title: Associations between salivary gland histopathologic diagnoses and phenotypic features of Sjögren's syndrome among 1,726 registry participants.
abstract: OBJECTIVE
  To examine associations between labial salivary gland (LSG) histopathology and other phenotypic features of Sjögren's syndrome (SS).
  
  
  METHODS
  The database of the Sjögren's International Collaborative Clinical Alliance (SICCA), a registry of patients with symptoms of possible SS as well as those with obvious disease, was used for the present study. LSG biopsy specimens from SICCA participants were subjected to protocol-directed histopathologic assessments. Among the 1,726 LSG specimens exhibiting any pattern of sialadenitis, we compared biopsy diagnoses against concurrent salivary, ocular, and serologic features.
  
  
  RESULTS
  LSG specimens included 61% with focal lymphocytic sialadenitis (FLS; 69% of which had focus scores of ≥1 per 4 mm²) and 37% with nonspecific or sclerosing chronic sialadenitis (NS/SCS). Focus scores of ≥1 were strongly associated with serum anti-SSA/SSB positivity, rheumatoid factor, and the ocular component of SS, but not with symptoms of dry mouth or dry eyes. Those with positive anti-SSA/SSB were 9 times (95% confidence interval [95% CI] 7.4-11.9) more likely to have a focus score of ≥1 than were those without anti-SSA/SSB, and those with an unstimulated whole salivary flow rate of <0.1 ml/minute were 2 times (95% CI 1.7-2.8) more likely to have a focus score of ≥1 than were those with a higher flow rate, after controlling for other phenotypic features of SS.
  
  
  CONCLUSIONS
  Distinguishing FLS from NS/SCS is essential in assessing LSG biopsies, before determining focus score. A diagnosis of FLS with a focus score of ≥1 per 4 mm², as compared to FLS with a focus score of <1 or NS/SCS, is strongly associated with the ocular and serologic components of SS and reflects SS autoimmunity.
authors.affiliation: University of California, San Francisco, CA, USA. troy.daniels@ucsf.edu
authors.forename: Troy E
authors.initials: TE
authors.lastname: Daniels
authors.forename: Darren
authors.initials: D
authors.lastname: Cox
authors.forename: Caroline H
authors.initials: CH
authors.lastname: Shiboski
authors.forename: Morten
authors.initials: M
authors.lastname: Schiødt
authors.forename: Ava
authors.initials: A
authors.lastname: Wu
authors.forename: Hector
authors.initials: H
authors.lastname: Lanfranchi
authors.forename: Hisanori
authors.initials: H
authors.lastname: Umehara
authors.forename: Yan
authors.initials: Y
authors.lastname: Zhao
authors.forename: Stephen
authors.initials: S
authors.lastname: Challacombe
authors.forename: Mi Y
authors.initials: MY
authors.lastname: Lam
authors.forename: Yvonne
authors.initials: Y
authors.lastname: De Souza
authors.forename: Julie
authors.initials: J
authors.lastname: Schiødt
authors.forename: Helena
authors.initials: H
authors.lastname: Holm
authors.forename: Patricia A M
authors.initials: PA
authors.lastname: Bisio
authors.forename: Mariana S
authors.initials: MS
authors.lastname: Gandolfo
authors.forename: Toshioki
authors.initials: T
authors.lastname: Sawaki
authors.forename: Mengtao
authors.initials: M
authors.lastname: Li
authors.forename: Wen
authors.initials: W
authors.lastname: Zhang
authors.forename: Beni
authors.initials: B
authors.lastname: Varghese-Jacob
authors.forename: Per
authors.initials: P
authors.lastname: Ibsen
authors.forename: Alicia
authors.initials: A
authors.lastname: Keszler
authors.forename: Nozomu
authors.initials: N
authors.lastname: Kurose
authors.forename: Takayuki
authors.initials: T
authors.lastname: Nojima
authors.forename: Edward
authors.initials: E
authors.lastname: Odell
authors.forename: Lindsey A
authors.initials: LA
authors.lastname: Criswell
authors.forename: Richard
authors.initials: R
authors.lastname: Jordan
authors.forename: John S
authors.initials: JS
authors.lastname: Greenspan
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016208:Databases, Factual; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012042:Registries; D012469:Salivary Glands / Q000473:pathology*; D012793:Sialadenitis / Q000150:complications / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications / Q000473:pathology*; D011795:Surveys and Questionnaires; D014987:Xerostomia / Q000150:complications / Q000473:pathology