Clinical and laboratory features of primary Sjögren’s syndrome complicated with mild to severe thrombocytopenia

Wu, Jian; Chang, Xin; Zhang, Jian; Liu, Cuiping; Liu, Mingxing; Chen, Weichang

doi:10.21037/atm-22-162

Cited by 5 publications

(11 citation statements)

References 26 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: 84%

“…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: 84%

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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“…Previous studies in pSS patients with severe ITP focused on the characteristics of patients rather than predictors of treatment response. More importantly, these studies did not consider the condition of bone marrow in pSS patients with severe ITP 3,4 …”

Section: Introductionmentioning

confidence: 99%

Predictive value of bone marrow megakaryocyte count for immunotherapeutic response in primary Sjögren's syndrome patients with severe immune thrombocytopenia: A single‐center case–control study in China

et al. 2023

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Objectives: To investigate potential predictors of treatment response in primary Sjögren's syndrome (pSS) patients with severe immune thrombocytopenia (ITP), with a focus on bone marrow megakaryocyte (MK) count. Methods:This case-control study included patients with pSS and severe ITP who were admitted to Peking Union Medical College Hospital and met the 2002 AECG or 2016 American College of Rheumatology / European League Against Rheumatism criteria for SS. Patients who had overlap other connective tissue diseases and with thrombocytopenia that could be explained by other causes were excluded. Severe ITP was defined as platelet count <20 × 10 9 /L. Response was evaluated at 3 months after treatment.Results: Sixty-eight eligible patients were included: 34 (50%) achieved complete remission (CR), 18 (26%) partial remission (PR) and 16 (24%) were non-responders (NRs).Fewer infections were found in the CR group (24%) than in the PR (50%) and NR (56%) groups (P = 0.04). The MK count (CR 32 vs PR 36 vs NR 4 per slide, P < 0.001) in the NR group was significantly lower than in the other groups. MK count >6.5 per slide predicted good treatment response, with 85.7% sensitivity, 88.1% specificity and 0.866 area under the curve. Logistic regression indicated that patients with more MKs were more likely to respond to immunotherapy (crude odds ratio [OR] 1.45, 95% CI 1.2-2.0, adjusted OR 1.68, 95% CI 1.2-2.7).Conclusions: MK count predicted response to immunosuppressive treatment in pSS patients with severe ITP. These patients are recommended to have bone marrow aspiration before treatment initiation. Clinicians should be aware of screening for infections during clinical practice.

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Characteristics and treatment responses of immune thrombocytopenia in patients with primary Sjögren syndrome

Sun¹,

Qi²,

Wei³

et al. 2023

International Immunopharmacology

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Clinical and laboratory features of primary Sjögren’s syndrome complicated with mild to severe thrombocytopenia

Cited by 5 publications

References 26 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

Predictive value of bone marrow megakaryocyte count for immunotherapeutic response in primary Sjögren's syndrome patients with severe immune thrombocytopenia: A single‐center case–control study in China

Characteristics and treatment responses of immune thrombocytopenia in patients with primary Sjögren syndrome

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