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Distinct clinical phenotypes of primary Sjögren’s syndrome differ by onset age: a retrospective study of 742 cases and review of the literature


1, 2, 3, 4, 5, 6, 7, 8, 9, 10

 

  1. Traditional Chinese Medicine Department of Rheumatism, China-Japan Friendship Hospital, Beijing, and Beijing Key Laboratory of Immune Inflammatory Disease, Beijing, China.
  2. Graduate School, Beijing University of Chinese Medicine, Beijing, China.
  3. Graduate School, Beijing University of Chinese Medicine, Beijing, China.
  4. Traditional Chinese Medicine Department, Peking University Third Hospital, Beijing, China.
  5. Graduate School, Beijing University of Chinese Medicine, Beijing, China.
  6. Traditional Chinese Medicine Department, Peking University Third Hospital, Beijing, China.
  7. Traditional Chinese Medicine Department, Peking University Third Hospital, Beijing, China.
  8. Traditional Chinese Medicine Department, Peking University Third Hospital, Beijing, China.
  9. Traditional Chinese Medicine Department of Rheumatism, China-Japan Friendship Hospital, Beijing, and Beijing Key Laboratory of Immune Inflammatory Disease, Beijing, China.
  10. Traditional Chinese Medicine Department of Rheumatism, China-Japan Friendship Hospital, Beijing, and Beijing Key Laboratory of Immune Inflammatory Disease, Beijing, China. taoqg1@sina.com

CER16210
2022 Vol.40, N°12
PI 2373, PF 2380
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PMID: 36441650 [PubMed]

Received: 12/09/2022
Accepted : 11/11/2022
In Press: 22/11/2022
Published: 20/12/2022

Abstract

OBJECTIVES:
To study the clinical characteristics of primary Sjögren’s syndrome (pSS) with different onset age, and perform a review of the literature to confirm if the clinical phenotypes are affected by onset age in patients with pSS.
METHODS:
Data of 742 patients with pSS were retrospectively analysed. Patients were divided into three groups according to onset age: young-onset pSS (YopSS, <35 years), adult-onset pSS (AopSS, ≥35 and ≤65 years), and elderly-onset pSS (EopSS, >65 years). Clinical characteristics were compared among three groups and further multiple comparisons were conducted by Bonferroni adjustment. The Chi-squared test for linear-by-linear association was used to explore variation tendency.
RESULTS:
This study included 105 (14.2%), 533 (71.8%), and 104 (14.0%) cases of YopSS, AopSS, and EopSS, respectively. YopSS demonstrated lower prevalence of dry mouth, abnormal Schirmer I tests, and interstitial lung disease (ILD), but higher proportions of low C3 and C4 levels, and ANA, anti-SSA, anti-SSB, and rheumatoid factor (RF) positivity than AopSS and EopSS. The proportions of dry mouth (p=0.004), abnormal Schirmer I tests (p=0.002), and ILD (p<0.001) tended to increase with the increase of onset age, while the prevalence of leukopenia (p=0.011), low C3 (p=0.001), low C4 (p=0.001), and ANA (p<0.001), anti-SSA (p<0.001), anti-SSB (p<0.001) and RF (p<0.001) positivity tended to decrease with an increase in onset age.
CONCLUSIONS:
YopSS demonstrated less dryness and ILD, but more immunologic disorders. ILD prevalence were directly proportional to onset age of pSS; however, leukopenia, hypocomplementaemia, and autoantibody positivity showed opposite trends.

DOI: https://doi.org/10.55563/clinexprheumatol/b4z1qu

Rheumatology Article