Sjögren Syndrome without Focal Lymphocytic Infiltration of the Salivary Glands.

TitleSjögren Syndrome without Focal Lymphocytic Infiltration of the Salivary Glands.
Publication TypeJournal Article
Year of Publication2020
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 Published2020 Mar
KeywordsAntibodies, Antinuclear, Autoantibodies, Autoantigens, Biopsy, Cell Movement, Gene Expression Regulation, Histological Techniques, Humans, Interferons, Keratoconjunctivitis Sicca, Lymphocytes, Rheumatoid Factor, Ribonucleoproteins, RNA, Small Cytoplasmic, Salivary Glands, Sjogren's Syndrome

OBJECTIVE: Primary Sjögren syndrome (SS) is characterized by a focal lymphocytic infiltrate in exocrine glands. We describe patients who lacked this key feature.

METHODS: 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.

RESULTS: 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.

CONCLUSION: 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.

Alternate JournalJ Rheumatol
PubMed ID31092717
PubMed Central IDPMC7304293
Grant ListU54 GM104938 / GM / NIGMS NIH HHS / United States
U19 AI082714 / AI / NIAID NIH HHS / United States
P30 AR053483 / AR / NIAMS NIH HHS / United States
P50 AR060804 / AR / NIAMS NIH HHS / United States
P30 AR073750 / AR / NIAMS NIH HHS / United States
I01 BX001451 / BX / BLRD VA / United States

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