Efecto de los antimaláricos sobre los diferentes dominios del índice de daño SLICC en pacientes de la cohorte GLADEL / Effect of antimalarials on the different domains of the SLICC damage index in patients of the GLADEL cohort

Objetivos: Estimar el efecto de los antimaláricos (AM) sobre los diferentes dominios del índice de daño SLICC (SDI). Métodos: Se estudiaron pacientes con diagnóstico clínico reciente (≤2 años) de lupus eritematoso sistémico (LES) de la cohorte GLADEL. Variable de estudio: aumento en los dominios del SDI desde el ingreso a la cohorte. Variables independientes: características sociodemográficas, clínicas, laboratorio y tratamientos. El efecto de los AM, como variable dependiente del tiempo, sobre los dominios más frecuentes del SDI (ajustado por factores de confusión) fue examinado con un modelo de regresión de Cox multivariado. Resultados: De 1466 pacientes estudiados, 1049 (72%) recibieron AM con un tiempo medio de exposición de 30 meses (Q1-Q3: 11-57) y 665 pacientes (45%) presentaron daño durante un seguimiento medio de 24 meses (Q1-Q3: 8-55); 301 eventos fueron cutáneos, 208 renales, 149 neuropsiquiátricos, 98 musculoesqueléticos, 88 cardiovasculares y 230 otros. Después de ajustar por factores de confusión, el uso de AM se asoció a un menor riesgo de daño renal (HR 0,652; IC 95%: 0,472-0,901) y en el límite de la significancia estadística (HR 0,701, IC 95%: 0,481-1,024) para el dominio neuropsiquiátrico. Conclusión: En GLADEL, el uso de AM se asoció independientemente a un menor riesgo de daño acumulado renal.

Objective: To assess the effects of antimalarials (AM) over the items of the SLICC Damage Index (SDI). Methods: Patients with recent (≤2 years) diagnosis of systemic lupus erythematosus (SLE) from the GLADEL cohort were studied. End-point: increase in items SDI since cohort entry. Independent variables (socio-demographic, clinical, laboratory and treatment) were included. The effect of AM as a time dependent variable on most frequent SDI items (adjusting for potential confounders) was examined with a multivariable Cox regression model. Results: Of the 1466 patients included in this analysis, 1049 (72%) received AM with a median exposure time of 30 months (Q1-Q3: 11-57). Damage occurred in 665 (45%) patients during a median follow-up time of 24 months (Q1-Q3: 8-55). There were 301 integument, 208 renal, 149 neuropsychiatric, 98 musculoskeletal, 88 cardiovascular and 230 others less frequently represented damages. After adjusting for potential confounders at any time during follow-up, a lower risk of renal damage (HR 0.652; 95% CI: 0.472-0.901) and borderline for neuropsychiatric damage (HR 0.701, 95% CI: 0.481-1.024) was found. Conclusion: In the GLADEL cohort, after adjustment for possible confounding factors, AM were independently associated with a reduced risk of renal damage accrual.

Compromiso renal en vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos. Recomendaciones de consenso de las Sociedades Chilenas de Nefrología y Reumatología / Renal involvement in antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides. Recommendations of the Chilean Societies of Nephrology and Rheumatology

Renal involvement is a frequent complication in antineutrophil cytoplasmic antibodies (ANCA)associated vasculitides, adding morbidity and mortality, such as chronic kidney disease and the need for renal replacement therapy. With the aim of reaching a consensus on relevant issues regarding the diagnosis, treatment and follow-up of patients with these diseases, the Chilean Societies of Nephrology and Rheumatology formed a working group that, based on a critical review of the available literature and their experience, raised and answered consensually a set of questions relevant to the subject. This document includes aspects related to the clinical diagnosis, the histological characteristics, the therapeutic alternatives to induce and maintain the remission of the disease, relapse surveillance strategies and complementary therapies.

Recomendaciones sobre el manejo del compromiso renal del lupus eritematoso sistémico: Documento de Consenso de las Sociedades Chilenas de Nefrología y Reumatología / A consensus of the Chilean Nephrology and Rheumatology Societies on renal involvement in systemic lupus erythematosus

Renal involvement affects over one half of patients with Systemic Lupus Erythematosus increasing their mortality and morbidity, including chronic renal disease and the need of renal replacement therapies. Aiming to achieve a consensus in the most relevant topics on diagnosis, therapy and follow-up of patients with lupus renal disease, the Chilean Societies of Nephrology and Rheumatology constituted a workgroup that, based on a critical review of the available literature and their experience, raised and answered by consensus a set of relevant questions. This document includes aspects related to the clinical diagnosis, the importance of a suitable histological classification, therapeutic alternatives to induce and maintain disease remission, strategies for follow-up, additional therapies and ginecological-obstetric issues.

Tratamientos biológicos para nefropatía lúpica / Biological treatment and lupus nephritis

Renal involvement in patients with Systemic Lupus Erythematosus is frequent causing considerable morbidity and mortality. Conventional therapy consists of immunosuppressive medications administered in two stages, induction followed by a maintenance phase. Different drugs result in similar outcomes although with adverse events and relapses. Here we present a brief review of the published literature regarding new approaches in therapy with emphasis on biological treatment and lupus nephritis...

La afectación renal en pacientes con Lupus Eritematoso Sistémico es frecuente, causando un aumento tanto de la morbilidad como la mortalidad. El tratamiento convencional incluye inmunosupresores en una fase de inducción y otra de mantención, con una eficacia similar entre ellos, sin embargo pueden presentar efectos adversos graves y recaídas de la enfermedad. A continuación se presenta una breve revisión de la literatura publicada respecto a los nuevos tratamientos biológicos y nefritis lúpica...

Galectin-8 binds to LFA-1, blocks its interaction with ICAM-1 and is counteracted by anti-Gal-8 autoantibodies isolated from lupus patients

Galectin-8 belongs to a family of mammalian lectins that recognize glycoconjugates present on different cell surface components and modulate a variety of cellular processes. A role of Gal-8 in the immune system has been proposed based on its effects in immune cells, including T and B lymphocytes, as well as the presence of anti-Gal-8 autoantibodies in the prototypic autoimmune disease systemic lupus erythematosus (SLE). We have previously described that Gal-8 induces apoptosis in activated T cells interacting with certain β1 integrins and this effect is counteracted by the anti-Gal-8 autoantibodies. Given that Gal-8 can potentially interact with several glycoproteins, here we analyzed the β2 integrin Lymphocyte Function-Associated Antigen-1 (LFA-1), which is involved in leukocyte cell adhesion and immunological synapses. We show by GST-pull down assays that Gal-8 interacts with LFA-1 and this interaction is inhibited by anti-Gal-8 autoantibodies isolated from SLE patients. In cell adhesion assays, Gal-8 precluded the interaction of LFA-1 with its ligand Intracellular Adhesion Molecule-1 (ICAM-1). These results suggest that Gal-8 can exert immunosuppressive action not only by inducing apoptosis in activated T cells but also by negatively modulating the crucial function of LFA-1 in the immune system, while function-blocking autoantibodies counteract these effects.

Actualización en lupus neuro-psiquiátrico con énfasis en déficit cognitivo / An update on neuropsychiatric lupus with emphasis in cognitive dysfunction

Background: physically and psychologically. We review herein the nomenclature and case definitions for neuropsychiatric lupus syndromes proposed by the American College of Rheumatology in 1999. We emphasize cognitive dysfunction and discuss etiological hypotheses, especially those related to the presence of antineuronal autoantibodies.

Guía clínica: lupus eritematoso sistémico (LES) / Systemic lupus erythematosus: a clinical guide

El Lupus Eritematoso Sistémico (LES) es una enfermedad inflamatoria, sistémica, crónica, de patogenia autoinmune. Sus manifestaciones varían desde afecciones leves a graves o fatales. En más común en mujeres y su prevalencia varía entre 40 a 200 casos/100.000 habitantes. El diagnóstico y reconocimiento precoz de sus manifestaciones sistémicas son críticos para una adecuada derivación, tratamiento y pronóstico de los pacientes. A petición del MINSAL, la Sociedad Chilena de Reumatología designó un grupo de trabajo para la elaboración de una guía clínica de LES. Objetivos: Definir niveles de atención, criterios de derivación según gravedad y elaborar recomendaciones para el diagnóstico, tratamiento y seguimiento de los principales compromisos del LES siguiendo la metodología de realización de guías clínicas. Metodología: Se siguieron las indicaciones para realización de guías clínicas basadas en criterios de evaluación (AGREE) y una combinación de criterios de medicina basada en la evidencia y consenso de expertos. La pesquisa bibliográfica se centró en la búsqueda de respuesta para 13 preguntas seleccionadas, respecto a: niveles de atención y criterios de derivación; abordaje general; principales compromisos graves del LES y situaciones especiales. Para cada pregunta se hizo una recomendación. La evidencia se estableció usando una escala tradicional. Además, se midió el grado de acuerdo (GdA) con las recomendaciones efectuadas, mediante una escala de 0 a 10 puntos, por los reumatólogos integrantes del grupo de trabajo y por cinco pares independientes. Resultados: Se desarrollaron 13 recomendaciones respecto a: 1) Rol del médico no especialista y criterios de derivación. 2) Rol del reumatólogo. 3) Sospecha y diagnóstico precoz del LES. 4) Pronóstico y gravedad. 5) Evaluación de actividad y daño en el LES. 6) Patología asociada al LES. 7) Fármacos utilizados en el LES y su toxicidad. 8) Bases diagnósticas de nefropatía lúpica. 9) Tratamiento de nefropatía lúpica...

Systemic lupus erythematosus (SLE) is an inflammatory, systemic and chronic disease of autoimmune pathogenesis. Manifestations vary from mild to serious or fatal conditions. It is most common among women and its prevalence varies between 40 to 200 cases/100.000 inhabitants. Early diagnosis as well as identification of systemic manifestations are critical for adequate referral, treatment and prognosis. At the request of Chile's health ministry, the Chilean Society of Rheumatology designated a work group to elaborate clinical guidelines for SLE. Objectives: Define levels of attention, criteria for referral according to seriousness, and elaborate recommendations for diagnosis, treatment and follow-up of the main disorders of SLE following the clinical guideline execution methodology. Methodology: Indications for the creation of clinical guidelines based on the AGREE evaluation criteria and a combination of medical criteria based on expert evidence and consensus were followed. Bibliographical investigation was centered on responding 13 selected questions with respect to: level of attention and referral criteria; general approach; main critical SLE compromises, and special situations. A recommendation was given for each question. Evidence was established using a traditional scale. Moreover, the degree of agreement was measured (GdA) with the recommendations carried out, by means of a scale from 0 to 10 by the rheumatologists who made up the work group and by five independent peers. Results: 13 recommendations were developed with respect to: 1) Role played by non-specialized physicians and referral criteria; 2) Role played by rheumatologist; 3) Suspicion and early diagnosis of SLE; 4) Prognosis and seriousness; 5) evaluation of SLE activity and damage; 6) Pathology associated to SLE; 7) Drugs used for SLE and their toxicity; 8) Diagnostic basis for lupus nephritis; 9) Treatment for lupus nephritis; 10) Neuropsychiatric manifestations of SLE; 11) SLE and...

Trombosis de senos venosos encefálicos como posible primera manifestación de enfermedad de Behçet: Caso clínico / Extensive cerebral vein thrombosis as first manifestation of Behçet's disease: Report of one case

Behçet’s disease (BD) is a systemic inflammatory vascular disease with several clinical manifestations and geographical differences in disease expression. In Middle Eastern countries it is one of the most common causes of cerebral venous thrombosis. We report a 29-year-old female admitted for acute headache and vomiting. A magnetic resonance image showed a large thrombosis of sagital and transverse sinuses. She developed oral and genital ulcers a week later. Ophthalmologic examination revealed left anterior uveitis and ipsilateral papilledema. Multiple studies ruled out a hypercoagulability syndrome. The patient used oral contraceptives. Anticoagulant therapy was prescribed. A biopsy of a genital ulcer demonstrated diffuse lymphocytic infiltration with vasculitis. After treatment with topical and systemic corticoids, her condition improved. Venous sinus thrombosis followed by oral and genital ulcers is an unusual presentation of Behçet’s disease.

Artritis reumatoide temprana: [revisión] / Early rheumatold arthritis: [review]

This article reviews some key issues of early rheumatoid arthritis such as the difficulties to recognize this condition during the first months after onset. Therefore, three reference diagnostic criteria have been proposed for any patient presenting with more than three simultaneously inflammed joints, involvement of  metacarpophalangeal or proximal interphalangeal joints and morning stiffness lasting more than 30 minutes. Antibodies to cyclic citrullinated peptides are new markers that can be used for diagnosis. The immediate treatment during the "opportunity window" at the onset of inflammation may avoid the erosive joint damage. The use of synthetic or biological disease modifying medications, specially tumor necrosis factor alpha antagonists, also contribute to this purpose. Primary care physicians should be aware of the early signs of the disease to provide an adequate treatment and referral to specialists.

Eficacia del infliximab en pacientes con síndrome de Behçet portadores de úveo-retinitis grave / Effectiveness of infliximab in patients with Behçet syndrome and severe uveoretinitis. Report of five cases

The efficacy and safety of repeated administration of infliximab was evaluated in five patients (two men, three women) with Behçet syndrome accompanied by severe uveoretinitis. Ocular and extra ocular inflammation was suppressed in all patients during the observation period without any serious adverse reactions. The results in these patients suggests that TNF-α blockade is effective in patients with severe ocular Behçet syndrome.

Autoanticuerpos anti-galectina-8 en pacientes con lupus eritematoso sistémico / Antibodies against galectin-8 in patients with systemic lupus erythematosus

Background: The family of lectins known as galectins (galectins 1-14) are involved in the regulation of the immune system and in oncogenesis. During a search for antigens recognized by antibodies produced by a patient with systemic lupus erythematosus (SLE) we found reactivity against galectin-8, for which autoantibodies have not been previously described. Aim: To determine the frequency of autoantibodies against galectin-8 in lupus patients compared with healthy controls. Patients and Methods: Galectin-8 was purified from a bacterial expression system and used in immunoblot assays as antigen to screen the sera of 55 SLE patients and matched controls. Disease activity was evaluated using the Mexican Modification of the Systemic Lupus Erythematosus Disease Activity Index (MEX-SLEDAI). Results: Reactivity against galectin-8 was detected in 30% of SLE patients, compared to 7% of controls (p=0.003). We could not detect any particular SLE manifestation associated to the presence of these autoantibodies. Conclusions: This is the first description of autoantibodies against galectin-8. Its higher frequency in patients with SLE suggests a pathogenic role. Further studies are needed to determine their clinical relevance.