RT Journal Article
SR Electronic
T1 Sjögren Syndrome without Focal Lymphocytic Infiltration of the Salivary Glands
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 394
OP 399
DO 10.3899/jrheum.181443
VO 47
IS 3
A1 Rohan Sharma
A1 Kaustubh S. Chaudhari
A1 Biji T. Kurien
A1 Kiely Grundahl
A1 Lida Radfar
A1 David M. Lewis
A1 Christopher J. Lessard
A1 He Li
A1 Astrid Rasmussen
A1 Kathy L. Sivils
A1 R. Hal Scofield
YR 2020
UL http://www.jrheum.org/content/47/3/394.abstract
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.