Abstract
IntroductionA subgroup of patients with primary Sjögren'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.MethodsAmong 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].ResultsPrevalence of ACA among 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 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.ConclusionsACA+/SS has a clinical phenotype intermediate between ACA-/SS and SSc and shows little tendency to evolve to SSc.
Highlights
A subgroup of patients with primary Sjögren’s Syndrome (SS) and positive anticentromere antibodies (ACA) were recognized as having features intermediate between SS and systemic sclerosis (SSc)
In an earlier, retrospective, study of 41 ACA positive patients a prevalence of 17% was reported for primary SS, for the first time establishing an association between ACA and primary SS [6]
In conclusion, we have found a lower frequency of dry eyes, hypergammaglobulinaemia, anti-Ro/SS-A and antiLa/SS-B autoantibodies and a higher frequency of Raynaud’s phenomenon and dysphagia in ACA positive patients (ACA+/SS) as compared to ACA negative SS patients (ACA-/SS) patients
Summary
A subgroup of patients with primary Sjögren’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. Sjögren’s Syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands. A subset of patients with primary disease, who present features intermediate between SS and limited cutaneous SSc has been previously recognized [4,5,6] Their common characteristic is the finding of anticentromere antibodies (ACA) detected by immunofluorescence on Hep-2 cells. It remains to be answered whether these ACA positive SS patients represent merely a SS subgroup or if they constitute a transitional phase in the evolution to full blown SSc. Our goal was to clinically and immunologically characterize ACA positive SS patients in comparison to both ACA negative SS patients and ACA positive SSc patients, and to determine their tendency to evolve to definite SSc
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