (21/27). Overall, 44.4% (12/27) of infected patients were asymptomatic. A total of 66.7% (18/27) of patients did not require medical assistance. Three patients required hospital admission because of COVID-19. Children with confirmed SARS-CoV-2 infection were less frequently in remission (52% vs 72%; p 0.014). Moderate disease activity and treatment with oral corticosteroids were associated with higher risk for SARS-CoV-2 (OR 5.05; CI 95%: 1.56-16.3 and OR 4.2; CI 95%: 1.26-13.9, respectively). In a cohort of Spanish paediatric patients with rheumatic diseases, clinical course of COVID-19 was mild, with more than one third of asymptomatic cases. Higher disease activity and oral corticosteroids appear to be risk factors for SARS-CoV-2 infection. Key Points • We aimed to investigate the seroprevalence of SARS-CoV-2 infection in a cohort of Spanish paediatric patients with RD, testing both symptomatic and asymptomatic patients. We also compared treatment and disease activity of patients with and without laboratory-confirmed SARS-CoV-2 infection. • In our cohort of 105 paediatric patients with rheumatic diseases, the clinical course of COVID-19 was mild and 44% of cases were asymptomatic. Three cases required hospital admission with no complications. Seroprevalence was 20%. • No association was found between disease activity or treatment with corticosteroids and symptomatic or asymptomatic infection. Higher disease activity and treatment with oral corticosteroids appeared to be risk factors for laboratory-confirmed SARS-CoV-2 infection.

Clinical characteristics and COVID-19 outcomes in a regional cohort of pediatric patients with rheumatic diseases.

BACKGROUND: This study aimed to assess the baseline characteristics and clinical outcomes of coronavirus disease 2019 (COVID-19) in pediatric patients with rheumatic and musculoskeletal diseases (RMD) and identify the risk factors associated with symptomatic or severe disease defined as hospital admission, intensive care admission or death. METHODS: An observational longitudinal study was conducted during the first year of the SARS-CoV-2 pandemic (March 2020-March 2021). All pediatric patients attended at the rheumatology outpatient clinics of six tertiary referral hospitals in Madrid, Spain, with a diagnosis of RMD and COVID-19 were included. Main outcomes were symptomatic disease and hospital admission. The covariates were sociodemographic and clinical characteristics and treatment regimens. We ran a multivariable logistic regression model to assess associated factors for outcomes. RESULTS: The study population included 77 pediatric patients. Mean age was 11.88 (4.04) years Of these, 30 patients (38.96%) were asymptomatic, 41 (53.25%) had a mild-moderate COVID-19 and 6 patients (7.79%) required hospital admission. The median length of hospital admission was 5 (2-20) days, one patient required intensive care and there were no deaths. Previous comorbidities increased the risk for symptomatic disease and hospital admission. Compared with outpatients, the factor independently associated with hospital admission was previous use of glucocorticoids (OR 3.51; p = 0.00). No statistically significant risk factors for symptomatic COVID-19 were found in the final model. CONCLUSION: No differences in COVID-19 outcomes according to childhood-onset rheumatic disease types were found. Results suggest that associated comorbidities and treatment with glucocorticoids increase the risk of hospital admission.

Utilidad de una consulta especializada en inmunodeficiencias primarias / Usefulness of a specialised primary immunodeficiencies clinic

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Uso de fármacos inmunomoduladores en una consulta de uveítis / Use of immunomodulatory drugs at a uveitis clinic

OBJETIVO: En el tratamiento de las uveítis no infecciosas se emplean corticoides y fármacos inmunomoduladores. Su uso ha aumentado en los últimos años y se ha enriquecido con la aparición de nuevos tratamientos. Sin embargo, no existen guías ni protocolos claros de actuación. El objetivo es analizar la respuesta a los fármacos empleados y las características de los pacientes atendidos en una consulta multidisciplinaria de uveítis. MATERIAL Y MÉTODOS: Estudio observacional retrospectivo de los pacientes atendidos desde enero de 2012 hasta diciembre de 2015. Se excluyen las uveítis infecciosas, posquirúrgicas, postraumáticas y los síndromes de enmascaramiento. RESULTADOS: Se incluyeron 216 pacientes. El 58,80% son uveítis sin afectación sistémica, la mayoría idiopáticas, y el 35,65% uveítis con afectación sistémica, asociadas principalmente a espondiloartritis. Las uveítis sin afectación sistémica y las uveítis anteriores se controlaron mejor que el resto con tratamiento local (p = 0,002 y p < 0,001, respectivamente). El 49,76% de los pacientes requirió tratamiento sistémico. De los pacientes tratados con inmunomoduladores, el 53,26% precisó un segundo fármaco y el 31,52%, un tercero. Las mujeres necesitaron inmunomoduladores con más frecuencia que los varones (p = 0,042). El inmunomodulador más empleado fue metotrexato. Las uveítis posteriores respondieron al segundo inmunomodulador peor que las anteriores (p = 0,006). CONCLUSIONES: Casi la mitad de los pacientes necesitaron un fármaco inmunomodulador y algunos precisaron varios cambios sucesivos de fármaco. Las uveítis intermedias resultaron las más refractarias al tratamiento

OBJECTIVE: The treatment of noninfectious uveitis includes steroids and immunomodulatory drugs, the use of which has increased in the last few years, and the options have been enriched with the development of new treatments. However, clear therapeutic guidelines and protocols have not been developed. The purpose is to analyze the response to the drugs used and the characteristics of the patients treated at a multidisciplinary uveitis clinic. MATERIAL AND METHODS: Observational and retrospective study of the patients attended to from January 2012 to December 2015. Infectious, posttraumatic and postoperative uveitis, as well as masquerade syndrome, were excluded. RESULTS: Two hundred six patients were included. Overall, 58.80% had uveitis without association of systemic disease, mostly idiopathic uveitis, and 35.65% had uveitis with systemic involvement, mainly related to spondyloarthritis. Uveitis without systemic association and anterior uveitis achieved disease control with local treatment more frequently than others (p=.002 and p <.001, respectively). In all, 49.76% of the patients required systemic treatment. Among those treated with immunomodulators, 53.26% needed a second drug and 31.52% needed a third drug. Women required immunomodulators more often than men (P=.042). Methotrexate was the most widely used immunomodulator. Posterior uveitis responded less favorably to the second immunomodulator than anterior uveitis (p=.006). CONCLUSIONS: Almost half of the patients needed an immunomodulatory drug and some of them required successive drug changes. Intermediate uveitis was the most treatment-refractory uveitis

Use of Immunomodulatory Drugs at a Uveitis Clinic. / Uso de fármacos inmunomoduladores en una consulta de uveítis.

OBJECTIVE: The treatment of noninfectious uveitis includes steroids and immunomodulatory drugs, the use of which has increased in the last few years, and the options have been enriched with the development of new treatments. However, clear therapeutic guidelines and protocols have not been developed. The purpose is to analyze the response to the drugs used and the characteristics of the patients treated at a multidisciplinary uveitis clinic. MATERIAL AND METHODS: Observational and retrospective study of the patients attended to from January 2012 to December 2015. Infectious, posttraumatic and postoperative uveitis, as well as masquerade syndrome, were excluded. RESULTS: Two hundred six patients were included. Overall, 58.80% had uveitis without association of systemic disease, mostly idiopathic uveitis, and 35.65% had uveitis with systemic involvement, mainly related to spondyloarthritis. Uveitis without systemic association and anterior uveitis achieved disease control with local treatment more frequently than others (p=.002 and p <.001, respectively). In all, 49.76% of the patients required systemic treatment. Among those treated with immunomodulators, 53.26% needed a second drug and 31.52% needed a third drug. Women required immunomodulators more often than men (P=.042). Methotrexate was the most widely used immunomodulator. Posterior uveitis responded less favorably to the second immunomodulator than anterior uveitis (p=.006). CONCLUSIONS: Almost half of the patients needed an immunomodulatory drug and some of them required successive drug changes. Intermediate uveitis was the most treatment-refractory uveitis.

Obesidad y episodios vasculares en la diabetes mellitus tipo 2 / Obesity and vascular events in type 2 diabetes mellitus

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