Factors associated with secondary immune thrombocytopenia in patients with primary Sjögren's syndrome: a retrospective study of 639 cases

Luo, Jing; Song, Weijiang; Chen, Jiaqi; Yang, Guoyan; Yang, Jianying; Yu, Xin-bo; Huang, Ziwei; Xu, Yifeng; Wang, Jianming; Tao, Qing-Wen

doi:10.55563/clinexprheumatol/8hgmjm

Cited by 5 publications

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“…Our results also suggested that low C4 levels may seriously influence the development of thrombocytopenia in patients with C-pSS. Finally, we observed that SSB and RF were negatively associated with thrombocytopenia in pSS, which is consistent with the conclusions of previous studies ( 23 , 25 , 26 ). However, more research is needed to verify these findings and explore the underlying mechanisms.…”

Section: Discussionsupporting

confidence: 93%

“…We observed that thrombocytopenia occurred in 22 (44.9%) patients, higher than 12.03%–23.9% reported by a few studies based on adult pSS patients ( 23 – 25 ), but similar to the 48.9% reported by Wu et al ( 26 ). However, published pediatric cohort studies focusing on thrombocytopenia secondary to pSS are lacking at present.…”

Section: Discussionsupporting

confidence: 82%

“…This suggests that pSS patients with thrombocytopenia may be less likely to experience exocrine gland involvement. Reportedly, ILD is negatively related to thrombocytopenia in pSS ( 23 , 24 , 26 ); however, due to the limited number of patients with ILD in our study, we analyzed the comparison of organ involvement, except that of hematology, and found that patients with thrombocytopenia were less likely to experience other organ involvement. Moreover, autoantibodies inducing complement-mediated destruction of platelets and inhibiting megakaryocyte function are reportedly involved in thrombocytopenia development ( 29 , 30 ).…”

Section: Discussionmentioning

confidence: 88%

“…Moreover, autoantibodies inducing complement-mediated destruction of platelets and inhibiting megakaryocyte function are reportedly involved in thrombocytopenia development (29,30). Several studies have also shown that complement levels were significantly reduced in patients with thrombocytopenia, and a low C4 level could predict more severe thrombocytopenia (23,25,26,31). Our results also suggested that low C4 levels may seriously influence the development of thrombocytopenia in patients with C-pSS.…”

Section: Discussionmentioning

confidence: 99%

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Clinical and laboratory features of childhood-onset primary Sjögren's syndrome: A retrospective study from China

et al. 2023

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IntroductionThe initial presentations of childhood-onset primary Sjögren’s syndrome (C-pSS) vary, making diagnosis challenging. We aimed to improve the diagnosis and evaluation of C-pSS by summarizing its clinical and laboratory features.MethodsA total of 49 patients with C-pSS between July 2015 and August 2022 in the Department of Rheumatology and Immunology of Shanghai Children's Medical Centre were enrolled in this study. Their clinical manifestations and laboratory examinations of these patients were compared based on the presence or absence of thrombocytopenia and parotitis and whether the immunological markers, including anti-nuclear antibodies (ANA), rheumatoid factor (RF), anti-Ro52/SSA antibodies (anti-SSA/Ro52), anti-Ro60/SSA antibodies (anti-SSA/Ro60), and anti-Ro/SSB antibodies (anti-SSB), were positive.ResultsThe mean age at C-pSS diagnosis was 10.34 ± 3.45 years, and the ratio of boys to girls was 1:6. In the thrombocytopenia group, parotitis (P = 0.044), organ involvement except for hematology (P = 0.002), positive anti-SSB (P = 0.004), and positive RF (P = 0.001) were less frequently observed. Complement C4 (P = 0.038) and white blood cells (P = 0.002) levels decreased and increased significantly, respectively. Anti-SSB (P = 0.010) and RF (P = 0.004) positivity were independent potential protective factors against thrombocytopenia in patients with C-pSS. In the parotitis group, higher ANA titers (P = 0.027), higher focus scores on labial gland biopsy (P = 0.024), and positive RF (P = 0.001), anti-SSA/Ro60 (P = 0.003), and anti-SSB (P = 0.001) were observed more frequently. Furthermore, positive anti-SSB (P = 0.012) and positive RF (P = 0.028) were independent risk factors for parotitis in patients with C-pSS. The hemoglobin level was significantly lower in patients with positive anti-SSA/Ro52 and positive anti-SSA/Ro60 results (P = 0.022 and P = 0.029, respectively), while immunoglobulin G level was significantly higher in patients in the same group (P = 0.048 and P = 0.007, respectively).ConclusionsPositive anti-SSB and positive RF values may be independent potential protective factors of thrombocytopenia in patients with C-pSS. In contrast, positive anti-SSB and positive RF were independent risk factors of parotitis in patients with C-pSS. More studies are needed to reveal the diagnostic role and pathogenic mechanism of immunological markers in C-pSS.

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Section: Discussionsupporting

confidence: 93%

Section: Discussionsupporting

confidence: 82%

Section: Discussionmentioning

confidence: 88%

Section: Discussionmentioning

confidence: 99%

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Clinical and laboratory features of childhood-onset primary Sjögren's syndrome: A retrospective study from China

et al. 2023

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“…Hematological parameters, such as platelet count and mean platelet volume, along with autoantibody profiles, provide essential insights into the disease state. [ 7 , 8 ] However, a comprehensive analysis encompassing the full spectrum of clinical and hematological parameters in pSS patients with secondary ITP is still lacking.…”

Section: Introductionmentioning

confidence: 99%

Comprehensive analysis of the clinical manifestations and hematological parameters associated with secondary immune thrombocytopenia in patients with primary Sjögren syndrome: An observational study

Yang

2024

Medicine

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Primary Sjögren Syndrome (pSS) is a chronic autoimmune disease that primarily affects exocrine glands and can lead to various extraglandular manifestations, including secondary immune thrombocytopenia (ITP). Understanding the clinical and hematological differences in pSS patients with and without secondary ITP is crucial for improved patient management and treatment strategies. This retrospective study, conducted from January 2020 to December 2023, involved a cohort of pSS patients, dividing them into 2 groups: those with secondary ITP and those without. Patients were evaluated using the European League Against Rheumatism Sjögren Syndrome Disease Activity Index (ESSDAI), EULAR Sjögren Syndrome Patient-Reported Index (ESSPRI), Health Assessment Questionnaire, and other hematological parameters. Inclusion criteria were based on the American-European Consensus Group or ACR/EULAR classification criteria for pSS. Exclusion criteria included other autoimmune or hematological disorders, prior splenectomy, recent blood transfusions, and lack of informed consent. Statistical analysis was performed using SPSS software, with various tests applied to analyze the data, including logistic regression to identify risk factors for secondary ITP. Significant differences were noted in fatigue, lymphadenopathy, arthritis, mean age, and ESSDAI scores between the secondary ITP and non-secondary ITP groups. Patients with secondary ITP exhibited higher platelet counts, more prevalent lymphopenia, higher immunoglobulin G (IgG) levels, lower complement 3 levels, and reduced white blood cell and hemoglobin levels. Logistic regression analysis identified lymphadenopathy as a risk factor and arthritis as a protective factor for the development of secondary ITP. The study reveals distinct clinical and hematological characteristics in pSS patients with secondary ITP, suggesting a higher disease activity in this subset. These findings underscore the need for further exploration of these associations to develop more precise treatment approaches for pSS, focusing on preventing secondary ITP and improving patient outcomes.

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Anti-SSA/SSB-negative primary Sjögren’s syndrome showing different clinical phenotypes: a retrospective study of 934 cases

et al. 2023

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Background Currently, only a few studies have described the general characteristics of patients with primary Sjögren’s syndrome (pSS) who tested negatives for anti-SSA and anti-SSB antibodies. We aimed to further investigate the clinical characteristics of these patients in a large sample. Methods Data from patients with pSS who were treated at a tertiary hospital in China between 2013 and 2022 were retrospectively analyzed. Clinical characteristics of the patients were compared between those with and without anti-SSA and anti-SSB antibody negativity. Factors associated with anti-SSA and anti-SSB negativity were identified by logistic regression analysis. Results Overall, 934 patients with pSS were included in this study, among whom 299 (32.0%) tested negative for anti-SSA and anti-SSB antibodies. Compared with patients testing positive for anti-SSA or anti-SSB antibodies, that testing negative for the two antibodies had a lower proportion of females (75.3% vs. 90.6%, p < 0.001) and thrombocytopenia (6.7% vs. 13.6%, p = 0.002), but a higher proportion of abnormal Schirmer I tests (96.0% vs. 89.1%, p = 0.001) and interstitial lung disease (ILD) (59.2% vs. 28.8%, p = 0.001). Anti-SSA and anti-SSB negativity was positively associated with male sex (odds ratio [OR] = 1.86, 95% confidence interval [CI]: 1.05, 3.31), abnormal Schirmer I tests (OR = 2.85, 95% CI: 1.24, 6.53), and ILD (OR = 2.54, 95% CI: 1.67, 3.85). However, it was negatively related to thrombocytopenia (OR = 0.47, 95% CI: 0.24, 0.95). Conclusion Approximately one third of pSS patients had anti-SSA and anti-SSB negativity. pSS patients testing negative for anti-SSA and anti-SSB showed a higher risk of abnormal Schirmer I tests and ILD, but a lower risk of thrombocytopenia.

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Factors associated with secondary immune thrombocytopenia in patients with primary Sjögren's syndrome: a retrospective study of 639 cases

Cited by 5 publications

References 0 publications

Clinical and laboratory features of childhood-onset primary Sjögren's syndrome: A retrospective study from China

Clinical and laboratory features of childhood-onset primary Sjögren's syndrome: A retrospective study from China

Comprehensive analysis of the clinical manifestations and hematological parameters associated with secondary immune thrombocytopenia in patients with primary Sjögren syndrome: An observational study

Anti-SSA/SSB-negative primary Sjögren’s syndrome showing different clinical phenotypes: a retrospective study of 934 cases

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