Sjögren's syndrome and sicca symptoms in patients with systemic sclerosis / Síndrome de Sjögren y síntomas de sicca en pacientes con esclerosis sistémica

Introduction: Several autoimmune diseases can be accompanied by dysfunction of the salivary glands, regardless of the presence or absence of association with Sjögren's syndrome (SS). A recent study by Maeshima and colleagues found salivary hyposecretion in 58.3% of patients with various connective tissue diseases, particularly systemic sclerosis (SSc). Objective: To determine the prevalence of SS and Sicca symptoms in patients with SSc. Assess whether the presence of SS in patients with SSc causes worsening of the disease. Methods: 69 SSc patients periodically monitored in the rheumatology clinic at NHU/UFMS composed the study. All patients were questioned about sicca symptoms and clinical features. We evaluated the RF levels, ANA, anti-Ro/La. Results and discussion: 69 SSc patients were enrolled in the study, with average age of 51.2 years, 98.3% females and 50% caucasian. Sicca symptoms were present in 48 patients (69.5%) with SSc; 43/69 patients (62.3%) with dry mouth and 46/69 patients (66.7%) with dry eye. Sicca symptoms were observed in patients with limited and diffuse form of the disease. The antinuclear antibody positivity was 95% and the rheumatoid factor (RF) was observed in 14 patients (23.3%). Anti-Ro (SSA) antibodies were detected in 11 patients (15.9%) and anti-La (SSB) in 6 patients (8.7%) in this study. Only 16 patients (23.2%) had true SS, according to the American-European Consensus Group on Classification Criteria for Sjögren's syndrome. The findings in the study corroborate data found in literature. Conclusion: This study confirms that Sicca symptoms are found in a large number of patients with SSc. Sjögren prevalence was observed in 23.2% of the SSc patients, including patients with limited and diffuse cutaneous subtype of disease. Key words: Sicca syndrome, Sjögren's syndrome, overlap, autoantibodies, systemic sclerosis

Introducción: Varias enfermedades autoinmunes pueden ir acompañadas de disfunción de las glándulas salivales, independientemente de la presencia o ausencia de asociación con el síndrome de Sjögren (SS). Un estudio reciente de Maeshima y sus colegas hallaron hiposecreción salival en el 58,3% de los pacientes con diversas enfermedades del tejido conectivo, particularmente la esclerosis sistémica (SSc). Objetivo: Determinar la prevalencia de los síntomas de SS y sicca en pacientes con SSc. Evaluar si la presencia de SS en pacientes con SSc provoca empeoramiento de la enfermedad. Métodos: 69 pacientes SSc periódicamente monitorizados en la clínica de reumatología en NHU/UFMS formaron parte del estudio. Todos los pacientes fueron interrogados acerca de síntomas sicca y características clínicas. Se evaluaron los niveles de FR, FAN, anti-Ro/La. Resultados y discusión: Se incluyeron 69 pacientes SSc en el estudio, con edad promedio de 51,2 años, 98,3% mujeres y 50% caucásicos. Los síntomas de Sicca estuvieron presentes en 48 pacientes (69,5%) con SSc; 43/69 pacientes (62,3%) con boca seca y 46/69 pacientes (66,7%) con ojo seco. Síntomas sicca se observaron en pacientes con forma limitada y difusa de la enfermedad. La positividad del anticuerpo antinuclear fue del 95% y el factor reumatoideo se observó en 14 pacientes (23,3%). Anticuerpos Anti-Ro (SSA) se detectaron en 11 pacientes (15,9%) y anti-La (SSB) en 6 pacientes (8,7%) en este estudio. Sólo 16 pacientes (23,2%) tenían verdaderas SS, según el Grupo de Consenso Americano-Europeo sobre los criterios de clasificación para el síndrome de Sjögren. Los hallazgos del estudio corroboran los datos encontrados en la literatura. Conclusión: Este estudio confirma que los síntomas sicca se encuentran en un gran número de pacientes con SSc. Se observó una prevalencia de Sjögren en el 23,2% de los pacientes con esclerodermia, incluyendo pacientes con subtipo cutáneo limitado y difuso

Autoanticorpos em esclerose sistêmica e sua correlação com as manifestações clínicas da doença em pacientes do Centro-Oeste do Brasil / Autoantibodies in systemic sclerosis and their clinical correlation in patients from a Midwestern region of Brazil

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 ...

[Autoantibodies in systemic sclerosis and their clinical correlation in patients from a Midwestern region of Brazil]. / Autoanticorpos em esclerose sistêmica e sua correlação com as manifestações clínicas da doença em pacientes do Centro-Oeste do Brasil.

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 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 5-10 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 and other variables related to laboratory abnormalities, as well as Rodnan skin score and skin, vascular, musculoskeletal, gastrointestinal, cardiopulmonary and renal manifestations. CONCLUSIONS: The clinical subtype of the disease and some clinical manifestations in SSc may correlate positively with the presence of specific autoantibodies.