|Title||Sjögren Syndrome without Focal Lymphocytic Infiltration of the Salivary Glands.|
|Publication Type||Journal Article|
|Year of Publication||2020|
|Authors||Sharma, R, Chaudhari, KS, Kurien, BT, Grundahl, K, Radfar, L, Lewis, DM, Lessard, CJ, Li, H, Rasmussen, A, Sivils, KL, R Scofield, H|
|Date Published||2020 Mar|
|Keywords||Antibodies, 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 Journal||J Rheumatol|
|PubMed Central ID||PMC7304293|
|Grant List||U54 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