Histopathological environment besides the focus score in Sjögren's syndrome

Int J Rheum Dis. 2014 Nov;17(8):898-903. doi: 10.1111/1756-185X.12502. Epub 2014 Oct 8.


Aim: To compare the prevalence of diverse histopathologic features among patients with Sjögren's syndrome (SS) and controls, and to evaluate their relationship with age, a focus score (FS) ≥ 1 and some clinical and serological SS features.

Methods: A blinded pathologist examined 63 SS and 11 control minor salivary gland biopsies. Focal lymphocytic sialadenitis (FLS) was defined as a focus score (FS) ≥ 1. We also evaluated lymphoepithelial lesions, germinal centers (GCs), epithelial metaplasia, dilatation and hyperplasia in the main secretory duct, perivascular cell infiltrate, adipose infiltration, acinar atrophy, interstitial fibrosis and lymphocytes/plasma cells remote from the FLS. We registered demographics, anti-Ro/La status and clinical features. We used Kendall's tau coefficients and logistic regression analysis.

Results: Sjögren's syndrome patients had a higher frequency of FS ≥ 1 (92% vs. 27%), acinar atrophy (78% vs. 18%), lymphocytes and plasma cells external to the FSL (92% vs. 64%) and stromal fibrosis (68% vs. 36%). A FS ≥ 1 correlated with the presence of GCs and acinar atrophy; whereas age correlated with duct dilation, duct epithelial hyperplasia, adipose infiltration and fibrosis. SS patients with hepatic involvement exhibited more frequent duct dilatation. After adjusting by age, anti-Ro/SSA (odds ratio [OR] 30.8, 95% CI 2.2-423.5, P = 0.01), a FS ≥ 1 (OR 54.3, 95% CI 4.8-612, P = 0.001) and fibrosis (OR 15.2, 95% CI 1.2-186.2, P = 0.03) were associated with SS.

Conclusion: Other histologic findings coexist with FLS, but only GC formation and acinar atrophy correlated with a FS ≥ 1. Age is mostly correlated with the remaining histological features. However, the clinical relevance of these findings is unknown.

Keywords: Sjögren's syndrome; histopathology; lip biopsy.

MeSH terms

  • Acinar Cells / pathology
  • Adult
  • Female
  • Humans
  • Logistic Models
  • Male
  • Middle Aged
  • Retrospective Studies
  • Salivary Ducts / pathology
  • Sialadenitis / pathology*
  • Sjogren's Syndrome / pathology*