Anticentromere antibody positive Sjogren's Syndrome: a retrospective descriptive analysis
IntroductionA 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 amongst SS patients was 3.7%. 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 theSSc 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 and shows little tendency to evolve to SSc.
Author: Vasiliki-Kalliopi BourniaKonstantina DiamantiPanayiotis VlachoyiannopoulosHaralampos Moutsopoulos
Credits/Source: Arthritis Research &Therapy 2010, 12:R47
Published on: 2010-03-19
Copyright by the authors listed above - made available via BioMedCentral (Open Access). Please
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