ABSTRACT
Introdução: a esclerose sistêmica (ES) é uma enfermidade do tecido conjuntivo de caráter autoimune caracterizada pela tríade de injúria vascular, autoimunidade (celular e humoral) e fibrose tecidual. Os autoanticorpos não parecem ser simplesmente epifenômenos, mas sim estarem envolvidos na patogênese da doença. Acredita-se que os autoanticorpos específicos da ES são responsáveis tanto pela amplificação da resposta imune quanto por alvejar os tipos celulares que são relevantes na fisiopatologia da ES. Objetivos: correlacionar o perfil de autoanticorpos específicos (anti-SCL70, ACA, anti-POL3) com as manifestações clínicas e laboratoriais observadas em 46 pacientes com ES da região Centro-Oeste do Brasil. Métodos: pesquisou-se a ocorrência de autoanticorpos específicos em 46 pacientes com diagnóstico de ES e correlacionou-se o tipo de autoanticorpo com as manifestações clínicas e laboratoriais encontradas. Resultados: dentre todos os pacientes avaliados, encontrou-se predomínio feminino (97,8%), idade média de 50,21 anos, cor branca (50%), forma limitada da doença (47,8%), tempo de diagnóstico entre cinco e 10 anos (50%) e tempo de evolução da doença de 9,38 anos. De acordo com o autoanticorpo específico, 24 pacientes apresentavam ACA positivo (52,2%), 15 apresentavam positividade para anti-SCL70 (32,6%) e sete apresentavam anti-POL3 positivo (15,2%). O autoanticorpo anti-SCL70 se correlacionou com a forma difusa da doença, com maior gravidade e atividade da doença, com pior qualidade de vida medida pelo índice HAQ, com maior prevalência de fenômeno de Raynaud objetivo e microcicatrizes de polpas digitais. O ACA se correlacionou com a forma limitada da doença, com o início mais precoce da enfermidade, bem como com maior prevalência de telangiectasias nos pacientes. Já o anti-POL3 se correlacionou com a forma difusa da doença, com maior ocorrência de fenômeno de Raynaud subjetivo e de atrofia muscular. Para as demais variáveis relacionadas ...
Introduction: Systemic sclerosis (SSc) is a connective tissue disease of autoimmune nature characterized by the triad of vascular injury, autoimmunity (cellular and humoral) and tissue fibrosis. Autoantibodies do not seem to be simply epiphenomena, but are involved in disease pathogenesis. It is believed that the SSc-specific autoantibodies are responsible both for amplifying immune response and targeting cell types that are relevant in the pathophysiology of SSc. Objectives: To correlate the profile of the following specific autoantibodies: anti-centromere (ACA), anti-topoisomerase I (topo I) and anti-RNA polymerase III (RNAP III) with clinical and laboratory manifestations were observed in 46 patients with SSc in the Midwest region of Brazil. Methods: The occurrence of specific autoantibodies in 46 patients with SSc was investigated, correlating the type of autoantibody with clinical and laboratory manifestations found. Results: Among all patients evaluated, we found a predominance of females (97.8%), mean age 50.21 years old, Caucasian (50%), limited cutaneous SSc (47.8%), time of diagnosis between 5 and 10 years (50%), and disease duration of 9.38 years. According to the specific autoantibody profile, 24 patients were ACA-positive (52.2%), 15 were positive for anti-topo I (32.6%), and 7 showed positive anti-RNAP III (15.2%). The anti-topo I autoantibody correlated with diffuse scleroderma, with greater disease severity and activity, with worse quality of life measured by the SHAQ index, with a higher prevalence of objective Raynaud's phenomenon and digital pitting scars of fingertips. The ACA correlated with limited scleroderma, with earlier onset of disease, as well as higher prevalence of telangiectasias. The anti-RNAP III correlated with diffuse scleroderma, with a higher occurrence of subjective Raynaud's phenomenon and muscle atrophy. There was no association between the positivity for anti-topo I, ACA and anti-RNAP III antibodies ...
Subject(s)
Humans , Male , Female , Middle Aged , Autoantibodies/blood , Scleroderma, Systemic/blood , Scleroderma, Systemic/immunology , Brazil , Cross-Sectional Studies , Prospective StudiesABSTRACT
BACKGROUND: Anti-topoisomerase I antibodies (anti-topo-I) have been known to be a specific serologic marker for systemic sclerosis (SSc). However, anti-topo-I have also been detected fre-quently in the sera of patients with diagnosis other than SSc since the enzyme linked immunosor-bent assay (ELISA) has been used widely. In order to clarify the clinical significance of anti-topo-I on ELISA, we analyzed the clinical features of the patients positive for anti-topo-I. METHODS: Anti-topo-I and other antinuclear antibodies (ANA) were investigated by conventional ELISA methods. The clinical characteristics were analyzed in 38 patients positive for anti-topo-I and 28 patients with SLE but negative for anti-topo-I. RESULTS: Of 38 patients positive for anti-topo-I, 15 were SLE and eight SSc. The mean level of anti-topo-I in the patients with SSc was higher than that in the patients with SLE (P=0.015). Of 15 anti-topo-I positive patients with SLE, 14 had one or more other ANAs in their sera whereas only one of eight anti-topo-I positive patients with SSc did (P=0.000). There was no significant difference in clinical characteristics between anti-topo-I positive and negative patients with SLE. The preva-lences of restrictive lung disease in both groups with SLE were significantly lower than that in the anti-topo-I positive patients with SSc (P=0.008). CONCLUSIONS: Anti-topo-I is not exclusively specific for SSc and present in a considerable subset of SLE. As well as the level of anti-topo-I, the coexistence of other ANAs is helpful to discriminate SLE from SSc. The Anti-topo-I detected by ELISA does not seem to be a risk factor for restrictive lung disease in the patients with SLE, unlike those with SSc.