Background/Purpose: Classical connective tissue diseases, such as SLE and RA have well documented progression of disease and damage accrual. However, the natural history of Sjögren’s syndrome (SS) has been less well documented, particularly in research settings where comprehensive multidisciplinary assessments can be performed at more than one timepoint. The objective of the present study was to re-evaluate past participants in the OMRF Sjögren’s research clinic (SRC) with the same protocol that was used for their initial research classification with the goal of documenting changes in their disease.

Methods: Questionnaires assessing health changes and willingness to be re-evaluated were mailed to 800 SRC participants. Twenty respondents (17 SS and 3 incomplete SS [iSS] both by AECG and ACR-EULAR criteria) participated in this pilot study in which all procedures performed in their initial evaluation were repeated.

Results: 356 (45%) questionnaires were answered (161 SS and 195 iSS). Subjectively, respondents reported equal or better status of ocular and oral symptoms but significantly worse fatigue and arthralgias (p<0.001) with no differences between SS and iSS. The 20 re-evaluated patients returned after an average of 5.4 years (range 2-9). Thirteen (65%) retained the initial disease classification, but 6 subjects (30%) went from SS to iSS and 1 (5.0%) from iSS to SS. It is noteworthy that this subject only met SS criteria by AECG and not by ACR-EULAR because his serology was anti-La (+) only with negative biopsy. Furthermore, only 2 subjects had a net increase in the number of criteria, while 18 had the same number (n=8) or fewer (n=10). There were no consistent patterns of change in the objective measures of lacrimal and salivary gland function: 5 subjects became Schirmer’s (+) and 1 reversed to (-); the opposite was the case for the ocular staining, with 5 becoming (-) and 1 becoming (+). The only unchanged results in all the recalls were the anti-Ro/anti-La status. The most intriguing results were the changes in the minor salivary gland biopsy; 4 (20%) subjects went from positive to negative biopsy resulting in a change in classification from SS to iSS in 3 cases. Moreover, the focus score was lower in the second biopsy of 10 (50%) cases, 4 (20%) had a higher score and the remaining 6 (30%) were unchanged. The morphology of the salivary gland tissue of these reversed cases showed extensive fibrosis, fatty infiltration and atrophy of the gland, precluding the lymphocytic infiltrates from meeting the definition of being surrounded by normal tissue. Further supporting the notion of worsening gland architecture, final focus score trended to inversely correlate with WUSF (r=-0.4; p=0.089). Complement levels and hypergammaglobulinemia did not predict stability or worsening of SS.

Conclusion: Re-evaluated patients showed little disease progression but steady presence of autoantibodies. Detrimental changes in salivary gland morphology were observed in later biopsies, even when classified as “negative” using current criteria. These results suggest that significant tissue destruction in long standing disease may lead to false negative biopsy results in spite of progressive glandular dysfunction.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/minimal-progression-of-disease-manifestation-in-patients-with-sjogrens-syndrome-re-evaluated-multiple-years-after-initial-disease-classification/