Clinical manifestations in anti-Ro52/SS-A antibody-seropositive patients with Sjögren's syndrome

Nakamura, Hideki; Morimoto, Shimpei; Shimizu, Toshimasa; Takatani, Ayuko; Nishihata, Shinya; Kawakami, Atsushi

doi:10.1080/25785826.2021.1919342

Cited by 5 publications

(6 citation statements)

References 41 publications

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“…This may be related to the limited number of patients with C-pSS and the relatively low probability of organ involvement in our study. Third, a close association between immunological markers and other laboratory indications identified in our study has also been reported in previous studies (32,34,38). We confirmed that IgG levels increased significantly in patients with positive anti-SSA/Ro52 or anti-SSA/Ro60.…”

Section: Discussionsupporting

confidence: 92%

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: 92%

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

et al. 2023

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“…Our markedly higher ESSDAI in our cohort may be explained by the manifestations of cryoglobulinaemia including cutaneous vasculitis and Raynaud’s phenomena ( Table 2 ). The incidence of cryoglobulinaemia was not explored in these earlier studies ( 4 , 14 ); however, the isolated anti-Ro52 subset positively correlated with RhF ( 4 , 12 , 14 ), consistent with our findings ( Table 2 ). Moreover, a cross-sectional study in a laboratory cohort of SS patients that measured cryoglobulins in serological subsets, found that isolated anti-Ro52 antibodies were associated with cryoglobulinaemia ( 32 ).…”

Section: Discussionsupporting

confidence: 90%

“…In SS, anti-Ro52 positivity may signify a severe subset of patients associated with rheumatoid factor (RhF) positivity ( 12 , 13 ). However, the value of stratifying SS patients by patterns of serum autoantibodies (anti-Ro52, anti-Ro60 and anti-La) has not been widely appreciated and inconsistencies remain in the literature.…”

Section: Introductionmentioning

confidence: 99%

Isolated anti-Ro52 identifies a severe subset of Sjögren’s syndrome patients

et al. 2023

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IntroductionSerum autoantibodies targeting the SSA/Ro proteins are a key component of the classification criteria for the diagnosis of Sjögren’s syndrome (SS). Most patients' serum reacts with both Ro60 and Ro52 proteins. Here we compare the molecular and clinical characteristics of patients diagnosed with SS with anti-Ro52 in the presence or absence of anti-Ro60/La autoantibodies.MethodsA cross-sectional study was performed. Patients in the SS biobank at Westmead Hospital (Sydney, Australia) that were positive for anti-Ro52 were included and stratified based on the absence (isolated) or presence (combined) of anti-Ro60/La, measured by line immunoassay. We examined clinical associations and the serological and molecular characteristics of anti-Ro52 using ELISA and mass spectrometry in serological groups.ResultsA total of 123 SS patients were included for study. SS patients with isolated anti-Ro52 (12%) identified a severe serological subset characterised by higher disease activity, vasculitis, pulmonary involvement, rheumatoid factor (RhF) and cryoglobulinaemia. Serum antibodies reacting with Ro52 in the isolated anti-Ro52 subset displayed less isotype switching, less immunoglobulin variable region subfamily usage and a lower degree of somatic hypermutation than the combined anti-Ro52 subset.ConclusionsIn our cohort of SS patients, isolated anti-Ro52 represents a severe subset of SS, and is associated with the presence of cryoglobulinaemia. We therefore provide clinical relevance to the stratification of SS patients by their sero-reactivities. It is possible that the autoantibody patterns may be immunological epiphenomena of the underlying disease process, and further work is required to unearth the mechanisms of the differential clinical phenotypes.

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“…To determine the immunosuppressive function of SoJIA-driven PBCECs, we investigated the monocyte responsiveness to lipopolysaccharide (LPS) in the presence or absence of PBCECs obtained from patients with SoJIA. We focused on the effect of CECs on monocytes but not lymphocytes, as SoJIA is an autoinflammatory disease involving excessive hypercytokinemia triggered by the activation of the innate immune system 35 , 36 . PBCECs were procured in combination with magnetic microbeads and fluorescent cell sorting from patient’s PBMCs to obtain a > 96% purity population (Fig.…”

Section: Resultsmentioning

confidence: 99%

The immunoregulatory function of peripheral blood CD71+ erythroid cells in systemic-onset juvenile idiopathic arthritis

Kanemasa

Ishimura

Eguchi

et al. 2021

Sci Rep

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CD71+ erythroid cells (CECs) are recognized to have an immunoregulatory function via direct cell–cell interaction and soluble mediators. Circulating CECs appear in newborns or patients with hemolytic and cardiopulmonary disorders. To assess the biological role of CECs in systemic inflammation, we studied the gene expression and function in systemic-onset juvenile idiopathic arthritis (SoJIA). Peripheral blood mononuclear cells of SoJIA patients expressed upregulated erythropoiesis-related genes. It represented the largest expansion of CECs during active phase SoJIA among other inflammatory diseases. Despite the opposing roles of erythropoietin and hepcidin in erythropoiesis, both serum levels were in concert with the amounts of SoJIA-driven CECs. Circulating CECs counts in inflammatory diseases were positively correlated with the levels of C-reactive protein, IL-6, IL-18, or soluble TNF receptors. Co-culture with active SoJIA-driven CECs suppressed secretions of IL-1β, IL-6, and IL-8 from healthy donor monocytes. The top upregulated gene in SoJIA-driven CECs was ARG2 compared with CECs from cord blood controls, although cytokine production from monocytes was suppressed by co-culture, even with an arginase inhibitor. CECs are driven to the periphery during the acute phase of SoJIA at higher levels than other inflammatory diseases. Circulating CECs may control excessive inflammation via the immunoregulatory pathways, partly involving arginase-2.

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Clinical manifestations in anti-Ro52/SS-A antibody-seropositive patients with Sjögren's syndrome

Cited by 5 publications

References 41 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

Isolated anti-Ro52 identifies a severe subset of Sjögren’s syndrome patients

The immunoregulatory function of peripheral blood CD71+ erythroid cells in systemic-onset juvenile idiopathic arthritis

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