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Subject: search.filters.subject.systemic lupus erythematosus

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    Time to diagnosis in systemic lupus erythematosus: Associated factors and its impact on damage accrual and mortality. Data from a multi-ethnic, multinational Latin American lupus cohort
    (2024)
    Romina Nieto
    ;
    Rosana Quintana
    ;
    Ernesto Zavala-Flores
    ;
    Rosa Serrano
    ;
    Karen Roberts
    <jats:sec><jats:title>Background</jats:title><jats:p> Systemic lupus erythematosus (SLE) often mimics symptoms of other diseases, and the interval between symptom onset and diagnosis may be long in some of these patients. Aims: To describe the characteristics associated with the time to SLE diagnosis and its impact on damage accrual and mortality in patients with SLE from a Latin American inception cohort. </jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p> Patients were from a multi-ethnic, multi-national Latin-American SLE inception cohort. All participating centers had specialized lupus clinics. Socio-demographic, clinical/laboratory, disease activity, damage, and mortality between those with a longer and a shorter time to diagnosis were compared using descriptive statistical tests. Multivariable Cox regression models with damage accrual and mortality as the end points were performed, adjusting for age at SLE diagnosis, gender, ethnicity, level of education, and highest dose of prednisone for damage accrual, plus highest dose of prednisone, baseline SLEDAI, and baseline SDI for mortality. </jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p> Of the 1437 included in these analyses, the median time to diagnosis was 6.0 months (Q1–Q3 2.4–16.2); in 721 (50.2%) the time to diagnosis was longer than 6 months. Patients whose diagnosis took longer than 6 months were more frequently female, older at diagnosis, of Mestizo ethnicity, not having medical insurance, and having “non-classic” SLE symptoms. Longer time to diagnosis had no impact on either damage accrual (HR 1.09, 95% CI 0.93–1.28, p = 0.300) or mortality (HR 1.37, 95% CI 0.88–2.12, p = 0.200). </jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p> In this inception cohort, a maximum time of 24 months with a median of 6 months to SLE diagnosis had no apparent negative impact on disease outcomes (damage accrual and mortality). </jats:p></jats:sec>
    Scopus© Citations 8  1
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    Scopus© Citations 21  1
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    Immune Dysregulation Mimicking Systemic Lupus Erythematosus in a Patient With Lysinuric Protein Intolerance: Case Report and Review of the Literature
    (2021)
    Josefina Longeri Contreras
    ;
    Mabel A. Ladino
    ;
    Katherine Aránguiz
    ;
    Gonzalo P. Mendez
    ;
    Zeynep Coban-Akdemir
    <jats:p>Lysinuric protein intolerance (LPI) is an inborn error of metabolism caused by defective transport of cationic amino acids in epithelial cells of intestines, kidneys and other tissues as well as non-epithelial cells including macrophages. LPI is caused by biallelic, pathogenic variants in <jats:italic>SLC7A7</jats:italic>. The clinical phenotype of LPI includes failure to thrive and multi-system disease including hematologic, neurologic, pulmonary and renal manifestations. Individual presentations are extremely variable, often leading to misdiagnosis or delayed diagnosis. Here we describe a patient that clinically presented with immune dysregulation in the setting of early-onset systemic lupus erythematosus (SLE), including renal involvement, in whom an LPI diagnosis was suspected post-mortem based on exome sequencing analysis. A review of the literature was performed to provide an overview of the clinical spectrum and immune mechanisms involved in this disease. The precise mechanism by which ineffective amino acid transport triggers systemic inflammatory features is not yet understood. However, LPI should be considered in the differential diagnosis of early-onset SLE, particularly in the absence of response to immunosuppressive therapy.</jats:p>
      5Scopus© Citations 18
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    Lupus eritematoso sistémico buloso: Una manifestación infrecuente en población pediátrica
    (2021)
    María Trinidad Hasbún Z.
    ;
    ROLLAN MARZOLO, MARIA PAZ  
    ;
    Ximena Chaparro R.
    ;
    Cecilia Fischer S.
    ;
    Adriana Castrillón V.
    <jats:p>El lupus eritematoso sistémico buloso (LESB) es una enfermedad ampollar subepidérmica autoinmune secundaria a la presencia de autoanticuerpos contra el colágeno VII de la membrana basal. Es considerada una variante de lupus eritematoso sistémico (LES), siendo infrecuente en la población pediátrica.Objetivo: Describir caso de paciente pediátrica con erupción ampollar compatible con LESB.Caso Clínico: Paciente de sexo femenino de 16 años de ascendencia mapuche, con antecedentes de LES diagnosticado a los 10 años de edad, en tratamiento. Consultó por erupción vesiculobulosa generalizada de 6 semanas de evolución, sin sintomatología sistémica. Se realizó biopsia para estudio histológico e inmunofluorescencia directa (IFD), confirmándose el diagnóstico de LESB. La paciente respondió favorablemente al tratamiento con dapsona en dosis de 100 mg/día (asociado a su tratamiento de base), sin nuevas reactivaciones a 8 años de seguimiento.Conclusión: El LESB es una manifestación infrecuente de LES. Aunque la clínica es similar a la de otras dermatosis ampollares, la correlación entre la presencia de LES, los hallazgos histopatológicos y de IFD permiten confirmar el diagnóstico. Si bien se ha reportado mayor riesgo de LES en población de ascendencia indígena, faltan estudios respecto a la asociación de LESB en esta etnia.</jats:p>
    Scopus© Citations 2  1
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    Lupus eritematoso neonatal, caso clínico
    (2023)
    Catalina Montané
    ;
    HOJMAN CANO, LIA PAULA  
    ;
    Trinidad Hasbún Zegpi
    <jats:p>El lupus eritematoso neonatal (LEN) es una patología autoinmune muy infrecuente, que ocurre en neonatos de madres que presentan auto-anticuerpos para antígenos citoplasmáticos del Síndrome de Sjögren. En la mayoría de los casos, la evolución es benigna hacia la resolución espontánea, pero existe un grupo de pacientes que desarrollan compromiso severo del tejido de conducción miocárdico, por lo que su detección oportuna es fundamental. Objetivo: Describir un caso clínico característico de lupus eritematoso neonatal y destacar la importancia del diagnóstico oportuno en madre y neonato. Caso Clínico: Mujer de 33 años, con antecedente de hipertensión arterial, consulta en dermatología por su neonato de 15 días de vida de sexo masculino, quien presenta aparición reciente de placas redondeadas, eritematosas, de bordes solevantados, no descamativas, compatibles con LEN. Se descartó compromiso de conducción miocárdica. En los exámenes del neonato destacaba neutropenia moderada, elevación leve de transaminasas y anticuerpos antiRo y antiLa positivos. En interrogación dirigida, la madre refiere historia personal de síntomas compatibles con enfermedades del tejido conectivo, tales como fatiga, alopecia y xeroftalmia. Se solicitan anticuerpos antinucleares a la madre, quien presenta título de 1/1280 con patrón moteado, anticuerpos anti Ro y La y anticuerpos anti-DNA doble hebra positivos, y Test de Schirmer compatible con ojo seco, por lo que se diagnostica Lupus Eritematoso Sistémico con Síndrome de Sjögren asociado. Se realiza seguimiento al lactante por 5 meses con remisión de los signos cutáneos y normalización de los exámenes de laboratorio. Conclusiones: Si bien, las manifestaciones cutáneas de LEN son transitorias y benignas en el neonato, estas pueden ir acompañadas de complicaciones de riesgo vital que requieren una búsqueda activa y un manejo oportuno por el equipo médico. Un 25% de las madres de hijos con LEN son asintomáticas, o desconocen su diagnóstico de LES previo al parto, por lo que el diagnóstico oportuno de LEN en un neonato permite diagnosticar a las madres asintomáticas, optimizando su seguimiento y manejo.</jats:p>
    Scopus© Citations 1  4
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    Effectiveness of an inactivated SARS-CoV-2 vaccine in children and adolescents: a large-scale observational study
    (2023)
    Alejandro Jara
    ;
    Eduardo A. Undurraga
    ;
    Juan Carlos Flores
    ;
    José R. Zubizarreta
    ;
    Cecilia González
      7Scopus© Citations 15
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    First Latin American clinical practice guidelines for the treatment of systemic lupus erythematosus: Latin American Group for the Study of Lupus (GLADEL, <i>Grupo Latino Americano de Estudio del Lupus</i>)–Pan-American League of Associations of Rheumatology (PANLAR)
    (2018)
    Bernardo A Pons-Estel
    ;
    Eloisa Bonfa
    ;
    Enrique R Soriano
    ;
    Mario H Cardiel
    ;
    Ariel Izcovich
    <jats:p>Systemic lupus erythematosus (SLE), a complex and heterogeneous autoimmune disease, represents a significant challenge for both diagnosis and treatment. Patients with SLE in Latin America face special problems that should be considered when therapeutic guidelines are developed. The objective of the study is to develop clinical practice guidelines for Latin American patients with lupus. Two independent teams (rheumatologists with experience in lupus management and methodologists) had an initial meeting in Panama City, Panama, in April 2016. They selected a list of questions for the clinical problems most commonly seen in Latin American patients with SLE. These were addressed with the best available evidence and summarised in a standardised format following the Grading of Recommendations Assessment, Development and Evaluation approach. All preliminary findings were discussed in a second face-to-face meeting in Washington, DC, in November 2016. As a result, nine organ/system sections are presented with the main findings; an ‘overarching’ treatment approach was added. Special emphasis was made on regional implementation issues. Best pharmacologic options were examined for musculoskeletal, mucocutaneous, kidney, cardiac, pulmonary, neuropsychiatric, haematological manifestations and the antiphospholipid syndrome. The roles of main therapeutic options (ie, glucocorticoids, antimalarials, immunosuppressant agents, therapeutic plasma exchange, belimumab, rituximab, abatacept, low-dose aspirin and anticoagulants) were summarised in each section. In all cases, benefits and harms, certainty of the evidence, values and preferences, feasibility, acceptability and equity issues were considered to produce a recommendation with special focus on ethnic and socioeconomic aspects. Guidelines for Latin American patients with lupus have been developed and could be used in similar settings.</jats:p>
    Scopus© Citations 88  7
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    Autoantibodies Against Proteins Previously Associated With Autoimmunity in Adult and Pediatric Patients With COVID-19 and Children With MIS-C
    (2022)
    Peter D. Burbelo
    ;
    Riccardo Castagnoli
    ;
    Chisato Shimizu
    ;
    Ottavia M. Delmonte
    ;
    Kerry Dobbs
    <jats:p>The antibody profile against autoantigens previously associated with autoimmune diseases and other human proteins in patients with COVID-19 or multisystem inflammatory syndrome in children (MIS-C) remains poorly defined. Here we show that 30% of adults with COVID-19 had autoantibodies against the lung antigen KCNRG, and 34% had antibodies to the SLE-associated Smith-D3 protein. Children with COVID-19 rarely had autoantibodies; one of 59 children had GAD65 autoantibodies associated with acute onset of insulin-dependent diabetes. While autoantibodies associated with SLE/Sjögren’s syndrome (Ro52, Ro60, and La) and/or autoimmune gastritis (gastric ATPase) were detected in 74% (40/54) of MIS-C patients, further analysis of these patients and of children with Kawasaki disease (KD), showed that the administration of intravenous immunoglobulin (IVIG) was largely responsible for detection of these autoantibodies in both groups of patients. Monitoring <jats:italic>in vivo</jats:italic> decay of the autoantibodies in MIS-C children showed that the IVIG-derived Ro52, Ro60, and La autoantibodies declined to undetectable levels by 45-60 days, but gastric ATPase autoantibodies declined more slowly requiring &amp;gt;100 days until undetectable. Further testing of IgG and/or IgA antibodies against a subset of potential targets identified by published autoantigen array studies of MIS-C failed to detect autoantibodies against most (16/18) of these proteins in patients with MIS-C who had not received IVIG. However, Troponin C2 and KLHL12 autoantibodies were detected in 2 of 20 and 1 of 20 patients with MIS-C, respectively. Overall, these results suggest that IVIG therapy may be a confounding factor in autoantibody measurements in MIS-C and that antibodies against antigens associated with autoimmune diseases or other human proteins are uncommon in MIS-C.</jats:p>
    Scopus© Citations 25  1
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    Factors associated with neuropsychiatric involvement in Latin American patients with systemic lupus erythematosus
    (2021)
    Leonor A Barile-Fabris
    ;
    Hilda Fragoso-Loyo
    ;
    Daniel Wojdyla
    ;
    Rosana Quintana
    ;
    Guillermo J Pons-Estel
    <jats:sec><jats:title>Introduction</jats:title><jats:p> Factors related to presentation of neuropsychiatric (NP) SLE manifestations, early in the course of the disease, and during follow up have not been clearly established. </jats:p></jats:sec><jats:sec><jats:title>Purpose</jats:title><jats:p> To identify disease and non-disease related factors associated with NP manifestations in early SLE. </jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p> We included 1193 patients from the GLADEL inception cohort free of NP involvement at cohort entry. We evaluated the association of demographic, clinical and laboratory data with NP involvement during follow-up. </jats:p></jats:sec><jats:sec><jats:title>Statistical methods</jats:title><jats:p> Independent factors associated with NP involvement were identified using a multivariable Cox regression model. </jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p> Factors independently associated with NP manifestations were: mestizo ethnicity (HR 1.701, 95% CI 1.282–2.258, p = 0.0002), myalgias/myositis (HR 1.832, 95% CI 1.335–2.515, p = 0.0002), pneumonitis (HR 2.476, 95% CI 1.085–5.648, p = 0.0312), shrinking lung (HR 2.428, 95% CI 1.074–5.493, p = 0.0331) and hemolytic anemia (HR 1.629, 95% CI 1.130–2.347, p = 0.0089). Longer disease duration at cohort entry (13 to 24 months) was associated with a lower risk of developing NP manifestations (HR 0.642, 95% CI 0.441–0.934, p = 0.0206). </jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p> Patients with myalgias/myositis, pneumonitis, shrinking lung and hemolytic anemia are at higher risk of NP involvement, whereas longer disease duration at cohort entry is associated with a lower risk of developing NP involvement. </jats:p></jats:sec>
      15Scopus© Citations 3
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      14Scopus© Citations 2