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Introducción: los ImTOR, sirolimus y everolimus son una alternativa de inmunosupresión en personas que han recibido transplantes renales. En este artículo, se describe la experiencia de pacientes que han experimentado una conversión a ImTOR, y a los que se les ha hecho un seguimiento por más de cinco años.Materiales y métodos: se incluyeron pacientes con transplantes renales desde 1995 hasta 2013, quienes tuvieron indicación de suspensión del inhibidor de calcineurina (ICN) después del tercer mes posterior al trasplante. Todos los pacientes fueron sometidos a biopsia renal antes de la administración de ImTOR. Ningún paciente tuvo diagnóstico de nefropatía crónica, IFTA >40 % o proteinuria >350 mg/24h. Se elaboró un análisis descriptivo para todas las variables. Para estudiar la supervivencia del paciente y del injerto, y la incidencia de rechazo agudo, se usó el método de Kaplan-Meier.Resultados: de 1273 trasplantes renales, la conversión de ICN a ImTOR se realizó en 166 casos (13 %). Al 78 % (n=129) se le administró sirolimus. El 13 % de los pacientes perdió la función del injerto y 7 pacientes (4,2 %) fallecieron. En el 37 % de los casos, se retiró el ImTOR. La principal causa de retiro fue el hallazgo de proteinuria patológica. La incidencia de rechazo agudo después del cambio a ImTOR fue de 9,6 %. La supervivencia del injerto tras uno y cinco años fue de 96,6 % y 83,5 %, respectivamente; y la supervivencia del paciente a uno y cinco años fue de 98 % y 97 %, respectivamente.Conclusiones: el uso de inhibidores ImTOR parece ser seguro en este grupo de pacientes trasplantados, pues hubo una baja tasa de rechazo y buena supervivencia del injerto.

Background:Cutaneous manifestations are observed in 59–85% of patients with SLE but less than 5% developed BSLE. In the GLADEL cohort, the prevalence is 0.41%. BSLE literature in children is scarceObjectives:to describe the clinical characteristics of the patients with BSLEMethods:series of cases between 2010-2019 of two reference centers. The cases met Camisa and Grimwood criteria for BSLEResults:5 cases had bullous lesions that resolved with residual hypopigmentation. One case had focal seizure and other patient had arthritis with leukopenia and thrombocytopenia. 2 patients had proteinuria <500 mg/24 hours. There were no cases of lupus nephritis. The median SLEDAI-2K score was 12 (IR: 8-17). All had ANAs in titers greater than 1:160 and four had anti-DNA (+). 5 patients had anti-RNP and 4 had anti-Sm. One case had anti-Ro/anti-La. All presented low C3 and 80% had low C4. 80% had ESR ≥20 mm/hour and CRP greater than 0.5 mg/dl in 60%. All presented clinical response with glucocorticoids and dapsone; one patient had methemoglobinemia that improved. At 3 months, the blisters did not recur in 4 patients except one case that presented relapse due to inadherence.Histologically, the most common finding was subepidermal blisters with neutrophils in the papilar dermis. DIF showed linear deposits of Igs and complement in 4 cases and granular deposits in one case; IgG/IgM were in 5 of the samples. IgA was positive in 60% and C3 in 80%Conclusion:In this series, BSLE was associated with neuropsychiatric, joint and haematological involvement in 40% of patients, without lupus nephritis. Such abnormalities had a parallel course to skin involvement, without recurrences. BSLE tends to have a single-phase behavior and in children unlike adults, severe renal involvement is uncommonReferences:[1]Pons-Estel GJ, et al. Lupus 2018; 27(10): 1753-1754Disclosure of Interests:None declared

Most autoimmune diseases (AIDs) during childhood debut with more severe and aggressive forms, with life-threatening conditions that increase the need for intensive care therapy. This study describes the clinical, laboratory, and health outcome features of pediatric patients with AIDs admitted to the pediatric intensive care unit (PICU). This is a retrospective cross-sectional study that included the clinical records of all pediatric patients with AIDs admitted to the PICU between 2011 and 2020 in Cali, Colombia. In total, 225 PICU admissions from 136 patients were evaluated. Median age was 13 (11–15) years, and the median disease duration was 15 (5–38.5) months. Systemic lupus erythematosus was the most prevalent disease (91, 66.9%), followed by vasculitis (27, 19.8%). The leading cause of PICU admission was AID activity (95, 44.3%). C-reactive-protein levels were associated with infections (p <0.0394). Mortality occurred in 12 (8.8%) patients secondary to AID activity, primarily, diffuse alveolar hemorrhage (6, 50%). A longer disease duration was associated with mortality (p <0.00398). AID activity was the leading cause of PICU admission and mortality. Pulse steroid therapy, mechanical ventilation, and inotropic and vasopressor support were associated with nonsurvival.

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