Associations between salivary gland histopathologic diagnoses and phenotypic features of Sjögren's syndrome among 1,726 registry participants

Abstract

Objectives The Sjögren’s International Collaborative Clinical Alliance (SICCA) is an ongoing NIH-funded registry whose cohort ranges from those with symptoms of possible Sjögren’s syndrome (SS) to those with obvious disease. Using this database we examined associations between labial salivary gland (LSG) histopathology and other phenotypic features of SS. Methods LSG biopsy specimens from SICCA participants underwent protocol-directed histopathological assessments. Among 1726 LSG specimens exhibiting any pattern of sialadenitis, we compared biopsy diagnoses against concurrent salivary, ocular and serological assessments. Results LSG specimens included 61% with focal lymphocytic sialadenitis, (FLS; 66% of which had focus scores [FS] ≥ 1 per 4 mm2) and 38% with non-specific or sclerosing chronic sialadenitis (NS/SCS). FS ≥ 1 was strongly associated with positive serum anti-SS-A/-B, rheumatoid factor and the ocular component of SS, but not with symptoms of dry mouth or eyes. Those with positive anti-SS-A/-B were 9 times more likely to have a FS ≥ 1 (95% CI: 7.4; 11.9) than FS<1 or another pattern, while those with unstimulated whole salivary flow < 0.1 ml/min were only 2 times more likely to have a FS ≥ 1 (95% CI:1.7; 2.8) than FS<1 or another pattern, while controlling for other phenotypic features of SS. Conclusions Distinguishing FLS from NS/SCS is essential in assessing LSG biopsies, before determining FS. A diagnosis of FLS with FS ≥ 1 per 4 mm2, as compared to FLS with FS< 1 or with NS/SCS, was strongly associated with the ocular and serological components of SS and reflects SS autoimmunity.

Is minor salivary gland biopsy more than a diagnostic tool in primary Sjo¨gren׳s syndrome? Association between clinical, histopathological, and molecular features: A retrospective study

“…Taking into account the pivotal role of GCs in driving B-and Tcell activation, it has been suggested that their presence in the pSS glandular infiltrates may affect the clinical picture and the progression of the disease in several ways [57]: (i) the higher degree of lymphocytic organization may lead to a more pronounced disruption of glandular architecture and secretory function impairment [31,58]; (ii) GC formation may increase lymphocyte activation and function, autoantibody production [57], and immune complex formation, leading to extraglandular manifestations [57][58][59]; and (iii) the development of B-cell NHL seems to rely on antigen-driven stimulation [60].…”

Decreased microRNA-181a and -16 expression levels in the labial salivary glands of Sj�gren syndrome patients

“…Specimens from SS patients demonstrated characteristic features of SS, including a salivary gland pathological focus (SGPF) score of ≥1 and presence of lymphoepithelial lesions (16), whereas specimens obtained from the control subjects did not present such features. Subsequently, SS patients were divided into two groups based on their SGPF scores, as follows: Scores of <3 (n=16) and scores of ≥3 (n=12) (17).…”

Clinical and laboratory profiles of primary Sjogren's syndrome in a Chinese population: A retrospective analysis of 315 patients

“…The histological hallmark is a focal lymphocytic infiltration of the exocrine glands, determined by minor salivary gland biopsy (MSGB). [1][2][3][4][5] The incidence of pSS is 0.3-0.7% in China, 6 and there were more female patients than male patients. At present, it is still difficult to establish the diagnosis of pSS because of lack of specific symptoms and laboratory markers.…”