Frecuencia de eventos cardiovasculares en pacientes con lupus eritematoso sistémico / Frequency of cardiovascular events in patients with systemic lupus erythematosus

Resumen Introducción: el progreso en los tratamientos para el lupus eritematoso sistémico (LES) resultó en una disminución de la mortalidad; sin embargo, la enfermedad cardiovascular y las complicaciones infecciosas aún son las principales causas de muerte. La evidencia apoya la participación del sistema inmunológico en la generación de la placa aterosclerótica, así como su conexión con las enfermedades autoinmunes. Objetivos: describir la frecuencia de eventos cardiovasculares (ECV) en el Registro de Lupus Eritematoso Sistémico de la Sociedad Argentina de Reumatología (RELESSAR) transversal, así como sus principales factores de riesgo asociados. Materiales y métodos: estudio descriptivo y transversal para el cual se tomaron los pacientes ingresados en el registro RELESSAR transversal. Se describieron las variables sociodemográficas y clínicas, las comorbilidades, score de actividad y daño. ECV se definió como la presencia de al menos una de las siguientes patologías: enfermedad arterial periférica, cardiopatía isquémica o accidente cerebrovascular. El evento clasificado para el análisis fue aquel posterior al diagnóstico del LES. Se conformaron dos grupos macheados por edad y sexo 1:2. Resultados: 1515 pacientes mayores de 18 años participaron del registro. Se describieron 80 pacientes con ECV (5,3%). En este análisis se incluyeron 240 pacientes conformando dos grupos. La edad media fue de 47,8 (14,4) y 47,6 (14,2) en el grupo con y sin ECV respectivamente. Los pacientes con ECV tuvieron mayor duración del LES en meses, mayor índice de Charlson, mayor SLICC (Systemic Lupus International Collaborating Clinics/American College of Rheumatology), mayor frecuencia de manifestaciones neurológicas, síndrome antifosfolípido, hospitalizaciones y uso de ciclofosfamida. Las únicas variables asociadas en el análisis multivariado fueron el índice de Charlson (p=0,004) y el SLICC (p<0,001). Conclusiones: los ECV influyen significativamente en nuestros pacientes, y se asocian a mayor posibilidad de daño irreversible y comorbilidades.

Abstract Introduction: progress in treatments for systemic lupus erythematosus (SLE) has resulted in a decrease in mortality; however, cardiovascular and infectious diseases remain the leading causes of death. Evidence supports the involvement of the immune system in the generation of atherosclerotic plaque, as well as its connection to autoimmune diseases. Objectives: to describe the frequency of cardiovascular disease (CVD) in the cross-sectional RELESSAR registry, as well as its associated variables. Materials and methods: a descriptive and cross-sectional study was performed using patients admitted to the cross-sectional RELESSAR registry. Sociodemographic variables, clinical variables, comorbidities, activity and damage scores were described. CVD was defined as at least one of the following: peripheral arterial disease, ischemic heart disease, or cerebrovascular accident. All patients with at least one CVD were included in our analysis (heart attack, central nervous system vascular disease, and peripheral arteries atherosclerotic disease). The event classified for the analysis was that after the diagnosis of SLE. SLE diagnosis was previous to CVD. Two groups matched by age and sex, 1:2 were formed. Results: a total of 1515 patients older than 18 years participated in the registry. Eighty patients with CVD (5.3%) were described in the registry. Two-hundred and forty patients were included, according to two groups. The mean age was 47.8 (SD 14.4) and 47.6 (SD 14.2) in patients with and without CVD, respectively. Patients with CVD had a longer duration of SLE in months, a higher Charlson index, a higher SLICC, increased frequency of neurological manifestations, antiphospholipid syndrome, hospitalizations, and use of cyclophosphamide. The associated variables in the multivariate were the Charlson Index (p=0.004) and the SLICC (p<0.001). Conclusions: CVDs have a significant influence on our patients, being associated with a greater possibility of damage and comorbidities.

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.

Primary Sjögren syndrome and development of another autoimmune rheumatic disease during the follow-up

Abstract Background: Primary Sjögren syndrome (pSS) is a chronic autoimmune disease with its main target being exocrine glands, and is the connective tissue disease more frequently associated with other autoimmune diseases. The aim of this study was to assess the frequency of another autoimmune rheumatic disease (ARD) developed in primary Sjögren syndrome (pSS) patients and to describe it's clinical, serological and histologic characteristics. Materials and methods: This is a retrospective cohort study. Data of patients with pSS diagnosis (American-European criteria 2002), included in the GESSAR database (Grupo de Estudio Síndrome de Sjögren, Sociedad Argentina de Reumatología) were analyzed. The development of a second ARD was registered during the follow up. Results: 681 patients were included, 94.8% female. The mean age was 54 (SD 14) years and mean age at diagnosis of 50 (SD 13) years. The mean follow-up was 4.7 (SD 4.9) years; 30 patients (4.41%, CI 95%: 3.1-5.7) developed a second ARD during the follow up, incidence rate was 9.1/1000 patients-year (IR 95%: 5.8-12.4/1000 patients-year), the most frequent being rheumatoid arthritis (RA). 96% out of these 30 patients had xerophthalmia, 86.2% xerostomia, 92% positive Schirmer test, 88.24% positive Rosa Bengala test, lisamine green or Ocular Staining Score, 81.2% positive unstimulated salivary flow, 82.1% Ro(+) and 33.33% La(+). Minor salivary gland biopsy had been performed in 14 of the 30 patients, 12 with positive results. There were no statistically significant differences respect baseline characteristics when comparing the patients who developed another ARD to the ones that did not. Conclusions: Of all the patients analyzed, 4.4% presented another ARD during their follow-up. It is important to be aware of this, to make an early and proper diagnosis and treatment of our patients. Key points Patients with primary Sjögren's Syndrome may develop another connective tissue disease during follow-up. The most frequently connective tissue disease developed during follow-up in the population of patients with primary Sjogren's Syndrome studied was rheumatoid arthritis. It is important to be aware of this to make an early and proper diagnosis.

Expresión clínica del Síndrome de Sjögren primario en pacientes adultos diagnosticados a edad menor o igual a 35 años versus mayores de 35 años / Clinical expression of primary Sjögren's syndrome in adult patients diagnosed at an age less than or equal to 35 years versus those older than 35 years

Objetivo: Describir y comparar las manifestaciones clínicas en pacientes adultos diagnosticados con Síndrome de Sjögren primario (SSp) a edad menor o igual a 35 años versus mayores a 35 años. Materiales y métodos: Se incluyeron pacientes mayores de 18 años de edad, con diagnóstico de SSp de acuerdo a los criterios de clasificación ACR - EULAR 2002/2016, registrados en la base de datos GESSAR (Grupo de Estudio Síndrome de Sjögren Sociedad Argentina de Reumatología). Resultados: Se incluyeron 665 pacientes. Cien (15,04%) con edad al diagnóstico ≤ 35 años, 92% mujeres. El promedio de edad del grupo > 35 años, fue de 54 + 11 años, 96% mujeres. Se encontraron diferencias estadísticamente significativas entre < 35 años vs > 35 años, en xeroftalmia (90,72% vs 95,64%, p: 0,04) y xerodermia (42,35% vs 57,36%, p: 0,03) y en los siguientes dominios del ESSDAI (EULAR Activity Index for primary Sjögren's syndrome): sistema nervioso periférico (4,05 vs 11,32, p: 0,03), respiratorio (6% vs 15,40%, p: 0,01) y renal (6% vs 1,59%, p: 0,02). Conclusión: Nuestro estudio sugiere un menor compromiso glandular en pacientes con SSp diagnosticados a menor edad, sin un patrón diferencial característico en cuanto al compromiso sistémico.

Objective: To describe and compare the clinical manifestations, in adult patients diagnosed with primary Sjögren's Syndrome at age less than or equal to 35 years versus those over 35 years of age. Materials and Methods: We analyzed the data of patients older than 18 years, with diagnosis of primary Sjögren's syndrome (American - European criteria 2002), included in the GESSAR database (Sjögren Syndrome Study Group of the Argentine Society of Rheumatology). Results: 665 patients were included. One hundred of them with an age at diagnosis less than or equal to 35 years and with a mean age at diagnosis of 29 + 4 years, 92% of them women. The average age at diagnosis of the group over 35 years was 54 + 11 years, 96% women. Statistically significant differences were found between less than or equal to 35 years vs over 35 years, in xerophthalmia (90.72% vs 95.64%, p: 0.04) and xeroderma (42.35% vs 57.36% , p: 0.03), and in the following domains of ESSDAI (EULAR Activity Index for primary Sjögren's syndrome): peripheral nervous system (4.05 vs 11.32, p: 0.03), respiratory (6% vs 15.40%, p: 0.01) and renal (6% vs 1.59%, p: 0.02). Conclusion: Our study suggests less glandular involvement in patients with pSS diagnosed at a younger age, without a characteristic differential pattern regarding systemic involvement.

Miocardiopatía no compactada como causa de Insuficiencia Cardíaca en Lupus Eritematoso Sistémico / Non compacted cardiomyopathy as a cause of Heart Failure in Lupus Erythematosus Systemic

La Miocardiopatía no compactada (MCNC) representa una anomalía de la morfología miocárdica frecuentemente asociada a una etiología genética, sin embargo, hay escasas descripciones de su asociación con enfermedades autoinmunes. Se presenta revisión de la literatura y el caso de una paciente lúpica que ingresa con signos de insuficiencia cardiaca descompensada con confirmación de VINC por ecocardiograma y resonancia magnética nuclear cardiaca (RMN-C).

Non compacted cardiomyopathy (NCNC) frequently represents an abnormality of myocardial morphology associated with a genetic etiology, however, there are few descriptions of its association with autoimmune diseases. We present a review of the literature and the case of a lupus patient who was admitted with signs of decompensated heart failure with confirmation of VINC by echocardiography and cardiac nuclear magnetic resonance (C-MRI).

Niveles de anticuerpos anti-anexina A5 y manifestaciones de síndrome antifosfolípido primario obstétrico / Levels of anti-anexinal antibodies A5 and manifestations of obstetric primary antiphospholipid syndrome

El Síndrome Antifosfolípidos (SAF) describe un trastorno trombofílico autoinmune caracterizado por complicaciones obstétricas. La Anexina A5 (Anx A5) es una proteína que se estudia como un nuevo autoantígeno presente en el SAF, la presencia de autoanticuerpos frente a Anx A5 podría causar trombosis placentaria y pérdida del embarazo. El objetivo de este estudio fue analizar los niveles de IgG e IgM anti-Anx A5 en mujeres con SAF primario obstétrico y su asociación con diferentes complicaciones en una población de la ciudad de Córdoba. Se trabajó con muestras de pacientes puérperas que asistieron al Hospital Córdoba y al Hospital Materno Neonatal durante los años 2013-2017 con diagnóstico de SAF obstétrico y un grupo control formado por pacientes con embarazos normales. En la mayoría de las pacientes estudiadas, los niveles de IgG e IgM anti-Anx A5 se encontraron por debajo del rango de referencia, se mostró un aumento estadísticamente significativo de los niveles de IgG en pacientes con SAF respecto al grupo control. Pero no existieron asociaciones específicas entre los niveles de anticuerpo y los tres tipos de manifestaciones clínicas presentes en los criterios de clasificación. Estos hallazgos podrían sugerir una relación entre los anticuerpos anti-Anx A5 con el SAF obstétrico.

Antiphospholipid Syndrome (APS) describes an autoimmune thrombophilic disorder characterized by obstetric complications. Annexin A5 (Anx A5) is a protein that is studied as a new autoantigen present in APS, the presence of autoantibodies against Anx A5 could cause placental thrombosis and possibly pregnancy loss. The aim of this study was to analyze levels of IgG and IgM anti-Anx A5 in women with primary obstetric APS and its association with different complications in a population of the city of Córdoba. We worked with samples of puerperal patients who attended the Córdoba Hospital and the Maternal Neonatal Hospital during the years 2013-2017 with a diagnosis of obstetric APS and a control group formed by patients with normal pregnancies. In most of the patients studied, levels of IgG and IgM anti-Anx A5 were below the reference range, is demonstrate an increase statistically significant in the levels of the IgG in patients with APS compared with control group. But there were no specific associations between antibody levels and the three types of obstetric clinical manifestations present in the classification criteria. These findings could suggest a relationship between anti-Anx A5 antibodies and obstetric APS.

Daño orgánico en Lupus Eritematoso Sistémico (LES) / Organic damage in Systemic Lupus Erythematosus

Las enfermedades autoinmunes sistémicas se caracterizan por una amplia y heterogénea variedad de manifestaciones clínicas. Las medidas propuestas para evaluar el pronóstico en ellas son la actividad de la enfermedad, el daño orgánico(DO) y la calidad de vida

Vasculopatía liveloide como presentación de síndrome antifosfolípido en el embarazo / Livedoid vasculopathy as an introduction to antiphospholipid syndrome during

Livedoid vasculopathy (LV) is an occlusive thrombotic disease that affects primarily the small blood vessels of the coger extremities and often is associated with recurrent painful ulcerations. The pathogenesis of Lvis unclear, but it is attributed to hypercoagulable state. We describe a 27-year old pregnant woman who developed a LV in the third trimester of her pregnancy. The skin biopsy revealed endothelial proliferation and fibrin thrombi in small vessels of the dermis. She had aCl Ig G and Ig M positive and lupus anticoagulant negative. Treatment with heparin, aspirin and steroids was required with dramatically improvement of the lesions