Background/Purpose:
Over the past 3 decades, 10 different classification criteria for Sjögren’s syndrome (SS) had been proposed. In 2001 and 2002 222 consecutive patients suspected of having primary SS (pSS) were each evaluated for pSS using the Copenhagen (COP), Californian (CA), European (EU) and American-European consensus group (AECG) criteria. 90 patients could be classified as pSS by at least one of the criteria.(1) The purpose of our current study was to prospectively compare the longitudinal performance of different classification criteria by reassessing the diagnosis in 2009 in the group of patients classified as pSS in 2001 and 2002.
Methods:
Eligible patients were invited for reassessment. In each patient we repeated diagnostic tests as required by any of the respective classification criteria. Additionally, we used the new ACR criteria (ocular stain score ≥3 was replaced by Rose Bengal score ≥ 3 following the Bijsterveld’s method) to classify the patients on the data from the initial cohort and at reassessment.(2)
Results:
In 2009 63/90 (70%) of patients from the initial cohort consented to participate. The flow of the patients is summarized in Figure 1.
During the 7.5-year follow up period we observed a transition from pSS to secondary SS (sSS) in 9/63 (14%) patients on average after 4.0±0.9 years. While cases of transition from pSS to sSS were observed for all criteria used to make the initial diagnosis of pSS, it was significantly more common if the diagnosis of pSS was initially made using AECG (17%, p=0.008), or ACR (17%, p= 0.016) criteria.
In the 34 patients who underwent a full diagnostic reassessment the diagnosis retention rate was statistically significant for the CA, AECG, COP and ACR criteria, but not for the EU criteria (Table 1). At reassessment 3/32 (9%), and 2/26 (8%) of patients initially diagnosed as pSS using the EU, and COP criteria, respectively could not be classified as pSS by any of the criteria.
Although the difference between classification using the AECG, and new ACR criteria almost reached statistical significance for the initial 90 patients (p=0.063), the difference was lost at reassessment (p=1.000).
Conclusion:
The longitudinal diagnosis retention rate is highest for the Californian and AECG criteria and lowest for the European criteria. Regardless of the classification criteria we observed that with time some patients develop sSS. When using classification criteria without mandatory positivity in immunoserology or histology there is a caveat of misclassification of patients as pSS.
Figure 1
Table 1
|
|
Meeting the criteria for pSS |
||||
|
Patients (100%) |
Copenhagen |
Californian |
European |
AECG 2002 |
ACR 2012 |
|
All the 34 pts. before 7.5 years |
26/34(76%) |
12/34(35%) |
32/34(94%) |
24/34(71%) |
22/34 (65%) |
|
pSS not confirmed after 7.5 years |
2/26 (8%) |
0/12 (0%) |
3/32 (9%) |
0/24 (0%) |
0/22 (0%) |
|
pSS confirmed anew after 7.5 years |
2/26 (8%) |
1/12 (8%) |
2/32 (6%) |
1/24 (4%) |
2/22 (9%) |
|
All the 34 pts. at follow-up |
26/34 (76%) |
13/34 (38%) |
31/34 (91%) |
25/34 (74%) |
24/34 (71%) |
|
|
Agreement between initial assessment and reassessment |
||||
|
Cohen’s Kappa (95% confidence interval; significance) |
κ=0.673 (0.377, 0.969; p=0.001) |
κ=0.937 (0.815, 1.000; p<0.001) |
κ=–0.076 (–0.148, –0.004; p=1.000) |
κ=0.927 (0.787, 1.000; p<0.001) |
κ=0.866 (0.688, 1.000; p<0.001) |
References
1. Novljan MP, et al. Scand J Rheumatol 2006;35:463-8.
2. Shiboski SC, et al. Arthritis Care Res 2012;64:475-87.
Disclosure:
M. Plešivčnik Novljan,
None;
Rotar,
None;
A. Ambrožič,
None;
G. Vidmar,
None;
M. Tomšič,
None.
« Back to 2012 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/longitudinal-evaluation-of-the-performance-of-different-classification-criteria-in-patients-with-primary-sjogrens-syndrome/