Natural History of Sjögren’s Syndrome Phenotypic Features in the Sjögren’s International Collaborative Clinical Alliance Registry

Caroline Shiboski¹, Alan N. Baer², Mi Y. Lam¹, Stephen Challacombe³, Hector Lanfranchi⁴, Morten Schiødt⁵, Hisanori Umehara⁶, Frederick B. Vivino⁷, Yan Zhao⁸, Yi Dong⁸, Bruce W. Kirkham⁹, Kenneth E. Sack¹⁰, Susumu Sugai¹¹, Cristina F. Vollenweider¹², Wen Zhang⁸, John S. Greenspan¹, Troy Daniels¹³, Lindsey A. Criswell¹⁴ and Sjögren's International Collaborative Clinical Alliance¹⁵, ¹Orofacial Sciences, University of California San Francisco, San Francisco, CA, ²Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, ³Kings College London, London, United Kingdom, ⁴University of Buenos Aires, Buenos Aires, Argentina, ⁵Oral and Maxillofacial Surgery, Rigshospitalet, Copenhagen, Denmark, ⁶Internal Medicine, Division of Hematology and Immunology, Kanazawa Medical University, Ishikawa, Japan, ⁷Medicine, Penn Presbyt Med Ctr, Philadelphia, PA, ⁸Rheumatology, PUMCH, Bejing, China, ⁹Rheum Dept/ Guys Hospital, Guys Hospital, London, United Kingdom, ¹⁰Dept of Medicine/Box0326, Univ of Calif-San Francisco, San Francisco, CA, ¹¹Kanazawa Medical University, Ishikawa, Japan, ¹²Rheumatology, German Hospital, Buenos Aires, Argentina, ¹³Orofacial Sciences, Box 0422, University of California San Francisco, San Francisco, CA, ¹⁴Department of Medicine, University of California, San Francisco, Rosalind Russell Medical Research Center for Arthritis, San Francisco, CA, ¹⁵Orofacial Sciences, University of California San Francisco, University of California San Francisco, CA

Meeting: 2012 ACR/ARHP Annual Meeting

Keywords: Sjogren's syndrome and auto-immunity

Session Information

Title: Sjögren's Syndrome - Clinical

Session Type: Abstract Submissions (ACR)

Background/Purpose: Sjögren’s syndrome (SS) is known to be a relatively stable or slowly progressing disease, however, few studies have actually followed patients over time while taking into account all its components. We explore changes in the phenotypic features (serologic/rheumatologic, oral, and ocular) of SS, and in SS status, using the new American College of Rheumatology (ACR) classification criteria for SS, among participants in the Sjögren’s International Collaborative Clinical Alliance (SICCA) registry over a 2-year time interval.

Methods: SICCA is an international registry enrolling participants with signs and/or symptoms suggestive of SS in 9 centers across 7 countries. It was created to develop new classification criteria, establish a patient data and biospecimen repository and make these available to the scientific community to explore the genotype, phenotype, pathogenesis and epidemiology of this chronic autoimmune disease. All participants found to have any objective measures of salivary hypofunction, ocular dryness, focal lymphocytic sialadenitis in a lip salivary gland (LSG) biopsy, or anti-SSA and/or B antibodies, are recalled 2 years after their baseline examinations to repeat all examinations and specimen collections. We explored change in phenotypic features and in SS status.

Results: As of September 30, 2011, 2510 participants had enrolled in SICCA, and 703, or nearly one third, presented for a 2-year follow-up visit. We found remarkable stability over time of both individual phenotypic features of SS and of SS status. For most phenotypic variables the percent unchanged exceeded 80%, ranging from 77% (for Schirmer’s test) to 96% (for anti-SSA/B). The ocular staining score (OSS that may range from 0 to 12) increased from baseline (median=5) to follow-up (median=6) (p<0.0001; signed-rank test). Among 168 participants found to have SS using the 2012 ACR classification criteria, 90% again met these criteria after 2 years. Among those who did not meet the ACR classification criteria at baseline, 11% had progressed and met them at the follow-up visit. One case of mucosa-associated lymphoid tissue lymphoma was detected in a follow-up LSG biopsy. Three other cases of non-Hodgkin’s lymphoma were diagnosed during the follow-up period by a study-independent physician.

Conclusion: There was remarkable stability over a 2-year time period of both individual phenotypic features of SS and of SS status. This suggests that to fully characterize longitudinal outcomes and progression, a longer follow-up interval may be needed. Funded by NIH/NIDCR/NEI N01-DE32636.

Disclosure:

C. Shiboski,
None;

A. N. Baer,

Merck Serono,

Cellgene,

M. Y. Lam,
None;

S. Challacombe,
None;

H. Lanfranchi,
None;

M. Schiødt,
None;

H. Umehara,
None;

F. B. Vivino,
None;

Y. Zhao,
None;

Y. Dong,
None;

B. W. Kirkham,
None;

K. E. Sack,
None;

S. Sugai,
None;

C. F. Vollenweider,
None;

W. Zhang,
None;

J. S. Greenspan,
None;

T. Daniels,
None;

L. A. Criswell,
None;

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