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J Rheumatol. 2019 May 15. pii: jrheum.181443. doi: 10.3899/jrheum.181443. [Epub ahead of print]

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

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Arthritis & Clinical Immunology Program, Oklahoma Medical Research Foundation; Department of Neurology, University of Arkansas Medical Sciences Center; Medical and Research Services, Oklahoma City Department of Veterans Affairs Medical Center; Departments of Medicine and Pathology, University of Oklahoma Health Sciences Center; College of Dentistry, University of Oklahoma Health Sciences Center; 6Current Affiliation: Human Genome Sequencing Center, Baylor College of Medicine. This work was supported in part by NIH grants AR053483, AR060804, AI0822714, GM104938 as well as US Department of Veterans Affairs grant BX001451. Conflict interest - RHS has received honoraria and travel expenses from Eisai Korea. Address Correspondence to: Hal Scofield, MD 825 NE 13th Street Oklahoma City, OK, USA 73104. E-mail



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.


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.


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.


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.


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