ABSTRACT
Introduction: After more than 20 years of sustained work, the Latin American Group for the Study of Lupus (GLADEL) has made a significant number of contributions to the field of lupus, not only in the differential role that race/ethnicity plays in its course and outcome but also in several other studies including the beneficial effects of using antimalarials in lupus patients and the development of consensus guidelines for the treatment of lupus in our region. Methods: A new generation of "Lupus Investigators" in more than 40 centers throughout Latin America has been constituted in order to continue the legacy of the investigators of the original cohort and to launch a novel study of serum and urinary biomarkers in patients with systemic lupus erythematosus. Results: So far, we have recruited 807 patients and 631 controls from 42 Latin-American centers including 339 patients with SLE without renal involvement, 202 patients with SLE with prevalent but inactive renal disease, 176 patients with prevalent and active renal disease and 90 patients with incident lupus nephritis. Conclusions: The different methodological aspects of the GLADEL 2.0 cohort are discussed in this manuscript, including the challenges and difficulties of conducting such an ambitious project.
ABSTRACT
OBJECTIVE: Central nervous system disease occurs in over 20% of patients with systemic lupus erythematosus (SLE) resulting in major morbidity and damage. Cognitive dysfunction is common in SLE, but the cause remains uncertain and treatment options are limited. This study explores the influence of clinical, neuropsychological factors and anti-neuronal antibodies on lupus damage accrual. METHOD: A prospective cohort with 99 SLE patients recruited between 2008 and 2013 and followed up in 2016 was established. Baseline evaluations were depression (MINI-Plus), cognitive function evaluating attention, visuospatial memory and executive functions, and anti-neuronal antibodies. Activity index (SLEDAI-2K) and SLICC/ACR Damage Index (SDI) were assessed at baseline and last follow-up. RESULTS: At baseline, median (interquartile range) age was 36.0 years (27.0-45.0), disease duration 3.7 years (0.4-12.4), SLEDAI-2K 6.0 (3.0-12.0), and SDI score 1.0 (0-1.0). Major depression was present in 23%, cognitive deficit in 18%, and received immunomodulators in 36%. Anti-dsDNA/N-methyl-D-aspartate receptor antibodies were present in 19%, anti-ribosomal P in 12%, and anti-neuronal surface P antigen (NSPA) in 5%. After a median follow-up of 55 months (interquartile range 39-78), 11% had damage accrual. In a multivariate analysis, baseline SDI, SLEDAI-2K, and immunomodulators use were associated with final damage, whereas SLEDAI-2K and immunomodulator use were also associated with accrual damage. Models including anti-NSPA showed impact on final and accrual damage. Cognitive deficit, depression, and other autoantibodies were not predictors. CONCLUSIONS: Disease activity and immunomodulator use associate with lupus damage. Of the anti-neuronal antibodies examined, anti-NSPA emerged as a potential poor prognostic factor, probably related to severe SLE onset requiring elevated corticosteroid doses. Key Points ⢠Anti-NSPA may be a worse prognostic factor in SLE. ⢠Other neuropsychological factors do not influence damage.
Subject(s)
Lupus Erythematosus, Systemic/psychology , Neurons/immunology , Adult , Cognitive Dysfunction/etiology , Depression/etiology , Female , Humans , Immunologic Factors/adverse effects , Longitudinal Studies , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/immunology , Middle AgedABSTRACT
El presente caso corresponde a un paciente con diagnóstico definitivo de telangectasia hemorrágica hereditaria ó Enfermedad de Rendu-Oster-Weber por cumplir 3 de 4 criterios clínicos de Curaçao, quien tiene el antecedente de absceso cerebral y perinefrítico tratados, sin haber sido diagnosticado previamente como tal. El paciente llega a nuestro servicio presentando un cuadro de anemia severa secundaria a epistaxis recurrentes, dolor neuropático de la extremidad inferior derecha y parámetros de laboratorio compatibles con respuesta inflamatoria. Se diagnosticó finalmente un absceso pelviano, cuya consulta tardía condicionó compromiso osteomielítico iliaco ipsilateral. Además se identificó una malformación arteriovenosa pulmonar que explica la formación de sus abscesos previos y del actual.
