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- Title
Sjögren Syndrome without Focal Lymphocytic Infiltration of the Salivary Glands.
- Authors
Sharma, Rohan; Chaudhari, Kaustubh S.; Kurien, Biji T.; Grundahl, Kiely; Radfar, Lida; Lewis, David M.; Lessard, Christopher J.; He Li; Rasmussen, Astrid; Sivils, Kathy L.; Scofield, R. Hal; Li, He
- Abstract
<bold>Objective: </bold>Primary Sjögren syndrome (SS) is characterized by a focal lymphocytic infiltrate in exocrine glands. We describe patients who lacked this key feature.<bold>Methods: </bold>We evaluated patients with sicca in a comprehensive clinic at which medical, dental, and ophthalmological examinations were performed. All subjects underwent a minor salivary gland biopsy with focus score calculation. Extraglandular manifestations were also determined. We categorized subjects as high, intermediate, or low in terms of expression of interferon (IFN)-regulated genes.<bold>Results: </bold>About 20% (51 of 229, 22%) of those classified as having primary SS had a focus score of zero. Compared to those with anti-Ro positivity and a focus score > 1.0, the patients with focus score of zero (who by classification criteria must be anti-Ro-positive) were statistically less likely to have anti-La (or SSB) and elevated immunoglobulin, as well as less severe corneal staining. The focus score zero patients were less likely to have elevated expression of IFN-regulated genes in peripheral blood mononuclear cells than anti-Ro-positive SS patients with a focal salivary infiltrate.<bold>Conclusion: </bold>There are only a few clinical differences between patients with primary SS with focus score zero and those with both anti-Ro and a focus score > 1.0. The small subset of focus score zero patients tested did not have elevated expression of IFN-regulated genes, but did have systemic disease. Thus, extraglandular manifestations are perhaps more related to the presence of anti-Ro than increased IFN. This may have relevance to pathogenesis of SS.
- Subjects
SJOGREN'S syndrome; INTERFERONS; AUTOANTIBODIES; EXOCRINE glands; SALIVARY glands; RESEARCH; BIOPSY; RESEARCH methodology; KERATOCONJUNCTIVITIS sicca; RNA; MEDICAL cooperation; EVALUATION research; LYMPHOCYTES; CELL motility; COMPARATIVE studies; HISTOLOGICAL techniques; GENES; RESEARCH funding; CARRIER proteins; ANTIGENS
- Publication
Journal of Rheumatology, 2020, Vol 47, Issue 3, p394
- ISSN
0315-162X
- Publication type
journal article
- DOI
10.3899/jrheum.181443