Background/Purpose: Patients with Sjögren’s syndrome (SS) and negative SSA/SSB serology (ie. seronegative SS) have phenotypic characteristics different than seropositive ones, and thus may constitute a disease subset with a unique pathogenesis. Since these patients are older and have a lower frequency of hypergammaglobulinemia, rheumatoid factor, hypocomplementemia, they might be expected to have a more benign phenotype. However, recent reports highlight that these patients have more severe pain and a higher frequency of small fiber sensory neuropathy. We sought to determine if self-reported neuropathic pain was more prevalent among primary SS participants of the Sjögren’s International Collaborative Clinical Alliance (SICCA).

Methods: We obtained data on 3297 participants (pts) of the 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. Responses of each pt to written questions regarding the presence and frequency of neuropathic pain symptoms were analyzed; these questions were adapted from validated tools for this purpose.

Results: SSA/SSB serology was positive in 1071 and negative in 290 of the primary SS participants. Reports of painful dysesthesia were higher in the seronegative SS pts: “persistent or frequent burning discomfort” (27 vs 18%, p=0.0006; χ²); “sharp ‘jabbing’ needle-like pain” (37 vs 28%, p=0.0038), and “more or less continuous ‘prickling’ or ‘tingling’ feeling” (40 vs 35%, p=0.0846). In contrast, there were no differences in reports of decreased ability to feel surface features, pain/cuts/injuries, and hot from cold. Depression, assessed by responses to the PHQ-9 questionnaire, was more frequent in the seronegative SS pts (scores≥10, 32 vs 22%, p=0.0004). In a multivariate analysis, we found that pts who reported dysesthetic neuropathic pain were 1.6 times more likely to have negative SSA/SSB serology (95% CI 1.1-2.2; p=0.013) than those with positive serology, after controlling for age, gender, ethnicity, duration of dry eye and dry mouth, and depression.

Conclusion: Self-report of neuropathic pain is more prevalent in seronegative primary SS, a finding which is independent of age, gender, ethnicity, duration of sicca symptoms, and concomitant depression.  Although our findings need to be verified with validated neuropathic pain scales, they suggest that seronegative SS has a pathogenesis unique from that of seropositive SS.

Research supported by NIH/NIDCR contract HHSN26S201300057C

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/seronegative-sjgrens-syndrome-is-associated-with-a-higher-frequency-of-patient-reported-neuropathic-pain-an-analysis-of-the-sjgrens-international-collaborative-clinical-alliance-cohort/