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Development of additional autoimmune diseases in a population of patients with primary Sjögren’s syndrome
  1. M N Lazarus,
  2. D A Isenberg
  1. Centre for Rheumatology, Department of Medicine, University College London, London, UK
  1. Correspondence to:
    Professor D A Isenberg
    Centre for Rheumatology, The Middlesex Hospital, University College London, Arthur Stanley House, 40–50 Tottenham Street, London W1T 4NJ, UK;


Background: To investigate whether patients with primary Sjögren’s syndrome (pSS) have an increased tendency to develop other autoimmune diseases.

Methods: A retrospective case note review was carried out on 114 patients in whom a diagnosis of pSS had been made in a department of rheumatology from 1979 onwards. The year of diagnosis of pSS was recorded, plus the diagnosis and year of diagnosis of any other identified autoimmune disease.

Results: Of the 114 patients with pSS, seven (6%) were male and 107 (94%) female. Mean age at diagnosis of pSS was 53 years (range 21 to 83). Patients were followed up for an average of 10.5 years (range 0 to 23). Thirty eight patients (33.3%) were diagnosed as having another autoimmune disease, while nine (7.9%) had two or more. Thirteen additional autoimmune diseases were identified. Twenty five diagnoses (51.0%) were made before the diagnosis of pSS, three (6.1%) within the same year, and 21 (42.9%) after the diagnosis. Hypothyroidism was the most common autoimmune disease (n = 16).

Conclusions: Although pSS is a relatively benign condition, affected individuals have an increased tendency to develop additional autoimmune diseases. Patients with pSS should be monitored on a regular basis for such diseases.

  • ACR, American College of Rheumatology
  • ANA, antinuclear antibodies
  • pSS, primary Sjögren’s syndrome
  • RF, rheumatoid factor
  • autoimmune disease
  • Sjögren’s syndrome

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