%0 Journal Article %J J Rheumatol %D 2020 %T Sjögren Syndrome without Focal Lymphocytic Infiltration of the Salivary Glands. %A Sharma, Rohan %A Chaudhari, Kaustubh S %A Kurien, Biji T %A Grundahl, Kiely %A Radfar, Lida %A Lewis, David M %A Lessard, Christopher J %A Li, He %A Rasmussen, Astrid %A Sivils, Kathy L %A Scofield, R Hal %K Antibodies, Antinuclear %K Autoantibodies %K Autoantigens %K Biopsy %K Cell Movement %K Gene Expression Regulation %K Histological Techniques %K Humans %K Interferons %K Keratoconjunctivitis Sicca %K Lymphocytes %K Rheumatoid Factor %K Ribonucleoproteins %K RNA, Small Cytoplasmic %K Salivary Glands %K Sjogren's Syndrome %X

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.

%B J Rheumatol %V 47 %P 394-399 %8 2020 Mar %G eng %N 3 %1 https://www.ncbi.nlm.nih.gov/pubmed/31092717?dopt=Abstract %R 10.3899/jrheum.181443