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Sjögren Syndrome or Sjögren Disease? The Histological and Immunological Bias Caused by the 2002 Criteria

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Abstract

The current 2002 classification criteria do not cover the broad clinical and immunological heterogeneity of primary Sjögren syndrome (SS), since five of the six criteria focus exclusively on glandular involvement and the remaining criterion is the mandatory presence of anti-Ro/La antibodies. The aim of this study was to analyze the clinical features of patients with a well-established diagnosis of primary SS who do not fulfill the 2002 classification criteria. Five hundred seven patients diagnosed with primary SS (1993 criteria) were consecutively included and followed up. Two hundred twenty-one (44%) patients did not fulfill the 2002 criteria. These patients were older at diagnosis (p < 0.001) and had a lower frequency of parotid enlargement (p = 0.002), fever (p = 0.041), arthritis (p = 0.041), vasculitis (p = 0.050), peripheral neuropathy (p = 0.002), cranial nerve involvement (p = 0.015), raised erythrocyte sedimentation rate ( ESR) levels (p < 0.001), anemia (p < 0.001), leukopenia (p = 0.037), hypergammaglobulinemia (p < 0.001), positive rheumatoid factor ( RF; p = 0.002), and cryoglobulinemia (p = 0.049) in comparison with those fulfilling 2002 criteria. However, there were no significant differences in the prevalence of sicca features, diagnostic tests, overall systemic involvement, antinuclear antibodies , complement levels, development of B-cell lymphoma, or survival. Patients with anti-Ro antibodies had the highest frequencies of systemic features, hematological abnormalities, and altered immunological markers. In conclusion, patients fulfilling the 2002 criteria, who have either a specific histological diagnosis (lymphocytic infiltration) or highly specific autoantibodies (Ro/La), might well be considered to have Sjögren “disease.” In contrast, etiopathogenic mechanisms other than lymphocytic-mediated epithelial damage could be involved in patients with negative Ro and negative biopsy, in whom the term Sjögren “syndrome” seems more adequate.

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Authors and Affiliations

  1. Department of Autoimmune Diseases, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Barcelona, Spain

    Manuel Ramos-Casals, Pilar Brito-Zerón, Marta Perez-De-Lis, Iratxe Jimenez, Maria-Jose Blanco, Albert Bove, Maria-Jose Soto, Miriam Akasbi & Candido Diaz

  2. Department of Public Health, and Statistic Unit, School of Medicine, University of Barcelona, Barcelona, Spain

    Juan Sentís

  3. CAP Les Corts, Grup de Recerca en Atenció Primària, IDIBAPS, IIS, Gesclinic, Hospital Clínic, Barcelona, Spain

    Antoni Siso

  4. Servei de Malalties Autoimmunes, Hospital Clínic, C/Villarroel, 170, 08036, Barcelona, Spain

    Manuel Ramos-Casals & Pilar Brito-Zerón

Authors
  1. Manuel Ramos-Casals
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  2. Pilar Brito-Zerón
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  3. Marta Perez-De-Lis
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  4. Iratxe Jimenez
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  5. Maria-Jose Blanco
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  6. Albert Bove
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  7. Maria-Jose Soto
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  8. Miriam Akasbi
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  9. Candido Diaz
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  10. Juan Sentís
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  11. Antoni Siso
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Corresponding author

Correspondence to Manuel Ramos-Casals.

Additional information

This study was supported by Grants La Marató de TV3 (071810) and Fondo de Investigaciones Sanitarias (080103).

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Ramos-Casals, M., Brito-Zerón, P., Perez-De-Lis, M. et al. Sjögren Syndrome or Sjögren Disease? The Histological and Immunological Bias Caused by the 2002 Criteria. Clinic Rev Allerg Immunol 38, 178–185 (2010). https://doi.org/10.1007/s12016-009-8152-z

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  • DOI: https://doi.org/10.1007/s12016-009-8152-z

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