American College of Rheumatology Classification Criteria for Sjögren's Syndrome: a Data-driven, Expert Consensus Approach in the Sjögren's International Collaborative Clinical Alliance Cohort

Overview

Journal Arthritis Care Res (Hoboken)

Specialty Rheumatology

Date 2012 May 8

PMID 22563590

Citations 494

Authors

S C Shiboski

C H Shiboski

L A Criswell

A N Baer

S Challacombe

H Lanfranchi

M Schiodt

H Umehara

F Vivino

Y Zhao

Y Dong

D Greenspan

A M Heidenreich

P Helin

B Kirkham

K Kitagawa

G Larkin

M Li

T Lietman

J Lindegaard

N McNamara

K Sack

P Shirlaw

S Sugai

C Vollenweider

J Whitcher

A Wu

S Zhang

W Zhang

J S Greenspan

T E Daniels

Affiliations

Soon will be listed here.

Abstract

Objective: We propose new classification criteria for Sjögren's syndrome (SS), which are needed considering the emergence of biologic agents as potential treatments and their associated comorbidity. These criteria target individuals with signs/symptoms suggestive of SS.

Methods: Criteria are based on expert opinion elicited using the nominal group technique and analyses of data from the Sjögren's International Collaborative Clinical Alliance. Preliminary criteria validation included comparisons with classifications based on the American–European Consensus Group (AECG) criteria, a model-based “gold standard”obtained from latent class analysis (LCA) of data from a range of diagnostic tests, and a comparison with cases and controls collected from sources external to the population used for criteria development.

Results: Validation results indicate high levels of sensitivity and specificity for the criteria. Case definition requires at least 2 of the following 3: 1) positive serum anti-SSA and/or anti-SSB or (positive rheumatoid factor and antinuclear antibody titer >1:320), 2) ocular staining score >3, or 3) presence of focal lymphocytic sialadenitis with a focus score >1 focus/4 mm2 in labial salivary gland biopsy samples. Observed agreement with the AECG criteria is high when these are applied using all objective tests. However, AECG classification based on allowable substitutions of symptoms for objective tests results in poor agreement with the proposed and LCA-derived classifications.

Conclusion: These classification criteria developed from registry data collected using standardized measures are based on objective tests. Validation indicates improved classification performance relative to existing alternatives, making them more suitable for application in situations where misclassification may present health risks.

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References

Shearn M . Sjögren's syndrome. Major Probl Intern Med. 1971; 2:1-262. View

Daniels T, Criswell L, Shiboski C, Shiboski S, Lanfranchi H, Dong Y . An early view of the international Sjögren's syndrome registry. Arthritis Rheum. 2009; 61(5):711-4. PMC: 2819471. DOI: 10.1002/art.24397. View

Skopouli F, Drosos A, Papaioannou T, Moutsopoulos H . Preliminary diagnostic criteria for Sjögren's syndrome. Scand J Rheumatol Suppl. 1986; 61:22-5. View

Moutsopoulos H, Webber B, Vlagopoulos T, Chused T, Decker J . Differences in the clinical manifestations of sicca syndrome in the presence and absence of rheumatoid arthritis. Am J Med. 1979; 66(5):733-6. DOI: 10.1016/0002-9343(79)91110-0. View

Qu Y, Tan M, Kutner M . Random effects models in latent class analysis for evaluating accuracy of diagnostic tests. Biometrics. 1996; 52(3):797-810. View

Daniels T, Cox D, Shiboski C, Schiodt M, Wu A, Lanfranchi H . Associations between salivary gland histopathologic diagnoses and phenotypic features of Sjögren's syndrome among 1,726 registry participants. Arthritis Rheum. 2011; 63(7):2021-30. PMC: 3128201. DOI: 10.1002/art.30381. View

Whitcher J, Shiboski C, Shiboski S, Heidenreich A, Kitagawa K, Zhang S . A simplified quantitative method for assessing keratoconjunctivitis sicca from the Sjögren's Syndrome International Registry. Am J Ophthalmol. 2009; 149(3):405-15. PMC: 3459675. DOI: 10.1016/j.ajo.2009.09.013. View

Fox R, Robinson C, Curd J, Kozin F, Howell F . Sjögren's syndrome. Proposed criteria for classification. Arthritis Rheum. 1986; 29(5):577-85. DOI: 10.1002/art.1780290501. View

Pepe M, Janes H . Insights into latent class analysis of diagnostic test performance. Biostatistics. 2006; 8(2):474-84. DOI: 10.1093/biostatistics/kxl038. View

10.

Vitali C, Bombardieri S, Moutsopoulos H, BALESTRIERI G, Bencivelli W, Bernstein R . Preliminary criteria for the classification of Sjögren's syndrome. Results of a prospective concerted action supported by the European Community. Arthritis Rheum. 1993; 36(3):340-7. DOI: 10.1002/art.1780360309. View

11.

Feenstra R, Tseng S . Comparison of fluorescein and rose bengal staining. Ophthalmology. 1992; 99(4):605-17. DOI: 10.1016/s0161-6420(92)31947-5. View

12.

Manthorpe R, ISAGER H, Prause J . Sjögren's syndrome. A review with emphasis on immunological features. Allergy. 1981; 36(3):139-53. DOI: 10.1111/j.1398-9995.1981.tb01829.x. View

13.

Masaki Y, Dong L, Kurose N, Kitagawa K, Morikawa Y, Yamamoto M . Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis. 2008; 68(8):1310-5. DOI: 10.1136/ard.2008.089169. View

14.

Baughman A, Bisgard K, Cortese M, Thompson W, Sanden G, Strebel P . Utility of composite reference standards and latent class analysis in evaluating the clinical accuracy of diagnostic tests for pertussis. Clin Vaccine Immunol. 2007; 15(1):106-14. PMC: 2223853. DOI: 10.1128/CVI.00223-07. View

15.

See C, Alemayehu W, Melese M, Zhou Z, Porco T, Shiboski S . How reliable are tests for trachoma?--a latent class approach. Invest Ophthalmol Vis Sci. 2011; 52(9):6133-7. PMC: 3176003. DOI: 10.1167/iovs.11-7419. View

16.

Singh J, Solomon D, Dougados M, Felson D, Hawker G, Katz P . Development of classification and response criteria for rheumatic diseases. Arthritis Rheum. 2006; 55(3):348-52. DOI: 10.1002/art.22003. View

17.

Theander E, Vasaitis L, Baecklund E, Nordmark G, Warfvinge G, Liedholm R . Lymphoid organisation in labial salivary gland biopsies is a possible predictor for the development of malignant lymphoma in primary Sjögren's syndrome. Ann Rheum Dis. 2011; 70(8):1363-8. PMC: 3128323. DOI: 10.1136/ard.2010.144782. View

18.

Fink A, Kosecoff J, Chassin M, Brook R . Consensus methods: characteristics and guidelines for use. Am J Public Health. 1984; 74(9):979-83. PMC: 1651783. DOI: 10.2105/ajph.74.9.979. View

19.

Vitali C, Bombardieri S, Jonsson R, Moutsopoulos H, Alexander E, Carsons S . Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002; 61(6):554-8. PMC: 1754137. DOI: 10.1136/ard.61.6.554. View

20.

BLOCH K, BUCHANAN W, WOHL M, Bunim J . SJOEGREN'S SYNDROME. A CLINICAL, PATHOLOGICAL, AND SEROLOGICAL STUDY OF SIXTY-TWO CASES. Medicine (Baltimore). 1965; 44:187-231. View