tailieunhanh - Báo cáo y học: "Anticentromere antibody positive Sjögren’s Syndrome: a retrospective descriptive analysis"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học General Psychiatry cung cấp cho các bạn kiến thức về ngành y đề tài: Anticentromere antibody positive Sjögren’s Syndrome: a retrospective descriptive analysis. | Bournia et al. Arthritis Research Therapy 2010 12 R47 http content 12 2 R47 RESEARCH ARTICLE Open Access Anticentromere antibody positive Sjogren s Syndrome a retrospective descriptive analysis Vasiliki-Kalliopi K Bournia Konstantina D Diamanti Panayiotis G Vlachoyiannopoulos Haralampos M Moutsopoulos Abstract Introduction A subgroup of patients with primary Sjogren s Syndrome SS and positive anticentromere antibodies ACA were recognized as having features intermediate between SS and systemic sclerosis SSc . Our goal was to describe this group clinically and serologically and define its tendency to evolve to full blown SSc. Methods Among 535 patients with primary SS we identified 20 ACA positive ACA SS . We compared them to 61 randomly selected ACA negative SS patients ACA- SS 31 ACA positive SSc patients with sicca manifestations SSc sicca and 20 ACA positive SSc patients without sicca manifestations SSc - sicca . Results Prevalence of ACA among SS patients was . Cases and controls did not differ in sex ratio and age at disease onset. ACA SS patients had a lower prevalence of dry eyes hypergammaglobulinaemia anti-Ro and anti-La antibodies and a higher prevalence of Raynaud s phenomenon and dysphagia compared to ACA- SS patients. They also had lower prevalence of telangiectasias puffy fingers sclerodactyly Raynaud s phenomenon digital ulcers and gastroesophageal reflux in comparison to both of the SSc subgroups and a lower prevalence of dyspnoea and lung fibrosis compared to the SSc sicca subgroup. Two patients originally having ACA SS evolved to full blown SSc. Four deaths occurred all among SSc patients. Kaplan Meier analysis showed a significant difference between cases and controls in time from disease onset to development of gastroesophageal reflux telangiectasias digital ulcers arthritis puffy fingers xerostomia hypergammaglobulinaemia and dysphagia. Conclusions ACA SS has a clinical phenotype intermediate between ACA- SS and SSc .