ABSTRACT
Abstract
Background:
Primary
Sjögren syndrome (pSS) is a chronic
autoimmune disease with its main target being
exocrine glands, and is the
connective tissue disease more frequently associated with other
autoimmune diseases. The aim of this study was to assess the frequency of another autoimmune
rheumatic disease (ARD) developed in primary
Sjögren syndrome (pSS)
patients and to describe it's clinical, serological and histologic characteristics. Materials and
methods:
This is a retrospective
cohort study. Data of
patients with pSS
diagnosis (American-European criteria 2002), included in the GESSAR database (Grupo de Estudio Síndrome de Sjögren, Sociedad
Argentina de Reumatología) were analyzed. The development of a second ARD was registered during the follow up.
Results:
681
patients were included, 94.8%
female. The mean age was 54 (SD 14) years and mean age at
diagnosis of 50 (SD 13) years. The mean follow-up was 4.7 (SD 4.9) years; 30
patients (4.41%, CI 95% 3.1-5.7) developed a second ARD during the follow up,
incidence rate was 9.1/1000
patients-year (IR 95% 5.8-12.4/1000
patients-year), the most frequent being
rheumatoid arthritis (RA). 96% out of these 30
patients had
xerophthalmia, 86.2%
xerostomia, 92% positive Schirmer test, 88.24% positive Rosa Bengala test, lisamine green or Ocular
Staining Score, 81.2% positive unstimulated salivary flow, 82.1% Ro(+) and 33.33% La(+).
Minor salivary gland biopsy had been performed in 14 of the 30
patients, 12 with positive results. There were no statistically significant differences
respect baseline characteristics when comparing the
patients who developed another ARD to the ones that did not.
Conclusions:
Of all the
patients analyzed, 4.4% presented another ARD during their follow-up. It is important to be aware of this, to make an early and proper
diagnosis and
treatment of our
patients. Key points
Patients with primary
Sjögren's Syndrome may develop another
connective tissue disease during follow-up. The most frequently
connective tissue disease developed during follow-up in the
population of
patients with primary
Sjogren's Syndrome studied was
rheumatoid arthritis. It is important to be aware of this to make an early and proper
diagnosis.