PT  - JOURNAL ARTICLE
AU  - Sharma, Rohan
AU  - Chaudhari, Kaustubh S.
AU  - Kurien, Biji T.
AU  - Grundahl, Kiely
AU  - Radfar, Lida
AU  - Lewis, David M.
AU  - Lessard, Christopher J.
AU  - Li, He
AU  - Rasmussen, Astrid
AU  - Sivils, Kathy L.
AU  - Scofield, R. Hal
TI  - Sjögren Syndrome without Focal Lymphocytic Infiltration of the Salivary Glands
AID  - 10.3899/jrheum.181443
DP  - 2020 Mar 01
TA  - The Journal of Rheumatology
PG  - 394--399
VI  - 47
IP  - 3
4099  - http://www.jrheum.org/content/47/3/394.short
4100  - http://www.jrheum.org/content/47/3/394.full
SO  - J Rheumatol2020 Mar 01; 47
AB  - 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.