Factors Associated With Mortality in Patients With Immune-Mediated Rheumatic Diseases and COVID-19 From Latin America: Data From Argentina, Mexico, and Brazil.

OBJECTIVE: To describe characteristics of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in patients with rheumatic immune-mediated inflammatory diseases (IMIDs) from Argentina, Mexico and Brazil, and to assess factors associated with mortality in this population. METHODS: Data from 3 national registries, SAR-COVID (Argentina), CMR-COVID (Mexico), and ReumaCoV-Brasil (Brazil), were combined. Adult patients with IMIDs and SARS-CoV-2 infection were recruited. Sociodemographic data, comorbidities, IMID clinical characteristics and treatment, and SARS-CoV-2 infection presentation and outcomes were recorded. RESULTS: A total of 4827 individuals were included: 2542 (52.7%) from SAR-COVID, 1167 (24.2%) from CMR-COVID, and 1118 (23.1%) from ReumaCoV-Brasil. Overall, 82.1% were female with a mean age of 49.7 (SD, 14.3) years; 22.7% of the patients were hospitalized, and 5.3% died because of COVID-19 (coronavirus disease 2019). Argentina and Brazil had both 4% of mortality and Mexico 9.4%. In the multivariable analysis, older age (≥60 years; odds ratio [OR], 7.4; 95% confidence interval [CI], 4.6-12.4), male sex (OR, 1.5; 95% CI, 1.1-2.1), living in Mexico (OR, 3.0; 95% CI, 2.0-4.4), comorbidity count (1 comorbidity: OR, 1.5; 95% CI, 1.0-2.1), diagnosis of connective tissue disease or vasculitis (OR, 1.8; 95% CI, 1.3-2.4), and other diseases (OR, 2.6; 95% CI, 1.6-4.1) compared with inflammatory joint disease, high disease activity (OR, 4.2; 95% CI, 2.5-7.0), and treatment with glucocorticoids (OR, 1.9; 95% CI, 1.4-2.5) or rituximab (OR, 4.2; 95% CI, 2.7-6.6) were associated with mortality. CONCLUSIONS: Mortality in patients with IMIDs was particularly high in Mexicans. Ethnic, environmental, societal factors, and different COVID-19 mitigation measures adopted have probably influenced these results.

[ADHEFAP: Clinical trial to evaluate an educational-behavioral telephone intervention by the primary care pharmacist to improve therapeutic adherence]. / ADHEFAP: ensayo clínico para evaluar una intervención telefónica educacional-conductual por el farmacéutico de atención primaria en la mejora de la adherencia terapéutica.

OBJECTIVE: Evaluate the improvement in therapeutic adherence of an intervention based on telephone monitoring by the primary care pharmacist. DESIGN: Randomized open controlled trial. LOCATION: This study was carried out in 2021 by a multidisciplinary team working with health professionals of thirteen health centers belonging to four health districts in Community of Madrid, Spain. PARTICIPANTS: These were patients (60-74 years) with polipharmacy classified as non-adherent according to the Morisky-Green test. Two hundred and twenty-four patients were originally enrolled, 87 of them were non-adherents. Of these, 15 were lost and 72 were finally randomized. Seventy-one patients completed the study (33 intervention group and 38 control group). INTERVENTIONS: Patients randomized to the intervention arm were included in a follow-up telephone program consisting of an interview at months 1, 2, and 3 to improve adherence. The Morisky-Green test was repeated at month 4 to assess improvement. In the control group this test was only performed at month 4. MAIN MEASUREMENTS: Adherence measured by Morisky-Green at baseline and at 4th month. RESULTS: The 72.7% of patients in the intervention group became adherent while only 34.2% did in the control arm, being the difference 38.5% (95% CI: 17.1-59.9), statistically significant (P=.001). CONCLUSIONS: After a follow-up educational-behavioral telephone intervention in non-adherent patients by the primary care pharmacist, therapeutic adherence was improved statistically significantly in the intervention group compared with the control group.

Children with systemic autoinflammatory diseases have multiple, mixed ethnicities that reflect regional ethnic diversity.

OBJECTIVES: To evaluate the ethnic diversity of children with a systemic autoinflammatory disease (SAID) in a multi-ethnic Canadian province. METHODS: Self-reported ethnicity of 149 children and adolescents with a SAID in British Columbia, Canada, was analysed for ethnic representation among individual patients, across the cohort, within particular SAIDs, and compared to provincial census data on ethnic diversity. RESULTS: Half of reported cases had a diagnosis of either PFAPA (23.5%) or an unclassifiable autoinflammatory syndrome (31.5%), with a monogenic SAID diagnosed in only 12.8% of cases. The majority of participants (73.1%) were mixed ethnicity with European and Asian heritage reported most frequently (57.0% and 23.0% of all responses, respectively). Ethnic diversity reflected regional diversity except for West Asian, Arabic, Jewish, and Eastern European heritage, which were over-represented in SAID patients, and Chinese descent, which was under-represented in our cohort compared to the general population of British Columbia. CONCLUSIONS: Results from this study show extensive multi-ethnic diversity in individual patients and across the various SAIDs inclusive of monogenic SAIDs that are frequently associated with particular ethnicities. Although not disproportionately represented, this is the first report of systemic autoinflammatory disease in Canadian children of Indigenous heritage.

Synthesis and profiling of benzylmorpholine 1,2,4,5-tetraoxane analogue N205: Towards tetraoxane scaffolds with potential for single dose cure of malaria.

A series of aryl carboxamide and benzylamino dispiro 1,2,4,5-tetraoxane analogues have been designed and synthesized in a short synthetic sequence from readily available starting materials. From this series of endoperoxides, molecules with in vitro IC50s versus Plasmodium falciparum (3D7) as low as 0.84 nM were identified. Based on an assessment of blood stability and in vitro microsomal stability, N205 (10a) was selected for rodent pharmacokinetic and in vivo antimalarial efficacy studies in the mouse Plasmodium berghei and Plasmodium falciparum Pf3D70087/N9 severe combined immunodeficiency (SCID) mouse models. The results indicate that the 4-benzylamino derivatives have excellent profiles with a representative of this series, N205, an excellent starting point for further lead optimization studies.

An Argentinean cohort of patients with rheumatic and immune-mediated diseases vaccinated for SARS-CoV-2: the SAR-CoVAC Registry-protocol and preliminary data.

BACKGROUND/OBJECTIVE: To evaluate the efficacy and safety of SARS-CoV-2 vaccine in patients with rheumatic and immune-mediated inflammatory diseases (IMIDs) in Argentina: the SAR-CoVAC registry. METHODS: SAR-CoVAC is a national, multicenter, and observational registry. Adult patients with rheumatic or IMIDs vaccinated for SARS-CoV-2 were consecutively included between June 1 and September 17, 2021. Sociodemographic data, comorbidities, underlying rheumatic or IMIDs, treatments received, their modification prior to vaccination, and history of SARS-CoV-2 infection were recorded. In addition, date and place of vaccination, type of vaccine applied, scheme, adverse events (AE), disease flares, and new immune-mediated manifestations related to the vaccine were analyzed. RESULTS: A total of 1234 patients were included, 79% were female, with a mean age of 57.8 (SD 14.1) years. The most frequent diseases were rheumatoid arthritis (41.2%), osteoarthritis (14.5%), psoriasis (12.7%), and spondyloarthritis (12.3%). Most of them were in remission (28.5%) or low disease activity (41.4%). At the time of vaccination, 21% were receiving glucocorticoid treatment, 35.7% methotrexate, 29.7% biological (b) disease modifying anti-rheumatic drugs (DMARD), and 5.4% JAK inhibitors. In total, 16.9% had SARS-CoV-2 infection before the first vaccine dose. Most patients (51.1%) received Gam-COVID-Vac as the first vaccine dose, followed by ChAdOx1 nCoV-19 (32.8%) and BBIBP-CorV (14.5%). Half of them (48.8%) were fully vaccinated with 2 doses; 12.5% received combined schemes, being the most frequent Gam-COVID-Vac/mRAN-1273. The median time between doses was 51 days (IQR 53). After the first dose, 25.9% of the patients reported at least one AE and 15.9% after the second, being flu-like syndrome and local hypersensitivity the most frequent manifestations. There was one case of anaphylaxis. Regarding efficacy, 63 events of SARS-CoV-2 infection were reported after vaccination, 19% occurred during the first 14 days post-vaccination, 57.1% after the first dose, and 23.8% after the second. Most cases (85.9%) were asymptomatic or mild and 2 died due to COVID-19. CONCLUSIONS: In this national cohort of patients, the most common vaccines used were Gam-COVID-Vac and ChAdOx1 nCoV-19. A quarter of the patients presented an AE and 5.1% presented SARS-CoV-2 infection after vaccination, in most cases mild. STUDY REGISTRATION: This study has been registered in ClinicalTrials.gov under the number: NCT04845997. Key Points • This study shows real-world data about efficacy and safety of SARS-CoV-2 vaccination in patients with rheumatic and immune-mediated inflammatory diseases. Interestingly, different types of vaccines were used including vector-based, mRNA, and inactivated vaccines, and mixed regimens were enabled. • A quarter of the patients presented an adverse event. The incidence of adverse events was significantly higher in those receiving mRAN-1273 and ChAdOx1 nCoV-19. • In this cohort, 5.1% presented SARS-CoV-2 infection after vaccination, in most cases mild.

Jealousy, Violence, and Sexual Ambivalence in Adolescent Students According to Emotional Dependency in the Couple Relationship.

BACKGROUND: Emotional dependency in couples involves excessive and dysfunctional emotional bonding. AIMS: This work aimed to determine the relationship between violence, jealousy, and ambivalent sexism according to emotional dependence in adolescent student couples. METHODS: A cross-sectional study. A total of 234 Spanish adolescents (69.7% female, Mage = 16.77, SD = 1.11) participated in the study. Participants completed an ad hoc interview and several validated tests (Partner's Emotional Dependency Scale, the Ambivalent Sexism Inventory, the Jealousy subscale of the Love Addiction Scale, the Conflict in Adolescent Dating Relationship Inventory). RESULTS: Of the sample, 40.6% indicated high emotional dependence and 14.5% extreme emotional dependence. Differences were observed according to gender (t = 3.92, p < 0.001), with adolescent boys scoring higher than adolescent girls. Extremely emotionally dependent participants showed differences in both violence (sexual, relational, verbal, and physical) and ambivalent sexism (hostile, benevolent) and jealousy scores. Generating a predictive model of emotional dependence, with the variable jealousy and ambivalent sexism as predictor variables, it was found that jealousy has the greatest predictive and major explanatory capacity (R2 = 0.297); with an R2 = 0.334. However, the contribution of the ASI-Hostile subscale was not significant when the ASI-Benevolent subscale was introduced into the model. Further, in a second model where the scores on jealousy and the couple conflict inventory's subscales were considered as predictors, are again jealousy makes the greatest predictive contribution and shows the greatest explanatory capacity (R2 = 0.296). It was found that the contribution is significant only for the predictive capacity of Sexual Violence and Relational Violence. In this sense, the educational context is one of the propitious places to detect and correct behaviors that may be indicative of potentially unbalanced and unbalancing relationships for adolescents.

[Variability of pharmaceutical provision to nursing homes from hospital pharmacy services]. / Variabilidad de la prestación farmacéutica a centros sociosanitarios residenciales desde los servicios de farmacia de hospital.

OBJECTIVE: To analyse the situation and the possible variability of  pharmaceutical provision to public nursing homes included in the "Project  pilot" from hospital pharmacy services of the Andalusian Health Service. METHOD: Cross-sectional multicenter study. A questionnaire of 36 questions including: nursing homes characteristics, legislation,  pharmacotherapeutic guide and prescription, preparation and transport,  dispensation, administration, pharmaceutical activity and healthcare data. RESULTS: We included 13 nursing homes. Everyone had a medicines store. All  dispensed in individualized unit dose. Three nursing homes did not have  a work space for the pharmacist. The pharmacist did not move to the nursing  home in three of them. CONCLUSIONS: Although there is some variability in the pharmaceutical provision to nursing homes included in the "Pilot Project", we  have identified strengths such as the dispensing of medicines as unit  dose system and opportunities to improve such as the increase of the  pharmacist's presence in nursing homes.

Objetivo: Analizar la situación y la posible variabilidad de la prestación farmacéutica a centros sociosanitarios residenciales públicos  incluidos en un proyecto piloto vinculados a un servicio de farmacia de hospital del Servicio Andaluz de Salud.Método: Estudio multicéntrico transversal. Se diseñó un cuestionario que incluye preguntas englobadas en: características del centro, legislación, guía farmacoterapéutica y prescripción, preparación y transporte, dispensación, administración, actividad farmacéutica y datos asistenciales.Resultados: Se incluyeron los 13 centros del proyecto piloto; todos  respondieron las 36 preguntas del cuestionario. Todos disponían de depósito de medicamentos. Todos dispensaban en dosis unitaria  individualizada. Tres centros sociosanitarios no disponían de puesto de  trabajo para el farmacéutico. El farmacéutico no se desplazaba al centro en  tres de ellos. Conclusiones: Aunque existe cierta variabilidad en la prestación  farmacéutica a los centros sociosanitarios del pilotaje, se han identificado fortalezas, como la dispensación de medicamentos en dosis  unitaria, y puntos de mejora como el aumento de la presencia del  farmacéutico en los centros.

ADHEFAP: ensayo clínico para evaluar una intervención telefónica educacional-conductual por el farmacéutico de atención primaria en la mejora de la adherencia terapéutica / ADHEFAP: Clinical trial to evaluate an educational-behavioral telephone intervention by the primary care pharmacist to improve therapeutic adherence

Objetivo: Evaluar la mejora en la adherencia terapéutica (AT) de una intervención basada en el seguimiento telefónico por parte del farmacéutico de atención primaria (FAP).Diseño: Ensayo clínico abierto aleatorizado con grupo control. Emplazamiento: Este estudio se realizó en el ámbito de la atención primaria por un equipo multidisciplinar de 13 centros de salud pertenecientes a 4 direcciones asistenciales de la Comunidad de Madrid durante el año 2021. Participantes: Pacientes polimedicados (60-74 años) que aceptasen participar y fueran clasificados como no adherentes según el test Morisky-Green. Se captaron 224 pacientes, de los cuales 87 fueron no adherentes. De estos se perdieron 15, aleatorizándose finalmente 72. Terminaron el estudio 71 pacientes, 33 en el grupo intervención y 38 en el grupo control. Intervenciones: Al grupo intervención se le incluyó en un programa de seguimiento telefónico que consistía en una entrevista para mejorar la adherencia en los meses 1, 2 y 3. Para evaluar la mejora se repetía el test Morisky-Green al 4.° mes. En el control únicamente se realizó el test de AT al 4.° mes. Mediciones principales: Adherencia, medida por el Morisky-Green al inicio y a los 4 meses. Resultados: En el grupo intervención el 72,7% de los pacientes pasaron a ser adherentes, mientras que en el grupo control el 34,2%, siendo la diferencia del 38,5% (IC 95%: 17,1-59,9) estadísticamente significativa (p=0,001). Conclusiones: Tras la intervención de seguimiento telefónico educacional-conductual a pacientes no adherentes por parte del FAP se mejora la AT de forma estadísticamente significativa en el grupo intervención respecto al grupo control.(AU)

Objective: Evaluate the improvement in therapeutic adherence of an intervention based on telephone monitoring by the primary care pharmacist. Design: Randomized open controlled trial. Location: This study was carried out in 2021 by a multidisciplinary team working with health professionals of thirteen health centers belonging to four health districts in Community of Madrid, Spain. ParticipantsThese were patients (60-74 years) with polipharmacy classified as non-adherent according to the Morisky-Green test. Two hundred and twenty-four patients were originally enrolled, 87 of them were non-adherents. Of these, 15 were lost and 72 were finally randomized. Seventy-one patients completed the study (33 intervention group and 38 control group). Interventions: Patients randomized to the intervention arm were included in a follow-up telephone program consisting of an interview at months 1, 2, and 3 to improve adherence. The Morisky-Green test was repeated at month 4 to assess improvement. In the control group this test was only performed at month 4. Main measurements: Adherence measured by Morisky-Green at baseline and at 4th month. Results: The 72.7% of patients in the intervention group became adherent while only 34.2% did in the control arm, being the difference 38.5% (95% CI: 17.1-59.9), statistically significant (P=.001). Conclusions: After a follow-up educational-behavioral telephone intervention in non-adherent patients by the primary care pharmacist, therapeutic adherence was improved statistically significantly in the intervention group compared with the control group.(AU)

[Hyponatremia induced by high-dose cyclophosphamide therapy: a retrospective cohort study Cyclophosphamide and Hyponatremia]. / Hiponatremia inducida por Ciclofosfamida en altas dosis: estudio de cohorte retrospectiva.

Cyclophosphamide-associated hyponatremia is an uncommon adverse effect. Published reports are inconclusive regarding patients under high-dose therapy. OBJECTIVE: To determine the incidence of hyponatremia in patients under high-dose cyclophosphamide therapy. METHODS: Retrospective cohort of adult patients who received high-dose cyclophosphamide therapy between 2010 and 2014 at the Hospital Italiano de Buenos Aires. The primary end-point was hyponatremia (defined as plasma sodium levels < 135 mEq/L), and secondary end-points were symptomatic hyponatremia, severe hyponatremia (plasma sodium levels < 120 mEq/L). RESULTS: A total of 96 patients received high-dose cyclophosphamide therapy during the study period. 27 patients met exclusion criteria and accounted for 28.1% , so that 69 patients were included in the primary analysis. Study population mean age was 56.5 years (SD 12.8) and 37.6% were female. The cumulative incidence were as follows: 52% (CI 95% 39¬­64) for hiponatremia, 5.8% (CI 95 % 0 ­12) for severe hyponatremia, and 8.7% (CI 95% 1.3­16) for symptomatic hyponatremia. The only independent variable associated with the development of hyponatremia was female gender (OR 3.89, CI 95% 1.02­8.55, p=0.04). Cumulative incidence found in this study appears higher than in previous reports, probably because only patients under high-dose cyclophosphamide therapy were included. Cumulative incidence of severe and symptomatic hyponatremia were lower

La hiponatremia asociada al uso de Ciclofosfamida es un efecto adverso infrecuente. Los estudios publicados no son concluyentes, especialmente respecto de los pacientes sometidos a dosis altas de dicho fármaco. Objetivos: Determinar la incidencia de hiponatremia en una subpoblación de pacientes que realizaron tratamiento con dosis altas de Ciclofosfamida. Materiales y Métodos: Estudio de cohorte retrospectivo de pacientes adultos que recibieron Ciclofosfamida a altas dosis en el período 2010-2014 en el Hospital Italiano de Buenos Aires. El evento primario fue la hiponatremia (definida como sodio plasmático menor a 135 meq/L) mientras que como eventos secundarios se tomaron la hiponatremia sintomática y la hiponatremia severa (valor menor 120 meq/L). Resultados: 96 pacientes recibieron altas dosis de Ciclofosfamida durante el período estudiado. Se excluyó un 27.4% de los pacientes por cumplir con los criterios de exclusión y se analizaron los datos de 69 pacientes. La edad media de la población fue de 56.5 años (DS 12.8) y un 37.7% de los pacientes eran mujeres. La incidencia de hiponatremia fue de 52% (IC95% 39-64), hiponatremia severa 5.8% (IC95% 0-12) y sintomática 8.7% (IC95% 1.3-16). La única variable asociada independientemente al desarrollo de hiponatremia fue el sexo femenino (OR: 3.89, IC95% 1.02-8.55, p=0.04). Si bien la incidencia observada resulta mayor a la observada en reportes previos, probablemente se deba a que solo consideramos la infusión de Ciclofosfamida a altas dosis. La hiponatremia severa y sintomática fue menor, aunque un 20% de los pacientes debieron prolongar su internación por esta causa.

Asociación entre la variación de glóbulos blancos y las manifestaciones clínicas, anticuerpos e infecciones en adultos con lupus eritematoso sistémico / White blood cells count variation in systemic lupus erythematosus: its association to disease manifestations, antibodies and risk of infections

Introducción: la asociación de leucopenia, linfopenia y neutropenia con la presencia de autoanticuerpos, manifestaciones clínicas e infecciones en pacientes con lupus eritematoso sistémico (LES) no está bien establecida. Los objetivos de este estudio fueron analizar los cambios en los recuentos de leucocitos y linfocitos en pacientes con LES y su asociación con manifestaciones clínicas, autoanticuerpos y riesgo de infecciones. Materiales y métodos: se recolectaron retrospectivamente los valores de leucocitos, linfocitos y neutrófilos. Se agruparon a los pacientes en cinco categorías: recuento de glóbulos blancos normales, leucopenia (persistente o intermitente) y linfopenia (persistente o intermitente). Se registraron las manifestaciones clínicas, los autoanticuerpos acumulados, el daño, la mortalidad, las infecciones y los tratamientos inmunosupresores recibidos de cada paciente. Resultados: se incluyeron 89 pacientes. La linfopenia (89%) fue la anormalidad más frecuente. La leucopenia intermitente y la persistente se detectaron en el 44% y en el 11% de los pacientes, respectivamente. La linfopenia intermitente y la persistente se hallaron en el 44% y en el 45% de los casos. En el análisis univariado, la presencia de rash discoide se asoció a leucopenia (20,4 vs. 5,1; p=0,059) y el tratamiento con mofetil micofenolato a un recuento normal de leucocitos (p=0,046). El compromiso neurológico se asoció a recuento normal de linfocitos (22,2% vs. 0% y 7,5%; p=0,027); los pacientes con anti-RNP (anti ribonucleoproteína nuclear) presentaron más frecuentemente linfopenia persistente (47% vs. 15,4% y 20%; p=0,007). Ninguno de los grupos se asoció a una mayor prevalencia de infecciones. En el análisis multivariado, el mofetil micofenolato se asoció negativamente a leucopenia (OR 0.33 IC 95% 0,1-0,9; p=0,042) y el compromiso neurológico se asoció negativamente a linfopenia (OR 0.08; p=0,022). Conclusiones: en el análisis univariado, el rash discoide se asoció a leucopenia y el anti-RNP a linfopenia. Al ajustar por otras variables significativas, el tratamiento con mofetil micofenolato se asoció a un recuento normal de leucocitos, mientras que las manifestaciones neurológicas se relacionaron a linfocitos normales. No se demostró asociación de las infecciones con ninguno de los grupos.

Introduction: leukopenia, lymphopenia and neutropenia association to clinical manifestations and infections in systemic lupus erythematosus (SLE) is not well defined. The objectives were to analize leucocytes and lymphocytes variations in SLE patients and their association to clinical manifestations, autoantibodies and infections risk. Materials and methods: total white blood cell (WBC) count, lymphocyte, and neutrophils counts were collected retrospectively. Data were grouped into normal WBC cell count, persistent or intermittent leucopenia and lymphopenia. Disease manifestations, accumulated autoantibodies, damage, mortality, infections and immunosuppressants ever received were registered. Results: study sample included 89 patients. Lymphopenia (89%) was the most common abnormality. Intermittent and persistent leukopenia were detected in 44% and 11% cases. Intermittent and persistent lymphopenia were found in 44% and 45% cases. In univariate analysis, discoid rash was associated to leukopenia (20.4 vs 5.1 p=0.059) and mycophenolate treatment to normal leukocyte count (p=0.046). Patients with neurological disorder tended to have normal lymphocyte counts rather than intermittent or persistent lymphopenia (22.2% vs 0% and 7.5% p=0.027); patients with anti-RNP tended to belong to the persistent lymphopenia group (47% vs 15.4% and 20% p=0.007). Infections were not associated to any of the categories. In multivariate analysis mycophenolate was negatively associated to leukopenia (OR 0.33 95% CI 0.1-0.9 p=0.042) while neurological disorder was negatively associated to lymphopenia (OR 0.08 p=0.022). Conclusions: in univariate analysis, discoid rash was associated to leukopenia and anti-RNP to lymphopenia. When adjusted to other significant variables, mycophenolate was related to normal leukocyte while neurological manifestations were to normal lymphocyte counts. Infections were not associated to any of the categories.

Lupus en Argentina. Pacientes no respondedores al tratamiento estándar y belimumab como posible opción. Datos del registro RELESSAR / Lupus in Argentina. Non-responders to standard treatment and belimumab as a possible option. RELESSAR record data

Introducción: el lupus es una enfermedad compleja y varias veces de difícil abordaje. Alcanzar la remisión es uno de los objetivos, incorporando opciones terapéuticas. Objetivos: describir las características generales de los pacientes según el estado de la enfermedad y el uso de belimumab. Materiales y métodos: estudio de corte transversal, registro RELESSAR. Se definió el estado de la enfermedad como: remisión: SLEDAI=0 y sin corticoides; baja actividad de la enfermedad: SLEDAI >0 y ≤4 y sin corticoides; control no óptimo: SLEDAI >4 y cualquier dosis de corticoides. Resultados: se incluyeron 1.277 pacientes, 23,4% en remisión, 12,6% en baja actividad y 63,8% con control no óptimo. En este último grupo eran más jóvenes y con menor duración de la enfermedad; presentaban mayores índices de actividad y cronicidad, y mayor empleo de inmunosupresores. Solo el 22,3% de los pacientes con criterio potencial de uso de belimumab (lupus eritematoso sistémico activo a pesar del tratamiento estándar) lo recibía en ese momento. Las variables asociadas a hospitalizaciones fueron: terapia con corticoides, ciclofosfamida y mayor SLICC. Conclusiones: se refleja la complejidad del manejo de estos pacientes y se visualizan aspectos estructurales como la desigualdad. El uso del belimumab resultaría beneficioso en los pacientes seleccionados.

Introduction: lupus is a complex disease and often difficult to approach. Achieving remission is one of the objectives, incorporating therapeutic options. Objectives: to describe the characteristics of the patients and the use of belimumab, according to the status of the disease. Materials and methods: cross-sectional study. Patients of the RELESSAR registry. Stratification: Remission: SLEDAI=0 and without corticosteroids. Low disease activity SLEDAI> 0 and ≤4 and without corticosteroids and non-optimal control: SLEDAI> 4 and any dose of corticosteroids. Results: a total of 1,277 patients were included, 23.4% in remission, 12.6% in low disease activity and 63.8% in non-optimal control. The last group was younger and had a shorter duration of the disease. They had higher activity and chronicity indices and greater use of immunosuppressants. Only 22.3% of the patients with potential criteria for the use of belimumab (activity disease despite standard treatment) were receiving it. The variables associated with hospitalizations were: corticosteroids, cyclophosphamide and higher SLICC. Those associated with severe infection: mycophenolate mofetil, azathioprine, corticosteroids, and higher SLICC. Conclusions: the complexity of the management of these patients is reflected, visualizing structural aspects such as inequality. The use of belimumab could be beneficial in selected patients.

Lupus en Argentina. Pacientes no respondedores al tratamiento estándar y belimumab como posible opción. Datos del registro RELESSAR / Lupus in Argentina. Non-responders to standard treatment and belimumab as a possible option. RELESSAR record data

Introducción: el lupus es una enfermedad compleja y varias veces de difícil abordaje. Alcanzar la remisión es uno de los objetivos, incorporando opciones terapéuticas. Objetivos: describir las características generales de los pacientes según el estado de la enfermedad y el uso de belimumab. Materiales y métodos: estudio de corte transversal, registro RELESSAR. Se definió el estado de la enfermedad como: remisión: SLEDAI=0 y sin corticoides; baja actividad de la enfermedad: SLEDAI >0 y ≤4 y sin corticoides; control no óptimo: SLEDAI >4 y cualquier dosis de corticoides. Resultados: se incluyeron 1.277 pacientes, 23,4% en remisión, 12,6% en baja actividad y 63,8% con control no óptimo. En este último grupo eran más jóvenes y con menor duración de la enfermedad; presentaban mayores índices de actividad y cronicidad, y mayor empleo de inmunosupresores. Solo el 22,3% de los pacientes con criterio potencial de uso de belimumab (lupus eritematoso sistémico activo a pesar del tratamiento estándar) lo recibía en ese momento. Las variables asociadas a hospitalizaciones fueron: terapia con corticoides, ciclofosfamida y mayor SLICC. Conclusiones: se refleja la complejidad del manejo de estos pacientes y se visualizan aspectos estructurales como la desigualdad. El uso del belimumab resultaría beneficioso en los pacientes seleccionados.

Introduction: lupus is a complex disease and often difficult to approach. Achieving remission is one of the objectives, incorporating therapeutic options. Objectives: to describe the characteristics of the patients and the use of belimumab, according to the status of the disease. Materials and methods: cross-sectional study. Patients of the RELESSAR registry. Stratification: Remission: SLEDAI=0 and without corticosteroids. Low disease activity SLEDAI> 0 and ≤4 and without corticosteroids and non-optimal control: SLEDAI> 4 and any dose of corticosteroids. Results: a total of 1,277 patients were included, 23.4% in remission, 12.6% in low disease activity and 63.8% in non-optimal control. The last group was younger and had a shorter duration of the disease. They had higher activity and chronicity indices and greater use of immunosuppressants. Only 22.3% of the patients with potential criteria for the use of belimumab (activity disease despite standard treatment) were receiving it. The variables associated with hospitalizations were: corticosteroids, cyclophosphamide and higher SLICC. Those associated with severe infection: mycophenolate mofetil, azathioprine, corticosteroids, and higher SLICC. Conclusions: the complexity of the management of these patients is reflected, visualizing structural aspects such as inequality. The use of belimumab could be beneficial in selected patients.

Variabilidad de la prestación farmacéutica a centros sociosanitarios residenciales desde los servicios de farmacia de hospital / Variability of pharmaceutical provision to nursing homes from hospital pharmacy services

Objetivo: Analizar la situación y la posible variabilidad de la prestación farmacéutica a centros sociosanitarios residenciales públicos incluidos en un proyecto piloto vinculados a un servicio de farmacia de hospital del Servicio Andaluz de Salud. Método: Estudio multicéntrico transversal. Se diseñó un cuestionario que incluye preguntas englobadas en: características del centro, legislación, guía farmacoterapéutica y prescripción, preparación y transporte, dispensación, administración, actividad farmacéutica y datos asistenciales. Resultados: Se incluyeron los 13 centros del proyecto piloto; todos respondieron las 36 preguntas del cuestionario. Todos disponían de depósito de medicamentos. Todos dispensaban en dosis unitaria individualizada. Tres centros sociosanitarios no disponían de puesto de trabajo para el farmacéutico. El farmacéutico no se desplazaba al centro en tres de ellos. Conclusiones: Aunque existe cierta variabilidad en la prestación farmacéutica a los centros sociosanitarios del pilotaje, se han identificado tanto fortalezas (por ejemplo, dispensación de medicamentos en dosis unitaria), como puntos de mejora (como el aumento de la presencia del farmacéutico en los centros)

Objective: To analyse the situation and the possible variability of pharmaceutical provision to public nursing homes included in the "Project pilot" from hospital pharmacy services of the Andalusian Health Service. Method: Cross-sectional multicenter study. A questionnaire of 36 questions including: nursing homes characteristics, legislation, pharmacotherapeutic guide and prescription, preparation and transport, dispensation, administration, pharmaceutical activity and healthcare data. Results: We included 13 nursing homes. Everyone had a medicines store. All dispensed in individualized unit dose. Three nursing homes did not have a work space for the pharmacist. The pharmacist did not move to the nursing home in three of them. Conclusions: Although there is variability in the pharmaceutical provision to nursing homes included in the "Pilot Project", we have identified strengths (unit dose system of medication distribution) and opportunities to improve (increase of the time of presence of the pharmacist in the nursing homes)

Enfermedad de Darier. Presentación en dos hermanos / Darier's disease. Report in two brothers

La enfermedad de Darier (ED) o disqueratosis folicular fue descripta por Darier y White en 1889. Se trata de una alteración de herencia autosómica dominante, causada por la mutación del gen ATP2A2. Esta mutación interfiere en los procesos de diferenciación y crecimiento celular calcio dependientes. El diagnóstico es clínico e histopatológico. Se manifiesta con pápulas y costras eritematoparduzcas queratósicas, pruriginosas, localizadas principalmente en áreas seborreicas, pápulas blanquecinas en mucosa oral y alteraciones ungueales. Presentamos dos casos de ED en dos hermanos y realizamos una revisión de esta entidad (AU)

Darier's disease (DD) or keratosis follicularis was described by Darier and White in 1889. It is an autosomic dominant inheritance disorder caused by mutation of ATP2A2 gene. This mutation interferes in the processes of cell differentiation and calcium dependent growth. Diagnosis is clinical and histopathological. Clinical features include itchy keratotic papules and crusts, located mainly on seborrheic areas, whitish papules on oral mucosa and nail changes. Two cases of DD in two brothers is reported and a review of this entity is made (AU)