Neuropsychiatric Lupus in clinical practice / Lúpus neuropsiquiátrico na prática clínica

ABSTRACT Systemic lupus erythematosus (SLE) is a chronic autoimmune disease involving multiple organs, characterized by the production of autoantibodies and the development of tissue injury. The etiology of SLE is partially known, involving multiple genetic and environmental factors. As many as 50% of patients with SLE have neurological involvement during the course of their disease. Neurological manifestations are associated with impaired quality of life, and high morbidity and mortality rates. Nineteen neuropsychiatric syndromes have been identified associated with SLE, and can be divided into central and peripheral manifestations. This article reviews major neuropsychiatric manifestations in patients with SLE and discusses their clinical features, radiological findings and treatment options.

RESUMO Lúpus eritematoso sistêmico (LES) é uma doença autoimune crônica que envolve múltiplos órgãos e sistemas, caracterizada pela produção de auto anticorpos e lesão tecidual. A etiologia do LES é parcialmente conhecida e envolve interação entre fatores genéticos e ambientais. Até 50% dos pacientes com LES apresentam envolvimento neurológico no decorrer da doença. Manifestações neurológicas estão associadas a prejuízo na qualidade de vida e altas taxas de mortalidade e morbidade. Foram identificadas 19 síndromes neuropsiquiátricas em pacientes com LES, divididas entre manifestações do sistema nervoso central e periférico. O objetivo deste artigo é revisar as manifestações neuropsiquiátricas mais importantes. Serão abordadas as características clínicas, os aspectos radiológicos e opções de tratamento dos eventos neuropsiquiátricos.

Childhood lupus: Experience from eastern India.

Objective. To delineate the clinical behavior of SLE in children from Eastern India and to the differences in disease pattern. Methods. In the present study, all 44 patients of pediatric SLE who were diagnosed over a period of 5 years in our pediatric rheumatology clinic were followed prospectively. The resultant database was analyzed using standard statistical methods. Results. About 3.9% of all rheumatology cases dealt with in the clinic in the last five yrs (n=1063) were SLE. The number of children in 5-8 yrs and 8-12 yrs age groups were 13 and 27, respectively. The overall female (n=35) to male (n=9) ratio in this study was 3.8:1. Renal, hematological and Neuropsychiatric features were most common major organ manifestations(54%,54% and 25% respectively). Joints and skin were the most common minor organs involved. Two case were ANA negative SLE. Among the typical features of ANA negative disease, only nephritis was found in these patients. Anti dsDNA was positive in 50 % cases (n=21). C3 levels were studied in all cases with nephritis (n=22) and 68 % (n=15) had hypocomplementemia. Anti Ro and anti La antibodies were positive in two cases of neonatal lupus. APLA, Anti Sm antibody and anti U1RNP were negative in the cases where testing was done. Conclusions. This study has tried to delineate the disease trends of childhood lupus from Eastern India. Certain important trends have emerged which are different from other contemporary Indian and International observations.