Peer-reviewed article
Clinical, immunological, and radiological characteristics of pulmonary involvement in childhood-onset primary Sjögren’s syndrome: a single-center retrospective study
Jiayun Ling, 吴同玉, Xiaohui Liu, Zhuqiang Wu, Caihui Yuan ·
Summary
Written by AI, not yet AICS's view · claude-sonnet-5-5This single-hospital retrospective study in China looked at lung involvement in 17 children with primary Sjögren's syndrome, a rare autoimmune disease. About half (9 of 17) had abnormal chest CT scans. Children with abnormal scans were younger at onset, and those with interstitial lung involvement were youngest and had different T and B lymphocyte proportions. In the 7 children with follow-up scans, the lung changes cleared completely in 3, improved in 2 and stayed stable in 2.
Key points
- 14 of 17 children (82.4%) were female, and the mean age at onset was 10.83 years.
- Abnormal chest CT findings were seen in 9 children (52.9%); pleural thickening was the most common (77.8% of those with abnormal CT).
- Age at onset was lower in the abnormal CT group than in the normal CT group (9.37 vs. 12.48 years, P = 0.036).
- 5 children had interstitial lung involvement, and 3 of them met radiological criteria for interstitial lung disease (2 organizing pneumonia, 1 nonspecific interstitial pneumonia).
- Among 7 children with follow-up imaging, 3 normalized completely, 2 improved and 2 remained stable.
Limitations
The study is small (17 children), retrospective and from one hospital, so the findings cannot be generalised. The authors describe the links with age and lymphocyte subsets as exploratory, and only 7 children had follow-up imaging.
Abstract
Objective To describe pulmonary involvement in childhood-onset primary Sjögren’s syndrome (cSS). Methods This retrospective study included 17 children with cSS diagnosed at Jiangxi Provincial Children’s Hospital between August 2021 and August 2025. Patients were classified by baseline chest high-resolution computed tomography (HRCT)as normal CT (n=8) or abnormal CT (n=9); among patients with abnormal CT, 5 had interstitial lung involvement. Clinical, laboratory, radiological, and outcome data were compared. Results Among 17 children, 14 were female (82.4%), and the mean age at onset was 10.83 ± 3.12 years. Abnormal chest CT findings were detected in 9 patients (52.9%). Age at onset was younger in the abnormal CT group than in the normal CT group (9.37 ± 2.67 vs. 12.48 ± 2.88 years, P = 0.036), and was lowest in the interstitial lung involvement subgroup (8.30 ± 2.51 years, P = 0.022). This subgroup had higher total T and CD4+ T lymphocyte proportions and a lower B lymphocyte proportion P = 0.026, 0.027, and 0.023, respectively. Pleural thickening was the most common CT abnormality (77.8%), followed by ground-glass opacities and nodules >3 mm (33.3% each). Among the 5 patients with interstitial lung involvement, 3 met radiological criteria for ILD, comprising 2 with organizing pneumonia and 1 with nonspecific interstitial pneumonia. During follow-up, 3 of 7 patients with longitudinal imaging achieved complete radiological normalization, 2 improved, and 2 remained stable. Conclusion Pulmonary abnormalities were common in children with cSS. Younger onset and aberrant T/B lymphocyte subsets showed exploratory associations with pulmonary involvement, which appeared mainly inflammatory and demonstrated favorable radiological evolution during follow-up.