Clinical features of primary Sjögren syndrome with purpura
Objective: To study the clinical characteristics of patients with primary Sjögren syndrome(pSS) with purpura. Methods: A total of 101 patients with pSS were enrolled from January 2017 through January 2020 in Department of Dermatology, Ruijin Hospital, Shanghai Jiao Tong University School...
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Format: | Article |
Language: | zho |
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Editorial Office of Journal of Diagnostics Concepts & Practice
2021-04-01
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Series: | Zhenduanxue lilun yu shijian |
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Online Access: | http://www.qk.sjtu.edu.cn/jdcp/fileup/1671-2870/PDF/1671-2870-2021-20-2-155.pdf |
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author | ZHAO Qian, ZHAO Xiaoqing, DIAO Licheng, SUN Fei, ZHENG Jie, ZHU Xuemei, CAO Hua |
author_facet | ZHAO Qian, ZHAO Xiaoqing, DIAO Licheng, SUN Fei, ZHENG Jie, ZHU Xuemei, CAO Hua |
author_sort | ZHAO Qian, ZHAO Xiaoqing, DIAO Licheng, SUN Fei, ZHENG Jie, ZHU Xuemei, CAO Hua |
collection | DOAJ |
description | Objective: To study the clinical characteristics of patients with primary Sjögren syndrome(pSS) with purpura. Methods: A total of 101 patients with pSS were enrolled from January 2017 through January 2020 in Department of Dermatology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine. The patients were divided into 2 groups: pSS with purpura (purpura group, n=15) and without purpura (non-purpura group, n=86). The clinical presentations and laboratory data were compared between the 2 groups. Results: Of the 15 patients with manifestation of purpura (14.9%), 12(80%) were diagnosed as hypergammaglobulinemia purpura and the other 3(20%) were cryoglobulinemia purpura, thrombocytopenic purpura, and pigmented purpuric dermatosis, respectively. Four cases developed purpura prior to the glandular symptoms of dry mouth and eyes, and 11 cases had purpura after presentations of glandular symptoms. Com-paring with the non-purpura group, the pSS patients with purpura group had earlier onset age [(41.6±13.5) years vs. (51.7±11.4) years, P=0.003], higher incidence of lymphoma (2/15 vs. 0, P=0.011); higher serum levels of rheumatoid factor (RF) (P=0.002), erythrocyte sedimentation rate (ESR) (P=0.019), immunoglobulin (Ig) G (P<0.001), γ-globulin (P=0.001), free Ig light (κ) chains (P<0.001), free Ig heavy (λ) chains (P=0.015), and lower levels of compliment components C3 (P=0.011) and C4 (P=0.021). Conclusions: As a common and heterogeneous dermatological manifestation of pSS patients revealed in this study, the diagnosis of pSS should be considered in patients presenting purpura. The pSS patients with purpura with early onset and abnormal laboratory parameters have increased risk of lymphoma and demanded further monitoring. |
first_indexed | 2024-04-11T04:34:03Z |
format | Article |
id | doaj.art-d7103df5fce748b6bdef16939bf0b122 |
institution | Directory Open Access Journal |
issn | 1671-2870 |
language | zho |
last_indexed | 2024-04-11T04:34:03Z |
publishDate | 2021-04-01 |
publisher | Editorial Office of Journal of Diagnostics Concepts & Practice |
record_format | Article |
series | Zhenduanxue lilun yu shijian |
spelling | doaj.art-d7103df5fce748b6bdef16939bf0b1222022-12-29T00:57:44ZzhoEditorial Office of Journal of Diagnostics Concepts & PracticeZhenduanxue lilun yu shijian1671-28702021-04-01200215516010.16150/j.1671-2870.2021.02.007Clinical features of primary Sjögren syndrome with purpuraZHAO Qian, ZHAO Xiaoqing, DIAO Licheng, SUN Fei, ZHENG Jie, ZHU Xuemei, CAO Hua0a. Department of Dermatology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China;b. Department of Pulmonary and Critical Care Medicine, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, ChinaObjective: To study the clinical characteristics of patients with primary Sjögren syndrome(pSS) with purpura. Methods: A total of 101 patients with pSS were enrolled from January 2017 through January 2020 in Department of Dermatology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine. The patients were divided into 2 groups: pSS with purpura (purpura group, n=15) and without purpura (non-purpura group, n=86). The clinical presentations and laboratory data were compared between the 2 groups. Results: Of the 15 patients with manifestation of purpura (14.9%), 12(80%) were diagnosed as hypergammaglobulinemia purpura and the other 3(20%) were cryoglobulinemia purpura, thrombocytopenic purpura, and pigmented purpuric dermatosis, respectively. Four cases developed purpura prior to the glandular symptoms of dry mouth and eyes, and 11 cases had purpura after presentations of glandular symptoms. Com-paring with the non-purpura group, the pSS patients with purpura group had earlier onset age [(41.6±13.5) years vs. (51.7±11.4) years, P=0.003], higher incidence of lymphoma (2/15 vs. 0, P=0.011); higher serum levels of rheumatoid factor (RF) (P=0.002), erythrocyte sedimentation rate (ESR) (P=0.019), immunoglobulin (Ig) G (P<0.001), γ-globulin (P=0.001), free Ig light (κ) chains (P<0.001), free Ig heavy (λ) chains (P=0.015), and lower levels of compliment components C3 (P=0.011) and C4 (P=0.021). Conclusions: As a common and heterogeneous dermatological manifestation of pSS patients revealed in this study, the diagnosis of pSS should be considered in patients presenting purpura. The pSS patients with purpura with early onset and abnormal laboratory parameters have increased risk of lymphoma and demanded further monitoring.http://www.qk.sjtu.edu.cn/jdcp/fileup/1671-2870/PDF/1671-2870-2021-20-2-155.pdf|primary sjögren syndrome|cutaneous manifestation|purpura|lymphoma |
spellingShingle | ZHAO Qian, ZHAO Xiaoqing, DIAO Licheng, SUN Fei, ZHENG Jie, ZHU Xuemei, CAO Hua Clinical features of primary Sjögren syndrome with purpura Zhenduanxue lilun yu shijian |primary sjögren syndrome|cutaneous manifestation|purpura|lymphoma |
title | Clinical features of primary Sjögren syndrome with purpura |
title_full | Clinical features of primary Sjögren syndrome with purpura |
title_fullStr | Clinical features of primary Sjögren syndrome with purpura |
title_full_unstemmed | Clinical features of primary Sjögren syndrome with purpura |
title_short | Clinical features of primary Sjögren syndrome with purpura |
title_sort | clinical features of primary sj x000f6 gren syndrome with purpura |
topic | |primary sjögren syndrome|cutaneous manifestation|purpura|lymphoma |
url | http://www.qk.sjtu.edu.cn/jdcp/fileup/1671-2870/PDF/1671-2870-2021-20-2-155.pdf |
work_keys_str_mv | AT zhaoqianzhaoxiaoqingdiaolichengsunfeizhengjiezhuxuemeicaohua clinicalfeaturesofprimarysjx000f6grensyndromewithpurpura |