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Publication Detail
A retrospective study of long-term outcomes in 152 patients with primary Sjogren's syndrome: 25-year experience.
  • Publication Type:
    Journal article
  • Publication Sub Type:
    Journal Article
  • Authors:
    Abrol E, González-Pulido C, Praena-Fernández JM, Isenberg DA
  • Publication date:
  • Pagination:
    157, 164
  • Journal:
    Clin Med (Lond)
  • Volume:
  • Issue:
  • Status:
  • Country:
  • Print ISSN:
  • PII:
  • Language:
  • Keywords:
    Sjögren’s syndrome, autoimmune diseases, long-term follow-up, neoplasia, non-Hodgkin’s lymphoma, Adult, Aged, Autoimmune Diseases, Cohort Studies, Female, Follow-Up Studies, Humans, Lymphatic Diseases, Lymphoma, Non-Hodgkin, Male, Middle Aged, Neoplasms, Odds Ratio, Prognosis, Pulmonary Fibrosis, Retrospective Studies, Risk Factors, Sjogren's Syndrome, Thyroiditis, Autoimmune, Vasculitis
The objective of this study was to evaluate the 25-year outcome of patients with primary Sjögren's syndrome (pSS). One hundred and fifty-two patients diagnosed with pSS (American-European classification criteria) were retrospectively and descriptively analysed (1986-2011). Of all 152 patients, 55.9% were alive, 18.4% had died and 25.7% discontinued follow-up (mostly due to old age). Malignancy affected 28.3% and non-Hodgkin's lymphoma (NHL) affected 10.5%. The adjusted risk for development of NHL was an odds ratio (OR) of 10.5 (95% confidence interval [CI]: 3.05-36.42) in patients with vasculitis (p<0.001), and OR 3.4 (95% CI 1.05-11.2) in the presence of glandular complications (parotid swelling, lymphadenopathy) (p < 0.041). Seventy-five patients (49.3%) developed other autoimmune diseases (autoimmune thyroid disease [15.8%], pulmonary fibrosis [7.2%] and vasculitis [10.5%]). Although the course of pSS is relatively benign, over 25 years patients experience more clinical complications than previously described. In addition, vasculitis and glandular manifestations were significant predictors for NHL.
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