Relation of Autoimmune Cytopenia to Glandular and Systemic Manifestations in Primary Sjögren Syndrome: Analysis of 113 Korean Patients

Koh, Jung Hee; Lee, Jennifer; Chung, So‐Hyang; Kwok, Seung‐Ki; Park, Sung-Hwan

doi:10.3899/jrheum.150058

Cited by 8 publications

(6 citation statements)

References 22 publications

(16 reference statements)

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“…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)(24)(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: 74%

“…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%

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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: 74%

Section: Discussionmentioning

confidence: 88%

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

et al. 2023

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“…According to previous studies, patients with Sjögren's syndrome are more susceptible to hematological abnormalities than general population. [10][11][12] these hematological abnormalities are known as autoimmune cytopenia, possibly related with the degree of extra-glandular or glandular organs involvement. [10][11][12] in this vulnerable population, certain antipsychotics use, especially risperidone, may further elevate the risk of leukopenia.…”

Section: Discussionmentioning

confidence: 99%

Risperidone-Induced Leukopenia in a Patient with Epilepsy and Sjögren’s Synd- rome: A Case Report

2019

Journal of Case Reports: Open Access

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A-39-year-old Taiwanese man with epilepsy for 30 years and Sjögren's syndrome for 3 years was admitted for newly diagnosed psychosis. He developed leucopenia after treatment with risperidone. After risperidone was discontinued, the white blood cell (WBC) count returned to reference values. For improved symptom control, he was rechallenged with a lower dose of risperidone under close monitoring, but the leukopenia redeveloped. After a shift to paliperidone use, the WBC count gradually normalized. When prescribing risperidone, physicians should be aware of its effects on leukopenia.

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“…The cytopenias are the main cellular abnormalities, while hyper- and hypogammaglobulinemia, monoclonal gammopathy, cryoglobulinemia, and the presence of autoantibodies are the most frequent humoral manifestations [ 175 ]. Leukopenia was reported in 19% to 22% of pSS patients [ 176 ], while neutropenia and lymphopenia were noted in 14 to 27.3% [ 177 ] and in 14 to 23.9% of the pSS patients, respectively [ 116 , 178 , 179 ]. Secondary to chronic inflammation, anemia may occur in between 17.1 and 23.9% of patients [ 180 ], while thrombocytopenia was reported between 29.3 to 30.5% of pSS patients [ 181 , 182 ].…”

Section: Extraglandular Manifestationsmentioning

confidence: 99%

The Spectrum of Extraglandular Manifestations in Primary Sjögren’s Syndrome

Mihai

Căruntu

Jurcuţ

et al. 2023

JPM

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Extraglandular manifestations (EGMs) in primary Sjogren’s syndrome (pSS) represent the clinical expression of the systemic involvement in this disease. EGMs are characterized by a wide heterogeneity; virtually any organ or system can be affected, with various degrees of dysfunction. The existing gaps of knowledge in this complex domain of extraglandular extension in pSS need to be overcome in order to increase the diagnostic accuracy of EGMs in pSS. The timely identification of EGMs, as early as from subclinical stages, can be facilitated using highly specific biomarkers, thus preventing decompensated disease and severe complications. To date, there is no general consensus on the diagnostic criteria for the wide range of extraglandular involvement in pSS, which associates important underdiagnosing of EGMs, subsequent undertreatment and progression to severe organ dysfunction in these patients. This review article presents the most recent basic and clinical science research conducted to investigate pathogenic mechanisms leading to EGMs in pSS patients. In addition, it presents the current diagnostic and treatment recommendations and the trends for future therapeutic strategies based on personalized treatment, as well as the latest research in the field of diagnostic and prognostic biomarkers for extraglandular involvement in pSS.

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Relation of Autoimmune Cytopenia to Glandular and Systemic Manifestations in Primary Sjögren Syndrome: Analysis of 113 Korean Patients

Abstract: Autoimmune cytopenia is closely associated with severe ocular surface damage in pSS. Therefore, assessment of xerophthalmia by ophthalmologists may be mandatory, particularly in patients with pSS with cytopenia, even if patients do not complain of eye dryness.

Cited by 8 publications

References 22 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

Risperidone-Induced Leukopenia in a Patient with Epilepsy and Sjögren’s Synd- rome: A Case Report

The Spectrum of Extraglandular Manifestations in Primary Sjögren’s Syndrome

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