title: The prognostic value of routinely performed minor salivary gland assessments in primary Sjögren's syndrome.
abstract: OBJECTIVE
  To investigate the prognostic value of the lymphocytic focus score (LFS) and the percentages of IgA+, IgM+ and IgG+ plasma cells for disease severity of primary Sjögren syndrome (pSS).
  
  
  METHODS
  Medical charts of 174 pSS patients were retrospectively analysed, comparing histology results (LFS and percentages of IgA+, IgM+ and IgG+ plasma cells) with disease outcomes as non-Hodgkin lymphoma (NHL) and clinical scores including cumulative EULAR (European League against Rheumatism) Sjögren syndrome disease activity index (ESSDAI) and the total number of extraglandular manifestations.
  
  
  RESULTS
  The mean LFS was significantly higher in patients developing NHL (3.0±0.894 vs 2.25±1.086; p=0.021). The threshold of ≥3 foci has a positive predictive value of 16% for lymphoma, and a negative predictive value of 98%. Only LFS ≥3 contributed significantly and independently to NHL development in a standard multiple regression model. Ig class distribution of plasma cells did not help to identify patients developing lymphoma. Patients with LFS ≥3, ≤40% IgA+ or ≥25% IgM+ plasma cells in salivary gland biopsy specimens had significantly enhanced systemic disease.
  
  
  CONCLUSIONS
  Routine histopathological minor salivary gland assessment has important prognostic value. The LFS might help to identify patients with an increased risk for lymphoma.
authors: Department of Rheumatology & Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: Anna P
authors: AP
authors: Risselada
authors: Department of Rheumatology & Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: Aike A
authors: AA
authors: Kruize
authors: Department of Pathology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: Roel
authors: R
authors: Goldschmeding
authors: Department of Rheumatology & Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: Floris P J G
authors: FP
authors: Lafeber
authors: Department of Rheumatology & Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: Johannes W J
authors: JW
authors: Bijlsma
authors: Department of Rheumatology & Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: Joel A G
authors: JA
authors: van Roon
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000378:metabolism; D007074:Immunoglobulin G / Q000378:metabolism; D007075:Immunoglobulin M / Q000378:metabolism; D008214:Lymphocytes / Q000276:immunology / Q000473:pathology; D008228:Lymphoma, Non-Hodgkin / Q000453:epidemiology / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000276:immunology* / Q000378:metabolism; D011237:Predictive Value of Tests; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D012470:Salivary Glands, Minor / Q000276:immunology* / Q000473:pathology*; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000453:epidemiology / Q000276:immunology* / Q000473:pathology*
chemical_list: D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M
keywords: Disease Activity; Outcomes Research; Sjøgren's Syndrome