Complicaciones maternas y perinatales en gestantes con lupus: Estudio de casos y controles / Maternal and Perinatal Complications in Pregnant Women with Lupus: Case control Study

Objetivo: Determinar la frecuencia de complicaciones materno-perinatales y factores clínicos asociados a estos resultados en estantes con lupus. Métodos: Se realizó un estudio de casos y controles a partir de historias clínicas de pacientes con diagnóstico Lupus Eritematoso Sistémico en embarazo, entre 2010-2022 en una institución de salud en Medellín-Colombia. Éstas se clasificaron como casos (pacientes con resultados adversos materno-perinatales) y controles (pacientes sin resultados adversos). Resultados: Se incluyó un total de 67 pacientes (35 casos y 32 controles). Las complicaciones maternas más frecuentes fueron los trastornos hipertensivos asociados al embarazo (71,4 %), incluyendo preeclampsia y una presentación importante de partos pretérmino (68,6 %). La nefritis lúpica previa y durante el embarazo, fue más frecuente en los casos que en los controles (31,4 % versus 9,4 %). Los compromisos cardiovasculares, de mucosas y musculo-esquelético, fueron más frecuentes durante el embarazo (31,4 %, 40 % y 34,3 %, respectivamente), coincidiendo con mayor actividad del lupus, principalmente durante el embarazo. El compromiso cardiovascular y de mucosas durante el embarazo, así como tener síndrome antifosfolípido se relacionaron con desenlace materno-perinatal adverso. Conclusión: Componentes clínicos propios de la enfermedad como la nefritis lúpica, el síndrome antifosfolípido, el compromiso cardiovascular, y de mucosas podrían predisponer a desenlaces maternos y/o perinatales adversos como trastornos hipertensivos asociados al embarazo, pretérmino, restricción de crecimiento fetal, entre otros(AU)

Objective: To determine the frequency of maternal-perinatal complications and the clinical factors associated with these outcomes in pregnant women with lupus. Methods: A case-control study was conducted using the medical records of patients diagnosed with pregnancy and lupus in a healthcare institution in Medellin, Colombia, between 2010 and 2022. The patients were classified as cases (patients with adverse maternal-perinatal outcomes) and controls (patients without adverse outcomes). Results: A total of 67 patients (35 cases and 32 controls) were included. The most frequent maternal complications were pregnancyassociated hypertensive disorders (71.4%), including preeclampsia and a significant presentation of preterm deliveries (68.6%). Lupus nephritis prior to and during pregnancy was more frequent in cases than in controls (31.4% versus 9.4%). Cardiovascular, mucosal and musculoskeletal compromises were more frequent during pregnancy (31.4%, 40% and 34.3%, respectively), coinciding with greater lupus activity, mainly during pregnancy. Cardiovascular and mucosal involvement during pregnancy, as well as having antiphospholipid syndrome, were related to adverse maternal-perinatal outcome. Conclusion: Clinical components of the disease such as lupus nephritis, antiphospholipid syndrome, cardiovascular and mucosal involvement, are factors that may predispose these patients to adverse maternal and/or perinatal outcomes, such as hypertensive disorders associated with pregnancy, low birth weight, preterm, fetal growth restriction, among others(AU)

Lupus de inicio tardío en un grupo de pacientes paraguayos

Introducción: Los pacientes con lupus eritematoso sistémico (LES) diagnosticados después de los 50 años presentan una enfermedad menos severa y un curso clínico más leve. El objetivo de este estudio es describir las características clínicas y de laboratorios del LES en pacientes de edad avanzada. Material y Método: Estudio observacional, descriptivo, de corte trasverso, retrospectivo, de pacientes con el diagnóstico de LES, de inicio posterior a los 50 años de edad, que consultaron en el Hospital Nacional, en el periodo comprendido entre diciembre de 2016 y mayo de 2024. Resultados: Se estudiaron 30 pacientes entre 51 y 87 años (edad media: 62,5 años ± 8,5), 16 mujeres (53,3%) y 14 varones 14 (46,6 %). El tiempo de enfermedad previo al diagnóstico fue de 59,4 ± 8.3 (50-80) días. La duración de la enfermedad fue en promedio 5 años ± 5,1 (1-26). Las principales manifestaciones clínicas fueron las artralgias 26 (86,6%), artritis 22 (72,3%), pérdida de peso 10 (33,3%) y fiebre prolongada (30%). Presentaron comorbilidades 19 pacientes (63,3 %), siendo la hipertensión arterial la principal. El anti-DNA fue positivo en 12 pacientes (42,8%), el anti-Ro en 5/25 pacientes (20%), el anti-Sm en 2/26 (7,9%). La eritrosedimentación en la primera hora fue ≥ 20 mm en 17/23 (73,9%). El 100% recibió tratamiento con hidroxicloroquina, mientras que recibieron corticoides 26 (86.6%) pacientes, micofenolato mofetil 7 (24,4%), ciclofosfamida 4 (13,3%). La mortalidad fue del 6,6 %. Conclusión: Los principales hallazgos fueron artralgias y artritis, siendo menos frecuentes los casos graves. La mayoría presentó comorbilidades, siendo la hipertensión arterial la más frecuente. La mortalidad fue del 6,6% de causa cardiovascular.

Introduction: Patients with systemic lupus erythematosus (SLE) diagnosed after the age of 50 have a less severe disease and a milder clinical course. The objective of this study is to describe the clinical and laboratory characteristics of SLE in elderly patients. Material and Method: Observational, descriptive, cross-sectional, retrospective study of patients with the diagnosis of SLE, with onset after 50 years of age, evaluated at the National Hospital, in the period between December 2016 and May of 2024. Results: 30 patients between 51 and 87 years old (mean age: 62.5 years ± 8.5) were studied, 16 women (53.3%) and 14 men (46.6%). The time to diagnosis was 59.4 ± 8.3 (50-80) days. The duration of the disease was on average 5 years ± 5.1 (1-26). The main clinical manifestations were arthralgia in 26 (86.6%), arthritis in 22 (72.3%), weight loss in 10 (33.3%) and prolonged fever (30%). Nineteen patients (63.3%) had comorbidities, the main one being high blood pressure. Anti-dsDNA was positive in 12 patients (42.8%), anti-Ro in 5/25 patients (20%), anti-Sm in 2/26 (7.9%). The erythrocyte sedimentation rate was ≥ 20 mm in 17/23 (73.9%). All patients were treatment with hydroxychloroquine, 26 (86.6%) patients received corticosteroids, 7 (24.4%) mycophenolate mofetil, 4 (13.3%) cyclophosphamide. Mortality was 6.6%. Conclusion: The main findings were arthralgia and arthritis, with severe cases being less frequent. The majority presented comorbidities, with high blood pressure being the most common. Mortality was 6.6% due to cardiovascular causes.

Papel del IFN-λ en la patogénesis del Lupus eritematoso sistémico: mecanismos Biomoleculares

Introducción: El lupus eritematoso sistémico (LES) es una enfermedad autoinmune que causa inflamación sistémica y alteraciones en la tolerancia inmunológica. La activación de los genes inducibles por interferón (IFN), contribuye en más del 50% de su patogenia. Objetivo: relacionar el papel del IFN-λ en la patogenia del LES. Materiales y Métodos: Búsqueda sistémica en base de datos; a través de las palabras claves del MeSH and DeCS. Fue incluido adicionalmente la palabra "Interferón Lambda". Resultados: Se encontró que la producción aberrante de interferón tipo I contribuye a la desregulación de IFN-λ, producido principalmente por células dendríticas plasmocitoides. Este proceso conduce a la estimulación inmunológica por autoanticuerpos y a un aumento de IFNλR-1 en células B, potenciando la generación de anticuerpos. IFN-λ3 se asocia particularmente con nefritis lúpica, y el IFN-λ en general aumenta la expresión de MHC-I, intensificando la respuesta de células T CD8+ y posiblemente afectando la tolerancia central y la regulación en el timo. Conclusión: Se destaca que el IFN-λ favorece la activación inmune, formación de inmunocomplejos, inflamación crónica y producción de autoanticuerpos, vinculándose niveles altos de IFN-λ3 con mayor actividad de la enfermedad.

Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease that causes systemic inflammation and alterations in immunological tolerance. The activation of interferon (IFN)-inducible genes contributes to more than 50% of its pathogenesis. Objective: to review the role of IFN-λ in the pathogenesis of SLE. Materials and Methods: Systemic search in database; through the MeSH and DeCS keywords. The word "Lambda Interferon" was additionally included. Results: Aberrant production of type I interferon was found to contribute to the deregulation of IFN-λ, produced mainly by plasmacytoid dendritic cells. This process leads to immunological stimulation by autoantibodies and an increase in IFNλR-1 in B cells, enhancing the generation of antibodies. IFN-λ3 is particularly associated with lupus nephritis, and IFN-λ generally increases MHC-I expression, enhancing the CD8+ T cell response and possibly affecting central tolerance and regulation in the thymus. Conclusion: It is highlighted that IFN-λ favors immune activation, formation of immune complexes, chronic inflammation and production of autoantibodies, linking high levels of IFN-λ3 with greater disease activity.

Síndrome antifosfolipídico trombótico en una mujer con enfermedad mixta del tejido conectivo. Reporte de caso / Thrombotic antiphospholipid syndrome in a woman with mixed connective tissue disease. Case report

Introducción: El síndrome de anticuerpos antifosfolípidos es una enfermedad au-toinmune sistémica poco frecuente, produce hipercoagulabilidad con riesgo de trombosis. Para el diagnóstico se utilizan los criterios ACR/EULAR APS del 2023. El tratamiento es anticoagulantes y antiagregantes plaquetarios. La enfermedad mixta del tejido conectivo es enfermedad autoinmunitaria sistémica con la asociación de manifestaciones clínicas de otras entidades autoinmunes. Objetivo:Describir la presentación de dos enfermedades sistémicas autoinmunes poco frecuentes en conjunto, con el propósito de contribuir con un enfoque prác-tico para el diagnóstico y manejo. Presentación del caso: Se describe una paciente de 37 años que presentó un episodio de tromboembolia pulmonar secundario a síndrome de anticuerpos anti-fosfolípidos y en los 6 meses previos tuvo síntomas compatibles con enfermedad mixta del tejido conectivo. Discusión: La presencia de dos entidades autoinmunes, síndrome de anticuerpos antifosfolípidos y enfermedad mixta del tejido conectivo presentadas en conjunto y cuyo debut de complicaciones fue una tromboembolia pulmonar, encontrándo-se presencia de múltiples autoanticuerpos positivos entre estas anticuerpos an-tifosfolipídicos y anti-U1 snRNP, es un reto diagnóstico al diferenciar entre otras enfermedades del tejido conectivo como lupus eritematoso sistémico, esclerosis sistémica cutánea, enfermedad mixta del tejido conectivo y artritis reumatoide. El tratamiento se basó en las características del paciente y su condición clínica al momento del diagnóstico. Conclusiones: El síndrome de anticuerpos antifosfolipídicos conlleva la presencia de un episodio trombótico, por otro lado, su asociación con una enfermedad mixta del tejido conectivo es poco frecuente y puede aumentar su morbimortalidad.

Introduction: Antiphospholipid antibody syndrome is a rare systemic autoimmu-ne disease that produces Antiphospholipid antibody syndrome is a rare systemic autoimmune disease that causes hypercoagulability with risk of thrombosis. For diagnosis, the ACR/EULAR APS 2023 criteria are used. Treatment is anticoagulants and antiplatelet agents.Mixed connective tissue disease is a systemic autoimmune disease with the asso-ciation of clinical manifestations of other autoimmune entities.Objective:To describe the presentation of two rare autoimmune systemic diseases toge-ther, with the purpose of contributing a practical approach to diagnosis and management.Case presentation: 37-year-old patient with an episode of pulmonary thromboem-bolism secondary to antiphospholipid antibody syndrome and in the previous 6 months he had symptoms compatible with mixed connective tissue disease.Discussion:The presence of two autoimmune entities, antiphospholipid antibody syndrome and mixed connective tissue disease presented together and whose de-but of complications was a pulmonary thromboembolism, finding the presence of multiple positive autoantibodies between these antiphospholipid antibodies and an-ti-U1 snRNP, is a diagnostic challenge in differentiating between other connective tissue diseases such as systemic lupus erythematosus, cutaneous systemic sclero-sis, mixed connective tissue disease and rheumatoid arthritis. Treatment was based on the patient's characteristics and clinical condition at the time of diagnosis.Conclusions: Antiphospholipid antibody syndrome entails the presence of a thrombotic episode; on the other hand, its association with a mixed connective tissue disease is rare and may increase its morbidity and mortality.

Value of precision nutrition in the inflammatory control of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of autoantibodies directed against endogenous antigens causing various clinical manifestations, chronic inflammation and tissue damage. Although the pathophysiology of SLE remains unknown, it is recognized that genetic, epigenetic, environmental and neuroendocrine factors are involved in the development of the disease and its complications. A notable proportion of patients with SLE also present obesity, and this dysmetabolic profile can cause renal, musculoskeletal and/or respiratory deterioration, fatigue, various pathophysiological alterations and functional deterioration. In this context, precision nutrition emerges as a promising tool in the inflammatory control of SLE, especially in patients with associated obesity. Various studies demonstrate the beneficial influence of balanced dietary patterns in macronutrients with foods rich in fiber, vitamins, minerals, antioxidants and polyphenols on the inflammatory control of SLE and the most diverse pathologies, highlighting the Mediterranean diet and plant-based diets. Finally, the intestinal microbiota may play a relevant role in this clinical scenario, since dysbiosis is associated with inflammatory processes and immune deregulation. It is believed that precision nutrition can modulate inflammatory profiles and immune dysfunctions to ensure better quality of life and metabolic well-being of SLE patients with the support of precision omics technologies.

El lupus eritematoso sistémico (LES) es una enfermedad autoinmune caracterizada por la producción de autoanticuerpos dirigidos contra antígenos endógenos causando diversas manifestaciones clínicas, inflamación crónica y daño tisular. Aunque la fisiopatología del LES sigue siendo desconocida, se reconoce que factores genéticos, epigenéticos, ambientales y neuroendocrinos están implicados en el desarrollo de la enfermedad y sus complicaciones. Una proporción notable de pacientes con LES presenta también obesidad, y este perfil dismetabólico puede producir deterioro renal, musculoesquelético y/o respiratorio, fatiga, diversas alteraciones fisiopatológicas y deterioro funcional. En este contexto, la nutrición de precisión emerge como una herramienta prometedora en el control inflamatorio del LES, especialmente en pacientes con obesidad asociada. Diversos estudios demuestran la influencia beneficiosa de patrones dietéticos equilibrados en macronutrientes con alimentos ricos en fibra, vitaminas, minerales, antioxidantes y polifenoles en el control inflamatorio del LES y de las más diversas patologías, destacando la dieta Mediterránea y las dietas basadas en plantas/vegetales. Por último, la microbiota intestinal puede tener un papel relevante en este escenario clínico, ya que la disbiosis se asocia con procesos inflamatorios y desregulación inmune. Se cree que con la nutrición de precisión se pueden modular los perfiles inflamatorios y las disfunciones inmunitarias para garantizar una mejor calidad de vida y el bienestar metabólico de los pacientes con LES con el apoyo de las tecnologías de precisión ómicas.

Prevalence of neonatal lupus in a small group of antibody-carrying mothers and frequency of complete atrioventricular block / Prevalencia de lupus neonatal en un pequeño grupo de madres portadoras de anticuerpos y frecuencia de bloqueo auriculoventricular completo

Abstract Background: Neonatal lupus (NL) is extremely rare and is caused by the transplacental passage of maternal IgG autoantibodies against Ro, La, and/or RNP proteins into the fetal circulation, which can cause congenital complete atrioventricular block (CCAB), permanent skin lesions, and liver involvement. Objective: To know the prevalence of NL in patients with CCAB and the clinical course in long-term follow-up. Methods: From January 1992 to December 2017, patients with CCAB were included. The presence of anti-SSA/Ro and anti-SSB/La antinuclear antibodies in maternal serum confirmed NL. Results: Eight patients were included with a follow-up of 10 ± 6 years; NL was concluded in 62.5%; two were male. One of them was diagnosed in utero, two at birth, and a pacemaker was implanted in them, one at 12 years of age and another at 15. The other two cases were diagnosed at 18 and 26 years of age, and permanent pacemakers were implanted 8 and 5 years later, respectively. In one case, a definitive pacemaker was not implanted in a newborn with only 1 year of follow-up. At delivery, 60% of the mothers were free of rheumatic disease, and altogether, they all had 19 children; none of them presented NL manifestations. Conclusions: CCAB is rare and frequently associated with a maternal autoimmune disease, practically all of them will require a definitive pacemaker at some point in their lives.

Resumen Antecedentes: El lupus neonatal (LN) es extremadamente raro y es ocasionado por el paso transplacentario de auto-anticuerpos maternos IgG contra las proteínas Ro, La y/o RNP a la circulación fetal que puede ocasionar bloqueo aurículo-ventricular completo congénito (BAVCC) permanente, lesiones dérmicas y afectación hepática. Objetivo: Conocer la prevalencia de LN en paciente con BAVCC y la evolución clínica en un seguimiento a largo plazo. Métodos: De enero de 1992 a diciembre 2017 se incluyeron paciente con BAVCC. La presencia de anticuerpos antinucleares anti-SSA/Ro y anti-SSB/La en suero materno confirmó LN. Resultados: Ocho pacientes fueron incluidos con seguimiento de 10 ± 6 años, el 62.5 % con LN; dos fueron del sexo masculino. Uno diagnosticado in útero, dos al nacimiento, en ellos se implantó marcapaso; uno a los 12 años de edad y otro a los 15. Los otros dos casos fueron diagnosticados a los 18 y 26 años, se implantó marcapaso definitivo en ellos 8 y 5 años después respectivamente. En un caso no se implantó marcapaso definitivo; un recién nacido con solo un año de seguimiento. Al dar a luz, el 60 % de las madres estaban libres de enfermedad reumática y en conjunto todas tuvieron 19 hijos, ninguno de ellos presentó manifestaciones de LN. Conclusiones: El BAVCC es raro y frecuentemente está asociado a una enfermedad autoinmune materna, prácticamente todos requerirán de marcapaso definitivo en alguna época de su vida.

Seguridad de la vacunación COVID-19 en una muestra de pacientes brasileños con lupus eritematoso sistémico / Safety of COVID-19 vaccination in a sample of Brazilian patients with systemic lupus erythematosus patients

Objetivos: estudiar los efectos secundarios y el riesgo de exacerbación de la en-fermedad después de la vacunación COVID-19 en una muestra de pacientes con lupus eritematoso sistémico (LES). Materiales y métodos: estudio retrospectivo que investigó 101 pacientes con LES. Se determinó la actividad de la enfermedad mediante el Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) antes y después de dos dosis de la vacuna contra el SARS-COv-2. Se registraron los efectos secundarios después de la vacunación. Resultados: los pacientes que recibieron dos dosis de la misma vacuna fue-ron el 10,3% para CoronaVAc, el 42,2% para Pfizer y el 47,3% para AstraZeneca. Se detectaron efectos secundarios en el 76,2% y la mayoría fue leve/moderado. Los más frecuentes fueron: dolor local (62,3%), cefalea (36,6%) y fatiga (34,6%). El cambio en la mediana del SLEDAI antes de la primera dosis y después de la segunda no fue estadísticamente significativo (p=0,68). Solo el 4,1% de los individuos aumentó el SLEDAI ≥ de 3 puntos. Conclusiones: la vacunación contra la COVID-19 fue bien tolerada y segura en pacientes con LES.

Objectives: to study the side effects and the risk of disease flare after COVID-19 vaccination in Brazilian patients with Systemic Lupus erythematosus (SLE). Materials and methods: this retrospective study investigated a sample of 101 SLE patients for disease activity through the SLE disease activity index (SLEDAI) prior to and after two vaccine doses against SARS-COv-2. Side effects after vaccination were recorded. Results: in this sample, patients receiving two doses of the same vaccine were 10.3% for CoronaVAc; 42.2% for Pfizer, and 47.3% for AstraZeneca. Side effects were detected in 76.2% of them, and most of them were mild and moderate; the most common were local pain (62.3%), headache (36.6%), and fatigue (34.6%). The SLEDAI prior to the first dose and after the second dose did not change significantly (p=0.68). Only 4.1% of individuals had increased in SLEDAI ≥than 3 points. Conclusions: vaccination against COVID-19 was well tolerated and safe in SLE patients

IL-1β and IL-17 in cutaneous lupus erythematous skin biopsies: could immunohistochemicals indicate a tendency towards systemic involvement?

Abstract Background: Only a fraction of patients with cutaneous lupus erythematosus (CLE) will eventually progress toward systemic disease (SLE). Objective: To find inflammatory biomarkers which could predict the progression of cutaneous lupus erythematosus (CLE) into systemic lupus erythematosus (SLE) using immunohistochemical (IHC) assays. Methods: Immunohistochemical markers for cytotoxic, inflammatory, and anti-inflammatory responses and morphometric methods were applied to routine paraffin sections of skin biopsies, taken from lesions of 59 patients with discoid lupus, subacute lupus, and lupus tumidus. For the diagnosis of SLE, patients were classified by both the American College of Rheumatology (ACR-82) and the Systemic Lupus International Collaborating Clinics (SLICC-12) systems. Results: Skin samples from CLE/SLE +patients presented higher expression of IL-1β (ARC-82: p = 0.024; SLICC-12: p = 0.0143) and a significantly higher number of cells marked with granzyme B and perforin (ARC: p = 0.0097; SLICC-12: p = 0.0148). Biopsies from CLE/SLE- individuals had higher expression of IL-17 (ARC-82: p = 0.0003; SLICC-12: p = 0.0351) and presented a positive correlation between the density of granzyme A+and FoxP3+ cells (ARC-82: p = 0.0257; SLICC-12: p = 0.0285) and CD8+ cells (ARC-82: p = 0.0075; SLICC-12: p = 0.0102), as well as between granulysin-positive and CD8+ cells (ARC-82: p = 0.0024; SLICC-12: p = 0.0116). Study limitations: Patients were evaluated at a specific point in their evolution and according to the presence or not of systemic disease. The authors cannot predict how many more, from each group, would have evolved towards SLE in the following years. Conclusions: In this cohort, immunohistochemical findings suggested that patients with a tendency to systemic disease will show strong reactivity for IL-1β, while those with purely cutaneous involvement will tend to express IL-17 more intensely.

Infecciones más frecuentes en pacientes con Lupus Eritematoso Sistémico internados en el Servicio de Clínica Médica del Hospital Nacional / Most frequent infections in patients with Systemic Lupus Erythematosus admitted to the Medical Clinic Service of the Hospital Nacional

Introducción: el lupus eritematoso sistémico (LES) se conoce como una enfermedad autoinmune multisistémica, cuya causa es desconocida, se caracteriza por la presencia de complejos inmunes y autoanticuerpos. En series internacionales se describe una presentación de procesos infecciosos en estos pacientes hasta en un 75 % de los casos, las infecciones ocurren tanto al inicio de la enfermedad como en etapas tardías, y son causa directa de muerte en 30 % a 60 % de los casos y motivo de hospitalización hasta de 30 %. Objetivos: determinar los procesos infecciosos más frecuentes en pacientes con lupus eritematoso sistémico internados en el Servicio de Clínica Médica del Hospital Nacional en el periodo 2022-2023. Metodología: diseño observacional, descriptivo de corte transversal de pacientes con diagnóstico de lupus eritematoso sistémico según criterios de European League Against Rheumatism (EULAR)-2019 que se encuentran internados en el servicio de Clínica Médica del Hospital Nacional (Itaugua-Paraguay) en el periodo de 2022-2023. Resultados: la edad media de los pacientes fue de 34 años, con predominio del sexo femenino en el 88.18 %. Las infecciones del aparato respiratorio fue la más frecuentemente diagnosticada en 50 (45.45 %) pacientes, seguido por la infección de vías urinarias 47 (42.38 %) pacientes, el condicionante de riesgo predominante fue el uso de corticoides en un total 105 (96.40 %) pacientes, se estableció en un total de 97 (88.18 %) pacientes con antibioticoterapia, dentro del aspecto demográfico de la zona urbana 56 (50.90 %) pacientes y rural 54(49.1 %). Conclusión: la infección del aparato respiratorio fue la infección más frecuente, el condicionante de riesgo predominante es el uso de corticoides y recibieron antibioticoterapia la cual en monoterapia fue la más utilizada

Introduction: systemic lupus erythematosus (SLE) is known as a multisystem autoimmune disease, whose cause is unknown, and is characterized by the presence of immune complexes and autoantibodies. In international series, presentation of infectious processes is described in these patients in up to 75 % of cases. Infections occur both at the beginning of the disease and in late stages, and are a direct cause of death in 30 % to 60 % of patients. Cases and reason for hospitalization up to 30 %. Objectives: determine the most frequent infectious processes in patients with systemic lupus erythematosus admitted to the Medical Clinic Service of the Hospital Nacional in the period 2022-2023. Methodology: observational, descriptive cross-sectional design of patients with a diagnosis of systemic lupus erythematosus according to criteria of European League Against Rheumatism (EULAR)-2019 who are admitted to the Medical Clinic service of the Hospital Nacional (Itauguá-Paraguay) in the period of 2022-2023. Results: the average age of the patients was 34 years, with a predominance of the female sex in 88.18 %. Respiratory system infections were the most frequently diagnosed in 50 (45.45 %) patients, followed by urinary tract infection in 47 (42.38 %) patients. The predominant risk factor was the use of corticosteroids in a total of 105 (96.40 %) patients, it was established in a total of 97 (88.18 %) patients with antibiotic therapy, within the demographic aspect of the urban area 56 (50.90 %) patients and rural 54 (49.1 %). Conclusion: respiratory tract infection was the most frequent infection, the predominant risk factor is the use of corticosteroids and they received antibiotic therapy, which in monotherapy was the most used.

Seguridad de la vacunación COVID-19 en una muestra de pacientes brasileños con lupus eritematoso sistémico

Abstract Objectives: to study the side effects and the risk of disease flare after COVID-19 vaccination in Brazilian patients with Systemic Lupus erythematosus (SLE). Materials and methods: this retrospective study investigated a sample of 101 SLE patients for disease activity through the SLE disease activity index (SLEDAI) prior to and after two vaccine doses against SARS-COv-2. Side effects after vaccination were recorded. Results: in this sample, patients receiving two doses of the same vaccine were 10.3% for CoronaVAc; 42.2% for Pfizer, and 47.3% for Astra-Zeneca. Side effects were detected in 76.2% of them, and most of them were mild and moderate; the most common were local pain (62.3%), headache (36.6%), and fatigue (34.6%). The SLEDAI prior to the first dose and after the second dose did not change significantly (p=0.68). Only 4.1% of individuals had increased in SLEDAI ≥than 3 points. Conclusions: vaccination against COVID-19 was well tolerated and safe in SLE patients.

Resumen Objetivos: estudiar los efectos secundarios y el riesgo de exacerbación de la enfermedad después de la vacunación COVID-19 en una muestra de pacientes con lupus eritematoso sistémico (LES). Materiales y métodos: estudio retrospectivo que investigó 101 pacientes con LES. Se determinó la actividad de la enfermedad mediante el Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) antes y después de dos dosis de la vacuna contra el SARS-COv-2. Se registraron los efectos secundarios después de la vacunación. Resultados: los pacientes que recibieron dos dosis de la misma vacuna fueron el 10,3% para CoronaVAc, el 42,2% para Pfizer y el 47,3% para AstraZeneca. Se detectaron efectos secundarios en el 76,2% y la mayoría fue leve/moderado. Los más frecuentes fueron: dolor local (62,3%), cefalea (36,6%) y fatiga (34,6%). El cambio en la mediana del SLEDAI antes de la primera dosis y después de la segunda no fue estadísticamente significativo (p=0,68). Solo el 4,1% de los individuos aumentó el SLEDAI ≥ de 3 puntos. Conclusiones: la vacunación contra la COVID-19 fue bien tolerada y segura en pacientes con LES.

Vasculitis cerebelosa por lupus eritematoso sistémico y descompresión suboccipital: reporte de caso / Cerebellar vasculitis due to systemic lupus erythematosus and suboccipital decompression: case report

RESUMEN Las vasculitis de las arterias o vasculitis cerebelosas en el lupus eritematoso sistémico (LES) son extremadamente infrecuentes y podrían estar inmersas o formar parte de los casos de LES neuropsiquiátrico (LESNP), stroke cerebeloso en el LES y grupo de vasculitis del sistema nervioso central (SNC ) por LES. La adición del manejo quirúrgico a esta entidad, mediante descompresión suboccipital, ha sido reportada en muy pocas publicaciones. Se presenta el caso de una paciente mujer de 18 años con LES, LESNP, stroke cerebeloso, con alta sospecha de vasculitis inflamatoria de las arterias cerebelosas a quien, a pesar del manejo médico, se le brindó descompresión suboccipital. La vasculitis inflamatoria de las arterias cerebelosas en el LES es una causa infrecuente de stroke cerebeloso de elevada mortalidad. Existe limitada evidencia científica en la realización de la descompresión suboccipital de esta entidad.

ABSTRACT Arterial vasculitis or cerebellar vasculitis in systemic lupus erythematosus (SLE) are extremely infrequent, and these might be included within cases of neuropsychiatric SLE (NPSLE), cerebellar stroke in SLE, and within the group of central nervous system vasculitis due to SLE. The addition of surgical management to this condition has been reported in few publications. We present the case of a 18- year old female patient with SLE, NPSLE, and cerebellar stroke, highly suspicious of inflammatory vasculitis affecting cerebellar arteries, in whom, in spite of medical management, suboccipital decompression was performed. Inflammatory vasculitis of cerebellar arteries in SLE is an infrequent cause of cerebellar stroke, and it has elevated mortality rates. There is limited scientific evidence with respect to suboccipital decompression for the management of this condition.

Healthcare encounters and outcomes of Filipino patients with Systemic Lupus Erythematosus managed at a Tertiary COVID-19 Referral Center

Objective@#The coronavirus disease 2019 (COVID-19) pandemic has limited healthcare delivery for patients with chronic diseases, including Systemic Lupus Erythematosus (SLE). This study aims to describe the outcomes of patients with SLE in a national COVID-19 referral center in the Philippines.@*Methods@#A review of records of all adult patients with SLE seen in the University of the Philippines-Philippine General Hospital (UP-PGH) from March 2020 to December 2021 was done. Data about patient characteristics, health encounters, and outcomes before and after the first visit during the study period were extracted. Descriptive statistics were employed.@*Results@#Our population of 403 patients was predominantly young (mean age 34.53 ± 11.14 years), female, and unemployed. This consisted of 370 known cases of SLE, 92 were diagnosed in institutions outside UP-PGH, and 33 new patients. Over the 22-month study period, there were 2,093 medical encounters, most of which were teleconsultations (81.70%). During an average gap of 53.6 ± 26.7 weeks between the last consultation and the first visit within the pandemic study period, 84 patients (22.70%) discontinued at least one of their SLE control medications, 68 (18.38%) patients developed a lupus flare, and 79 (21.35%) were hospitalized for various reasons. On their return to the rheumatology clinic during the pandemic, 37.47% were in lupus flare, 28.29% needed to be hospitalized, and 20 died. However, 86.75% of flares were controlled. During subsequent health encounters, 48 patients had a new flare (43 of these were controlled) and 20 died. The most common reason for hospitalization (n=160) was lupus disease flare and the most common cause of death (n=40) was pneumonia. Sixty patients acquired COVID-19 infection from which most recovered and four died.@*Conclusion@#Audio teleconsultation was the most common method used by our lupus cohort to interact with their doctors during the pandemic. There was an average of a year-long interruption in medical care for 62.70%. More than a third developed a disease flare and 15% acquired COVID-19 but outcomes were good in more than 85%. Despite the challenges posed by the pandemic, the majority of our lupus cohort who were able to continue their treatment had favorable outcomes.

Research progress on the manifestations and prognosis of neonatal lupus erythematosus in various systems / 中国当代儿科杂志

Neonatal lupus erythematosus (NLE) is caused by the transmission of maternal anti-Ro/SSA antibodies, anti-La/SSB antibodies, and other autoantibodies to the fetus through the placenta. Usually, with the disappearance of autoantibodies in the children's body, abnormal changes in the mucocutaneous, blood system, and digestive system can spontaneously subside, but the damage to various systems caused by autoantibodies may persist for a long time. This article provides a comprehensive review of the manifestations and prognosis of NLE in various systems, including mucocutaneous, blood system, circulatory system, nervous system, digestive system, respiratory system, aiming to provide reference for clinical work.

Complete congenital heart block in the offspring of an asymptomatic woman with isolated high titer anti-ro antibody

@#Among pregnant women, 1-2% are anti-Ro positive and while half of them have symptoms of connective tissue disease, the rest are asymptomatic. The presence of anti-Ro is of concern because of the risk of congenital heart block in the child. We report the case of an asymptomatic 27-year-old G2P1(1001) woman, who presented with persistent fetal bradycardia in her 21st week of gestation (AOG) and was found to have elevated titers for anti-Ro (>320 U/ml). Hydroxychloroquine 200 mg/day and prednisone 10 mg/day were given from the 33rd week of gestation up until the delivery. At 37 weeks AOG, she delivered a live male neonate with a complete heart block. On the 6th day of life, the infant remained bradycardic, hence a pacemaker was inserted and heart rate maintained at 100-120 bpm. On subsequent follow-ups, the mother and child did not develop any systemic manifestations and the infant was thriving well. While a diseased condition may not be apparent in a pregnant anti-Ro positive woman, the risk of neonatal lupus (NL) is demonstrated in this patient’s case. This report illustrates how prenatal care of an asymptomatic woman led to the discovery of a fetal abnormality and served to prepare the family and the medical team to ably handle the birth and subsequent care of a neonate with NL.

Research progress on immunologic skin diseases in China / 中国免疫学杂志

Immunologic skin diseases encompass a spectrum of immune system-mediated autoimmune or inflammatory skin conditions,such as lupus erythematosus,psoriasis,atopic dermatitis,and vitiligo.Immunologic skin diseases are characterized by an unclear pathogenesis,complex disease processes,diverse clinical manifestations,and treatment difficulties,thereby presenting sig-nificant diagnostic and therapeutic challenges.Notably,Chinese researchers have achieved numerous innovative research findings on immunologic skin diseases in recent years.Over the past decade,Chinese scholars have contributed 11 919 SCI papers to the field of immune dermatosis,with more than 10 being published in the world's leading medical journals.These publications include one in Sci-ence,one in Nature,two in Cell,three in The New England Journal of Medicine,two in The Lancet,and one in Nature Medicine,as well as four in Immunity.Here,we aim to present a comprehensive summary of Chinese research progress pertaining to the pathogene-sis,diagnosis and treatment of immunologic skin diseases.

Clinical effect of Ziyin Qingshenyin on lupus nephritis with Yin deficiency and internal heat syndrome / 中国医科大学学报

Objective To observe the clinical effect of Ziyin Qingshenyin on lupus nephritis(LN)with Yin deficiency and internal heat syndrome.Methods Sixty patients diagnosed with LN were selected from June 2020 to May 2022 from our hospital as research partici-pants and randomly divided into control group(n=30)and combination group(n=30).The control and combination groups were adminis-tered conventional Western medicine and conventional Western medicine with Ziyin Qingshenyin,respectively.Clinical efficacy,traditional Chinese medicine(TCM)syndrome score and efficacy,renal function index[serum albumin(ALB),serum creatinine(sCr),hemoglobin(Hb),blood urea nitrogen(BUN),and 24 h urinary protein quantitation(24 h Upro)],immunological indicators[complement C3,com-plement C4,immunoglobulin(Ig)M,IgG,and IgA],inflammatoryindicators[C-reactive protein(CRP)and erythrocyte sedimentation rate(ESR)],disease activity score of systemic lupus erythematosus(SLEDAI),and safety were determined.Results The total effective rate(86.67%,83.33%)of the combination group was higher than that of the control group(63.33%,60.00%)(P＜0.05).Following treatment,the five syndrome scores of heat,sore throat,night sweats,and macula in combination group were lower than those in the control group(P＜0.05).After treatment,24 h Upro,BUN,sCr,IgM,IgG,IgA,CRP,ESR,and SLEDAI scores in the combination group were lower than those in control group,while ALB,Hb,complement C3,and complement C4 were higher than those in the control group(P＜0.05).There was no significant difference in the incidence of adverse reactions between the two groups(P＞0.05).Conclusion The Ziyin Qingshen-yin treatment has notable effects on LN with Yin deficiency and internal heat syndrome,which can improve renal function and immunolog-ical indexes,reduce inflammatory factors,and contribute to disease control.

The impact of apoB/apoA1 on lupus nephritis and renal insufficiency and its diagnostic value / 中国医师杂志

Objective:To analyze the impact and diagnostic value of apoB/apoA1 on lupus nephritis (LN) and its renal dysfunction.Methods:A total of 134 patients diagnosed with systemic lupus erythematosus (SLE) at the Taihe Hospital of Anhui University of Traditional Chinese Medicine from July 2019 to January 2023 were selected and divided into LN group ( n=82) and simple SLE group ( n=52). According to the glomerular filtration rate (e-GFR), LN was divided into a group with normal renal function ( n=42) and a group with renal insufficiency ( n=40). We compared the differences in clinical data between different groups and analyzed the diagnostic value of apoB, apoA1, and apoB/apoA1 for LN and its renal insufficiency. Results:The results of binary logistic regression analysis showed that apoB/apoA1, SLE disease activity index (SLEDAI), anti double stranded DNA (dsDNA), complement 3 (C3), and albumin (ALB) were independent influencing factors for LN ( OR=4.033, 1.179, 3.148, 0.374, 0.879, all P<0.05). The area under the curve (AUC) of apoB, apoA1, and apoB/apoA1 for diagnosing LN were 0.623, 0.662, and 0.742, respectively. The diagnostic efficacy of apoB/apoA1 was higher than that of apoB and apoA1, and the differences are statistically significant (all P<0.05). Regardless of whether confounding factors were adjusted or not, apoB/apoA1 were all risk factors for LN with e-GFR>60 ml/(min·1.73 m 2), e-GFR 30-60 ml/(min·1.73 m 2), and e-GFR<30 ml/(min·1.73 m 2) (all P<0.05). ApoB/apoA1, anti dsDNA, C3, and ALB were all independent influencing factors for renal insufficiency LN ( OR=3.778, 2.669, 0.415, 0.884, all P<0.05). The AUC for diagnosing renal insufficiency LN in apoB, apoA1, and apoB/apoA1 were 0.623, 0.640, and 0.730, respectively. The diagnostic efficacy of apoB/apoA1 was higher than that of apoB and apoA1, and the differences were statistically significant (all P<0.05). Conclusions:ApoB/apoA1 is an independent influencing factor for the occurrence of LN and renal insufficiency LN, and has good diagnostic value for LN and renal insufficiency LN.

Screening and analysis of ferroptosis related genes in peripheral blood mononuclear cells of patients with systemic lupus erythematosus / 中华风湿病学杂志

Objective:To analyze the differentially expressed genes in PBMCs of patients with systemic lupus erythematosus (SLE) by bioinformatics methods screening and analyzing the key genes related to ferroptosis, and explore the possible mechanism of ferroptosis involved in the pathogenesis of SLE at the transcription level.Methods:The data sets and samples of healthy people (HC) and SLE patients who met the screening criteria were retrieved from the Gene Expression Omnibus (GEO), a sub-database of the National Center for Biotechnology Information (NCBI). The differentially expressed genes, GO enrichment analysis and KEGG pathway enrichment analysis were analyzed by GEO2R, R language and related software packages. The protein interaction network (PPI) of differential genes was analyzed by STRING, Cytoscape and other tools to explore the key genes and pathways. In addition, real-time quantitative reverse transcription PCR (RT-qPCR) was used to verify the expression of key genes. Mann-Whitney U test was used to compare the expression of key genes in PBMCs between the two groups. Spearman rank correlation analysis was used to explore the relationship between SLE disease activity and the level of key genes. Results:Six data sets were included in this study. A total of 166 genes related to ferroptosis were differentially expressed between SLE and HC groups. The differential genes were specifically expressed in alveolar macrophages, neutrophils, CD49 + cells and CD31 + cells. GO enrichment analysis and KEGG pathway enrichment analysis showed that the differentially expressed genes were mainly involved in multiple signaling pathways closely related to SLE, such as oxidative stress response, infection and TNF signaling pathway. Hub genes screened by different algorithms all suggested RELA as a key gene, and RT-qPCR confirmed that compared with the RELA gene expression level in the HC group [0.75(0.37,1.13)], the expression level in SLE group [2.02 (1.19,4.06)] was increased, the difference was statistically significant ( Z=-3.08, P=0.002), and was positively correlated with the corresponding SLEDAI score of SLE samples ( r=0.52, P=0.019). Conclusion:The ferroptosis of many immune cells, including alveolar macrophages and CD49 + NK cells, is involved in the pathogenesis of SLE. RELA may be involved in the ferroptosis of PBMCs in SLE through the NF-κB pathway.

Analysis of clinical characteristics and the related factors to flare in patients with systemic lupus erythematosus / 中华风湿病学杂志

Objective:To summarize the clinical characteristics and factors that may affect the flare of patients with systemic lupus erythematosus (SLE).Methods:A total of 300 patients with SLE who were treated with standard treatment in the outpatient clinic of the department of rheumatology and immunology of the Second Affiliated Hospital of Air Force Military Medical University of PLA, were enrolled, and the patients were divided into 24 patients in the complete response group, 40 cases in the no response group, 192 cases in the treatment response group, and 44 cases in the low disease activity group according to the response to treatment. The differences in clinical characteristics and survival rates between the groups were compared and analyzed. Comparisons of count data were made using analysis of variance (ANOVA), comparisons of measurement data were made using the chi-square test or the Fisher′s ecact test, and survival rates were expressed as Kaplan-Meier curves. Cox regression analysis was adapted to explore risk factors for flare in these patients.Results:A total of 300 patients were followed. With a median follow-up time of 18 (1, 36) months, a total of 42 patients experienced flare. The clinical characteristics of the four groups were compared, and there were significant differences in age ( F=4.39, P=0.005), the presence of lupus nephritis ( χ2=12.66, P=0.005), hemoglobin level ( F=2.73, P=0.044), NLR level( F=3.88, P=0.010), cystatin C level( F=3.11, P=0.027), anti-RNP antibody ( χ2=12.04, P=0.007), anti-Sm antibody ( χ2=8.33, P=0.040), anti-SSB antibody ( P=0.014), anti-nucleosome antibody ( P=0.014), and anti-ribosomal P protein antibody ( χ2=11.83, P=0.008). There was no significant difference in survival between the four groups. Cox analysis showed that the combination of other autoimmune diseases [ HR(95%CI)=3.23(1.58, 6.57), P=0.001], anti-Sm antibody [ HR(95%CI)=2.15(1.04, 4.43), P=0.038], and anti-RNP antibody [ HR(95%CI)=2.54(1.13, 5.68), P=0.023] were risk factors for flare in patients with SLE who could reach the treatment target. Conclusion:Patients with SLE with different treatment responses have different clinical features, and all treatment can significantly improve the recurrence rate no matter what level of response to treatment. Patients concurrent with other autoimmune diseases, positive anti-Sm antibodies, and positive anti-RNP antibodies are at highrisk of flare.

The role of tofacitinib in early atherosclerosis in mice with systemic lupus erythematosus / 中华风湿病学杂志

Objective:To investigate the effect of tofacitinib on early atherosclerosis of patients with systemic lupus erythematosus and explore the possible relationship between lupus nephritis and early atherosclerosis of systemic lupus erythematosus.Methods:Sixteen 8-week-old female MRL/lpr mice with a body weight of 20~25 g were selected and randomly divided into the treatment group and placebo group, with 8 mice in each group. The treatment group diluted tofacitinib by normal saline, and given at a dose of 10 mg·kg -1·d -1, and the placebo group (starch tablets) administered the medication in the same way as the treatment group for a total of 8 weeks. The ELISA method was applied to detect serum anti-dsDNA antibody levels in the two groups of mice. Bradford method protein concentration was used to determine the level of urine protein in mice. Automatic biochemical analyzer was used to detect blood lipids, urea nitrogen, serum creatinine, complement C3, complement C4 levels. Western blotting was used to determine the protein expression levels of monocyte chemoattractant protein-1 (MCP-1), non-receptor protein tyrosine kinase family 1 (JAK1), signal transducer and activator of transcription 1 (STAT1) and signal transducer and activator of transcription 2 (STAT2) in aortic and kidney tissues. After the aortic arch section were prepared, oil red O was used to stain the sections, and the vascular plaque area and intimal thickness were evaluated by ImageJ software. The kidneys were dissected and stained with HE, and the active lesions of lupus nephritis were evaluated using the glomerular activity scoring system. SPSS 23.0 software was used for statistical analysis, in which the between-group comparison was performed using two independent samples t-test, and the correlation analysis was performed using the Spearman method. Results:①The serum anti-dsDNA antibody expression level in the treatment group [(5.2±1.0) U/ml] was lower than that in the placebo group [(6.9±1.2) U/ml], ( Z=-3.07, P=0.008), and the levels of complement C3 and complement C4 were higher than those in the placebo group [(293±10) mg/L vs. (260±19) mg/L, Z=2.72, P=0.017]; (16±6) mg/L vs. (8±9) mg/L, Z=3.78, P=0.006]. There was no significant difference in serum BUN and Scr between the treatment group and the placebo group [(10.6±0.7) mmol/L vs. (11.5±1.1) mmol/L, Z=-1.96, P=0.071; (17±5) μmol/L vs. (22±6) μmol/L, Z=-1.79, P=0.095]. ② Compared with the placebo group, the levels of LDL, TC and TG in the treatment group decreased [(0.83±0.15) mmol/L vs. (1.08±1.05) mmol/L, Z=-3.95, P=0.001; (2.90±0.08) mmol/L vs. (1.81±0.97) mmol/L, Z=-5.17, P=0.001; (1.10±0.08) mmol/L vs. (1.60±0.42) mmol/L, Z=-3.23, P=0.013], and HDL level increased [(2.02±0.99) mmol/L vs. (1.81±0.97) mmol/L, Z=4.42, P=0.001]. ③ Compared with the placebo group, the levels of aortic MCP-1, JAK1, STAT1 and STAT2 in the treatment group were reduced [(0.17±0.30) vs. (0.23±0.05), Z=-3.06, P=0.009; (0.83±0.09) vs. (1.05±0.19), Z=-3.07, P=0.008; (0.77±0.07) vs. (0.94±0.13), Z=-2.83, P=0.014; (0.70±0.07) vs. (0.82±0.09), Z=-2.83, P=0.013], the aortic plaque area and aortic intimal thickness were lower than those in the placebo group [(12±31) μm 2vs. (1 242±1 101) μm 2, Z=-3.12, P=0.016; (63±7) μm vs. (82.10±8.06) μm, Z=-5.13, P<0.001]. ④ Compared with the placebo group, the urine protein level and glomerulonephritis activity score in the treatment group were decreased [(0.08±0.03) mg/mL vs. (0.20±0.11) mg/mL, Z=-3.08, P=0.015; (1.79±0.38) vs. (2.79±0.14) points, Z=-7.08, P<0.001)], and renal tissue MCP-1, JAK1, STAT1.Compared with the placebo group, STAT2 levels were reduced [(0.364±0.040) vs. (0.425±0.021), Z=-3.85, P=0.003; (0.689±0.074) vs. (0.838±0.068), Z=-4.19, P=0.001; (0.508±0.070) vs. (0.646±0.019), Z=-2.85, P=0.015; (0.618±0.062) vs. (0.740±0.101), Z=-2.94, P=0.013. ⑤ The glomerular mobility scores of the two groups were positively correlated with LDL, TCHO, TG, aortic plaque area and aortic intimal thickness ( r=0.51, P=0.043; r=0.79, P<0.001; r=0.64, P=0.008; r=0.82, P<0.001; r=0.74, P=0.001), and negatively correlated with HDL ( r=-0.53, P=0.036). The urine protein levels in the two groups were positively correlated with LDL, TC, TG, aortic plaque area and aortic intimal thickness ( r=0.67, P=0.004; r=0.68, P=0.004; r=0.53, P=0.033; r=0.80, P<0.001; r=0.74, P=0.001), and negatively correlated with HDL ( r=-0.57, P=0.021). Conclusion:The severity of lupus nephritis is correlated with atherosclerosis and dyslipidemia in the early stage of systemic lupus erythematosus. Tofacitinib may reduce the degree of early arteriosclerosis and lupus nephritis in MRL/LPR mice, and reduce blood lipid levels, which may be effective in improving the prognosis of SLE and improving the survival rate of patients.