why choose us

300×250 Ad Slot

Research Article: Clinical, immunological, and radiological characteristics of pulmonary involvement in childhood-onset primary Sjögren’s syndrome: a single-center retrospective study

Date Published: 2026-10-06

Abstract:
To describe pulmonary involvement in childhood-onset primary Sjögren’s syndrome (cSS). 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. 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. 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.

Introduction:
Primary Sjögren’s syndrome (pSS) is a chronic systemic autoimmune disease pathologically characterized by lymphocytic infiltration of the exocrine glands. It can involve multiple organs and cause irreversible functional impairment, substantially affecting long-term prognosis and quality of life ( 1 , 2 ). Childhood-onset primary Sjögren’s syndrome (cSS) is rare ( 3 ), accounting for approximately 1% of all pSS cases in global multicenter cohorts ( 4 , 5 ). Compared with adult-onset pSS, cSS shows greater clinical…

Read more

300×250 Ad Slot