Background/Purpose: (1) To analyze the clinical presentation and the clinical course of Sjögren’s Syndrome (pSS) in Caucasian patients with an “early-onset” pSS and (2) to compare the characteristics of the disease between this group of patients and a control group of patients with pSS and a “typical onset” of the disease.

Methods:

A retrospective systematic search through an Italian computerized pSS database, including 1445 patients, was performed in order to select the patients with an “early-onset” of the disease who were defined as those patients aged below the 10th percentile at the time of pSS diagnosis. All the patients enrolled in the database have been recruited at 5 Rheumatology University Italian medical centers and the data have been systematically entered in a standardized form and updated at regular intervals during follow-up since 2009. For all patients, the following parameters were retrieved: age at diagnosis, sex, disease duration, glandular and extra-glandular disease-related manifestations, laboratory features, serological profiles, medical treatments and lymphoproliferative complications. Categorical variables were compared using chi square test; continuous variables were compared using Student’s t-test. A 2-tailed value of p < 0.05 was taken to indicate statistical significance.

Results:

The systematic search selected 1192 pSS patients out of the entire cohort (AECG 2002). Median (IQR) age at pSS diagnosis was 52 (42-62) years and the tenth percentile was 33 years. By using the tenth percentile as a cut-off, we identified 125/1192 (10.5%) as the “early-onset” group whereas the remaining 1067/1192 (89.5%) represented the control group. Median (IQR) disease duration was significantly longer in the “early-onset” group (7 (3-14) versus 3 (1-8) years, p≤0.0001).  Patients with an early disease onset presented a lower frequency of subjective dry mouth (p=0.003) and a higher prevalence of parotid enlargement (p≤0.0001), Raynaud’s phenomenon (p=0.04) and tubular renal disease (p=0.04). Moreover, patients with an “early-onset” pSS presented more frequently a positivity for rheumatoid factor (RF) (p≤0.0001), anti-Ro/SS-A (p≤0.0001), and anti-La/SS-B antibodies (p≤0.0001), low C3 levels (p=0.006), hyper-gammaglobulinemia (p≤0.0001), and leukocytopenia (p=0.006). No differences were detected between the two groups regarding low C4 levels, cryoglobulins, purpura, peripheral nervous system involvement and lymphoproliferative complications.

Conclusion: The age at the onset of pSS may influence the diagnostic algorithm of the disease due to the lower prevalence of subjective sicca symptoms. Parotid enlargement, kidney involvement, laboratory and serological abnormalities seemed to be distinctive features of the “early-onset” pSS. However, despite the higher frequency of parotid gland enlargement, patients with “early-onset” pSS apparently did not present a phenotype at higher risk of lymphoma.  

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/how-does-a-younger-age-at-the-onset-of-sjogrens-syndrome-pss-influence-the-clinical-presentation-and-the-clinical-course-of-the-disease/