Date: Sunday, November 8, 2015
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: Anti-centromere antibodies (ACA) define a subgroup of Sjögren’s syndrome (SS) patients who are often older, have more frequent Raynaud’s phenomenon, and a lower frequency of anti-SSA and anti-SSB antibodies, hyperglobulinemia, rheumatoid factor, and leucopenia. We sought to determine whether the presence of ACA is associated with more severe impairment of exocrine glandular function in SS.
Methods: We obtained data on 3297 participants of the Sjögren’s International Collaborative Clinical Alliance (SICCA) registry with suspected or established SS for whom there were complete data on the three objective criteria for SS (as defined by the ACR classification criteria). We identified 1361 who could be classified with SS by these criteria and who did not have a definite diagnosis of an underlying connective tissue disease by criteria extant at the time of SICCA registration. The presence of anti-centromere antibodies was determined by immunofluorescence on HEp-2 cells and was performed as part of the routine serologic evaluation of SICCA participants (Quest Diagnostics, Madison, NJ).
Results: ACA were present in 82 (6%) of the 1361 primary SS participants and were associated with an older median age (59 vs 52; p<0.0001), female gender (99 vs 93%; p=0.0379), and lower frequencies of anti-SSA and/or anti-SSB antibodies (29 vs 82%, p<0.0001), rheumatoid factor (39 vs 60%, p=0.0003), and IgG>1445 mg/dl (30 vs 58%). Median focus score was higher in the ACA (+) patients: 2.8 vs 2.5, p=0.0433). There was an increased frequency of Raynaud’s phenomenon (62 vs 28%, p<0.0001), sclerodactyly (16 vs 1%, p<0.0001) and dilated nailfold capillary loops (20 vs 5%, p<0.0001) in the ACA (+) vs ACA (-) subjects. The 2013 Systemic Sclerosis classification criteria were fulfilled by 14/82 (17%) of the ACA (+) subjects. Exocrine gland function was worse in the ACA (+) compared to the ACA (-) subjects: Schirmer’s test value (mean of both eyes): median 4.5 vs 6.5 mm/5 min, p=0.0002; unstimulated whole saliva flow rate: median 0.077 vs 0.367 ml/5 min, p<0.0001. In univariate analyses, duration of dry eye and dry mouth symptoms was not associated with ACA status. Individuals with ACA had an increased risk of UWSF<0.1 ml/min [OR=12.1 (95% CI, 4.9-40.7)] and Schirmer value <5 mm/5 min [OR=2.5 (95% CI, 1.5-4.4) after correcting for age, gender, presence of anti-SSA/SSB, and focus score.
Conclusion: ACA are uncommon in primary SS but define a subset characterized by more severe impairment of exocrine glandular function, in addition to older age and a lower frequency of anti-SSA/SSB antibodies, rheumatoid factor and hyperglobulinemia. This impairment of glandular function is independent of age, gender, focus score, and SSA/SSB serology. Although the majority have Raynaud’s phenomenon, only a minority have sufficient other clinical features to satisfy the 2013 classification criteria for systemic sclerosis.
Research supported by NIH/NIDCR contract HHSN26S201300057C
To cite this abstract in AMA style:Baer AN, Medrano L, McAdams-Demarco M. Anti-Centromere Antibodies Are Associated with More Severe Exocrine Glandular Dysfunction in Primary Sjögren’s Syndrome: An Analysis of the Sjögren’s International Collaborative Clinical Alliance Cohort [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/anti-centromere-antibodies-are-associated-with-more-severe-exocrine-glandular-dysfunction-in-primary-sjgrens-syndrome-an-analysis-of-the-sjgrens-international-collaborative-clinical-all/. Accessed January 21, 2020.
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