Sjögren's Syndrome without focal lymphocytic infiltration of the salivary glands.

TitleSjögren's Syndrome without focal lymphocytic infiltration of the salivary glands.
Publication TypeJournal Article
Year of Publication2019
AuthorsSharma, R, Chaudhari, KS, Kurien, BT, Grundahl, K, Radfar, L, Lewis, DM, Lessard, CJ, Li, H, Rasmussen, A, Sivils, KL, R Scofield, H
JournalJ Rheumatol
Date Published2019 May 15

OBJECTIVE: Primary Sjögren's syndrome (SS) is characterized by a focal lymphocytic infiltrate in exocrine glands. We undertook this study to describe patients that lacked this key feature.

METHODS: We evaluated subjects 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. Extra-glandular manifestations were also determined. We categorized subjects as high, intermediate, or low in terms of the expression of interferon-regulated genes.

RESULTS: About 20% (51 of 229, 22%) of those classified as primary Sjögren's syndrome had a focus score of zero. Compared to those with anti-Ro positivity and a focus score >1.0, the focus score zero patients (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. In addition, the focus score zero patients were less likely to have elevated expression of interferon-regulated genes in peripheral blood mononuclear cells than anti-Ro positive SS subjects with a focal salivary infiltrate.

CONCLUSION: There are only a few clinical differences between primary Sjögren's syndrome patients with focus score zero and those with both anti-Ro and a focus score >1.0. Nonetheless, the small subset of focus score zero patients tested did not have elevated expression of interferon-regulated genes, but did have systemic disease. Thus, extra-glandular manifestations are perhaps more related to the presence of anti-Ro than increased interferon. This may have relevance to Sjögren's syndrome pathogenesis.

Alternate JournalJ. Rheumatol.
PubMed ID31092717