RESUMO
Objective The objective of this study was to examine neuropsychiatric lupus in a Black Caribbean population. Methods We reviewed Barbados National Lupus Registry patients with ≥4 American College of Rheumatology criteria and a diagnosis of neuropsychiatric lupus using the American College of Rheumatology 19 case definitions. Results From 366 patients with four or more American College of Rheumatology criteria for systemic lupus erythematosus, 55 (15%) had evidence of neuropsychiatric lupus. There were 51 females and four males (F:M = 13:1) with a median age of 31 years. A total of 76.4% had a single neuropsychiatric lupus complication and 23.6% had two or three complications occurring sequentially or concurrently. The top three complications were psychosis - 49.1% (95% CI 35.8, 62.5); ischaemic stroke - 32.7% (21.4, 46.5); and generalized tonic-clonic seizures - 12.7% (6.0, 24.8). Twelve of the American College of Rheumatology 19 neuropsychiatric syndromes were represented: 91.2% central; 8.8% peripheral. There were 521 observation years, and for 32 patients (58%) neuropsychiatric lupus was a presenting feature. For the remaining 23 (42%) the first neuropsychiatric lupus event came after systemic lupus erythematosus diagnosis - median time of two years. Of the 22 deaths, systemic lupus erythematosus nephritis caused almost half (45.5%) at a median age of 32. The prevalence of nephritis was lower in the neuropsychiatric lupus subgroup (25.5%) compared with the Barbados National Lupus Registry data (47%) ( P = 0.01). Ischaemic stroke caused 22.7% of deaths at a median age of 46 and was the main cause of chronic neurologic deficits amongst survivors. Conclusion Neuropsychiatric lupus was an early cause of morbidity in systemic lupus erythematosus with predominantly singular central nervous system complications, the most common of which was psychosis. Most deaths occurred at a young age, principally from systemic lupus erythematosus nephritis. Ischaemic stroke was the main neurologic cause of death and disability.
Assuntos
População Negra , Lúpus Eritematoso Sistêmico/epidemiologia , Nefrite Lúpica/epidemiologia , Vasculite Associada ao Lúpus do Sistema Nervoso Central/epidemiologia , Adulto , Barbados/epidemiologia , Isquemia Encefálica/epidemiologia , Isquemia Encefálica/etnologia , Isquemia Encefálica/etiologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/etnologia , Nefrite Lúpica/etnologia , Vasculite Associada ao Lúpus do Sistema Nervoso Central/complicações , Vasculite Associada ao Lúpus do Sistema Nervoso Central/etnologia , Masculino , Pessoa de Meia-Idade , Transtornos Psicóticos/epidemiologia , Transtornos Psicóticos/etnologia , Transtornos Psicóticos/etiologia , Sistema de Registros , Acidente Vascular Cerebral/epidemiologia , Acidente Vascular Cerebral/etnologia , Acidente Vascular Cerebral/etiologia , Fatores de TempoRESUMO
The identity of the protein antigens targeted by anti-cytoplasmic antibodies in lupus was discovered 30 years ago. These antigens are three acidic ribosomal phosphoproteins, P0, P1, and P2. Precise identification of the shared epitope on these three proteins enabled sensitive and specific immunoassays to be developed. Anti-P antibodies are highly specific for systemic lupus erythematosus (SLE) and occur in 15%-35% of patients, depending on ethnicity as well as the age of onset. Increased frequencies of detection of anti-P have been reported in childhood SLE as well as in neuropsychiatric, renal, and hepatic disease. While longitudinal studies by the Systemic Lupus International Collaborating Clinics (SLICC) consortium supported the association of anti-P with neuropsychiatric lupus, the predictive value of antibody determination remains controversial. This is likely explained by the heterogeneity of neuropsychiatric lupus as well as by the different methodologies used for assay. A number of experimental studies have suggested a direct pathogenic role for anti-P antibodies in brain disease. Findings include cross reactivity between anti-P and a neuronal surface antigen, which was detected in areas of the brain involved in memory, cognition, and emotion. Direct injection of anti-P antibodies into the brains of rodents was also associated with abnormal electrical activity and behavioral disturbances. Taken together, research over the last 30 years has established anti-P antibodies as a useful diagnostic marker of SLE and at least a subset of patients with neuropsychiatric disease. Further research is required to fine tune the association of anti-P with clinical manifestations and establish beyond high probability a pathophysiologic role for the antibodies.