Childhood-onset primary Sjögren’s syndrome in a tertiary center in China: clinical features and outcome
Abstract Objectives To characterize the clinical features and outcomes of childhood-onset primary Sjögren’s syndrome (pSS). Methods Patients less than 18 years old who were diagnosed with pSS by paediatric rheumatologists were included, and all patients were applied the 2002 American-European Consen...
| Main Authors: | , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2023-01-01
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| Series: | Pediatric Rheumatology Online Journal |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12969-022-00779-3 |
| _version_ | 1811175926618128384 |
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| author | Yinv Gong Haimei Liu Guomin Li Tao Zhang Yifan Li Wanzhen Guan Qiaoqian Zeng Qianying Lv Xiaomei Zhang Wen Yao Yu Shi Hong Xu Li Sun |
| author_facet | Yinv Gong Haimei Liu Guomin Li Tao Zhang Yifan Li Wanzhen Guan Qiaoqian Zeng Qianying Lv Xiaomei Zhang Wen Yao Yu Shi Hong Xu Li Sun |
| author_sort | Yinv Gong |
| collection | DOAJ |
| description | Abstract Objectives To characterize the clinical features and outcomes of childhood-onset primary Sjögren’s syndrome (pSS). Methods Patients less than 18 years old who were diagnosed with pSS by paediatric rheumatologists were included, and all patients were applied the 2002 American-European Consensus Group (ACEG) criteria, the 2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for pSS, or the 1999 proposed juvenile pSS criteria. The electronic medical records of patients with pSS from 2013 to 2020 were collected and analysed. Results Thirty-nine patients were included. Of them, 27 (69.2%), 38 (97.4%) and 35 (89.7%) patients fulfilled the AECG criteria, ACR/EULAR criteria and proposed juvenile pSS criteria, respectively. The female:male ratio was 3.9:1. The median ages at first signs or symptoms and at diagnosis were 9.2 (4.7, 14.5) years and 10.9 (6.3, 15.0) years, respectively. The main clinical manifestations were rash or purpura (20, 51.3%), followed by fever (12, 30.8%), glandular enlargement/recurrent parotitis (10, 25.6%), and dry mouth and/or dry eyes (9, 23.1%). Twenty-eight (56.4%) patients had systemic damage, the most common of which was haematological involvement (14, 35.9%), followed by hepatic (13, 33.3%) and renal involvement (8, 20.5%). Thirty-eight (97.4%) patients underwent labial minor salivary gland biopsy, and all exhibited focal lymphocytic sialadenitis. All patients had a global ESSDAI score ≥ 1 at diagnosis, and the median total score at diagnosis was 8 (2, 31). Thirty-six (92.3%) patients were followed up for a median time of 23.6 (7.9, 79.5) months, and three patients developed systemic lupus erythematosus (SLE) at follow-up times of 13.3, 38.8 and 63.8 months. Conclusions The presentation of childhood-onset pSS is atypical, and extraglandular manifestations and systemic involvement are more common than in adult-onset pSS. Labial salivary gland biopsy is vital for patients with probable pSS. Some patients may develop SLE over time. |
| first_indexed | 2024-04-10T19:43:53Z |
| format | Article |
| id | doaj.art-29ecce081eb64207a75505dfebccc3f6 |
| institution | Directory Open Access Journal |
| issn | 1546-0096 |
| language | English |
| last_indexed | 2024-04-10T19:43:53Z |
| publishDate | 2023-01-01 |
| publisher | BMC |
| record_format | Article |
| series | Pediatric Rheumatology Online Journal |
| spelling | doaj.art-29ecce081eb64207a75505dfebccc3f62023-01-29T12:06:36ZengBMCPediatric Rheumatology Online Journal1546-00962023-01-012111910.1186/s12969-022-00779-3Childhood-onset primary Sjögren’s syndrome in a tertiary center in China: clinical features and outcomeYinv Gong0Haimei Liu1Guomin Li2Tao Zhang3Yifan Li4Wanzhen Guan5Qiaoqian Zeng6Qianying Lv7Xiaomei Zhang8Wen Yao9Yu Shi10Hong Xu11Li Sun12Department of Rheumatology, Children’ Hospital of Fudan University, National Children’s Medical CenterDepartment of Rheumatology, Children’ Hospital of Fudan University, National Children’s Medical CenterDepartment of Rheumatology, Children’ Hospital of Fudan University, National Children’s Medical CenterDepartment of Rheumatology, Children’ Hospital of Fudan University, National Children’s Medical CenterDepartment of Rheumatology, Children’ Hospital of Fudan University, National Children’s Medical CenterDepartment of Rheumatology, Children’ Hospital of Fudan University, National Children’s Medical CenterDepartment of Rheumatology, Children’ Hospital of Fudan University, National Children’s Medical CenterDepartment of Rheumatology, Children’ Hospital of Fudan University, National Children’s Medical CenterDepartment of Rheumatology, Children’ Hospital of Fudan University, National Children’s Medical CenterDepartment of Rheumatology, Children’ Hospital of Fudan University, National Children’s Medical CenterDepartment of Rheumatology, Children’ Hospital of Fudan University, National Children’s Medical CenterDepartment of Rheumatology, Children’ Hospital of Fudan University, National Children’s Medical CenterDepartment of Rheumatology, Children’ Hospital of Fudan University, National Children’s Medical CenterAbstract Objectives To characterize the clinical features and outcomes of childhood-onset primary Sjögren’s syndrome (pSS). Methods Patients less than 18 years old who were diagnosed with pSS by paediatric rheumatologists were included, and all patients were applied the 2002 American-European Consensus Group (ACEG) criteria, the 2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for pSS, or the 1999 proposed juvenile pSS criteria. The electronic medical records of patients with pSS from 2013 to 2020 were collected and analysed. Results Thirty-nine patients were included. Of them, 27 (69.2%), 38 (97.4%) and 35 (89.7%) patients fulfilled the AECG criteria, ACR/EULAR criteria and proposed juvenile pSS criteria, respectively. The female:male ratio was 3.9:1. The median ages at first signs or symptoms and at diagnosis were 9.2 (4.7, 14.5) years and 10.9 (6.3, 15.0) years, respectively. The main clinical manifestations were rash or purpura (20, 51.3%), followed by fever (12, 30.8%), glandular enlargement/recurrent parotitis (10, 25.6%), and dry mouth and/or dry eyes (9, 23.1%). Twenty-eight (56.4%) patients had systemic damage, the most common of which was haematological involvement (14, 35.9%), followed by hepatic (13, 33.3%) and renal involvement (8, 20.5%). Thirty-eight (97.4%) patients underwent labial minor salivary gland biopsy, and all exhibited focal lymphocytic sialadenitis. All patients had a global ESSDAI score ≥ 1 at diagnosis, and the median total score at diagnosis was 8 (2, 31). Thirty-six (92.3%) patients were followed up for a median time of 23.6 (7.9, 79.5) months, and three patients developed systemic lupus erythematosus (SLE) at follow-up times of 13.3, 38.8 and 63.8 months. Conclusions The presentation of childhood-onset pSS is atypical, and extraglandular manifestations and systemic involvement are more common than in adult-onset pSS. Labial salivary gland biopsy is vital for patients with probable pSS. Some patients may develop SLE over time.https://doi.org/10.1186/s12969-022-00779-3Primary Sjögren's syndromeChildhoodDiagnosisClinical featuresOutcome |
| spellingShingle | Yinv Gong Haimei Liu Guomin Li Tao Zhang Yifan Li Wanzhen Guan Qiaoqian Zeng Qianying Lv Xiaomei Zhang Wen Yao Yu Shi Hong Xu Li Sun Childhood-onset primary Sjögren’s syndrome in a tertiary center in China: clinical features and outcome Pediatric Rheumatology Online Journal Primary Sjögren's syndrome Childhood Diagnosis Clinical features Outcome |
| title | Childhood-onset primary Sjögren’s syndrome in a tertiary center in China: clinical features and outcome |
| title_full | Childhood-onset primary Sjögren’s syndrome in a tertiary center in China: clinical features and outcome |
| title_fullStr | Childhood-onset primary Sjögren’s syndrome in a tertiary center in China: clinical features and outcome |
| title_full_unstemmed | Childhood-onset primary Sjögren’s syndrome in a tertiary center in China: clinical features and outcome |
| title_short | Childhood-onset primary Sjögren’s syndrome in a tertiary center in China: clinical features and outcome |
| title_sort | childhood onset primary sjogren s syndrome in a tertiary center in china clinical features and outcome |
| topic | Primary Sjögren's syndrome Childhood Diagnosis Clinical features Outcome |
| url | https://doi.org/10.1186/s12969-022-00779-3 |
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