Shuo Cheng, Suo Zhang, Jiaming Huang, Xue Guo, Meiying Wang
{"title":"Treatment of Lupus Retinopathy With the Combination of Telitacicept and Methylprednisolone: A Case Report","authors":"Shuo Cheng, Suo Zhang, Jiaming Huang, Xue Guo, Meiying Wang","doi":"10.1111/1756-185x.70509","DOIUrl":"https://doi.org/10.1111/1756-185x.70509","url":null,"abstract":"","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"28 12","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145739722","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yufeng Pan, Zhiyuan Qiu, Junjun Wang, Di Jin, Jingjing Ma
<p>Primary immune thrombocytopenia (ITP) is an acquired autoimmune disease (AID) marked by isolated thrombocytopenia. Its main clinical manifestation is an increased risk of skin and mucosal bleeding associated with platelet levels. The pathogenic mechanism is primarily attributed to immune abnormalities mediated by antiplatelet antibodies and disturbances in lymphocyte subsets. The antinuclear antibody (ANA) is a key serological marker for the diagnosis and evaluation of AID. There is a trend toward higher rates of ANA positivity in ITP patients. Humoral, cellular immunity, and epigenetic regulation constitute the unique immunophenotypic characteristics of ANA-positive ITP. In these patients, serum complement C3 levels are significantly reduced, and antiplatelet antibodies co-deposited with complement C1q to form immune complexes, leading to platelet damage through complement-dependent cytotoxicity mediated by autoantibodies [<span>1</span>]. The proportion of granular megakaryocytes is significantly higher in the ANA-positive group compared to the negative group. Overexpression of genes related to the TGF-β/Smad pathway and hypermethylation in the promoter region of the TET2 gene promote fibrosis of the bone marrow microenvironment, resulting in impaired platelet production [<span>2</span>]. ANA-positive ITP exhibits a more complex immunological profile than typical ITP, with a higher risk of progression to AID, more pronounced immune dysregulation, more severe clinical manifestations, and a poorer prognosis. This observation led us to investigate whether ANA-positive ITP constitutes a distinct clinical entity and warrants classification within the spectrum of rheumatic disorders.</p><p>Current studies indicate that 17.5% to 33.3% of ITP patients are already ANA-positive. Women of childbearing age (20–40 years) constitute a high-risk group for developing ANA-positive ITP in adults [<span>3</span>]. This characteristic is closely associated with immune microenvironment dysregulation, sex hormone levels (estrogen and progesterone), and genetic polymorphisms. The latest study involving 360 patients with primary ITP showed that the incidence of AID (81.2% vs. 52.5%) and the risk of progression (a 16.8-fold increase) we significantly higher in the ANA-positive group than in the ANA-negative group. ANA positivity, anti-SSB antibodies, and high C3 levels were identified as independent predictors of disease progression in the ANA-positive group [<span>4</span>]. Furthermore, the 10-year CTD conversion risk exceeds 40% in individuals with both high-titer ANA and low complement levels. The treatment effect and prognosis of ANA-positive ITP patients were negatively correlated with titer values, and the risk of thrombotic events and bleeding was significantly higher than in the negative group.</p><p>The 2019 International ITP Working Group guidelines suggest that ANA may be considered as an early warning indicator of ITP translational risk, but ANA is stil
{"title":"ANA-Positive Primary Immune Thrombocytopenia: An Independent Clinical Entity—Potential Connective Tissue Diseases?","authors":"Yufeng Pan, Zhiyuan Qiu, Junjun Wang, Di Jin, Jingjing Ma","doi":"10.1111/1756-185x.70512","DOIUrl":"https://doi.org/10.1111/1756-185x.70512","url":null,"abstract":"<p>Primary immune thrombocytopenia (ITP) is an acquired autoimmune disease (AID) marked by isolated thrombocytopenia. Its main clinical manifestation is an increased risk of skin and mucosal bleeding associated with platelet levels. The pathogenic mechanism is primarily attributed to immune abnormalities mediated by antiplatelet antibodies and disturbances in lymphocyte subsets. The antinuclear antibody (ANA) is a key serological marker for the diagnosis and evaluation of AID. There is a trend toward higher rates of ANA positivity in ITP patients. Humoral, cellular immunity, and epigenetic regulation constitute the unique immunophenotypic characteristics of ANA-positive ITP. In these patients, serum complement C3 levels are significantly reduced, and antiplatelet antibodies co-deposited with complement C1q to form immune complexes, leading to platelet damage through complement-dependent cytotoxicity mediated by autoantibodies [<span>1</span>]. The proportion of granular megakaryocytes is significantly higher in the ANA-positive group compared to the negative group. Overexpression of genes related to the TGF-β/Smad pathway and hypermethylation in the promoter region of the TET2 gene promote fibrosis of the bone marrow microenvironment, resulting in impaired platelet production [<span>2</span>]. ANA-positive ITP exhibits a more complex immunological profile than typical ITP, with a higher risk of progression to AID, more pronounced immune dysregulation, more severe clinical manifestations, and a poorer prognosis. This observation led us to investigate whether ANA-positive ITP constitutes a distinct clinical entity and warrants classification within the spectrum of rheumatic disorders.</p><p>Current studies indicate that 17.5% to 33.3% of ITP patients are already ANA-positive. Women of childbearing age (20–40 years) constitute a high-risk group for developing ANA-positive ITP in adults [<span>3</span>]. This characteristic is closely associated with immune microenvironment dysregulation, sex hormone levels (estrogen and progesterone), and genetic polymorphisms. The latest study involving 360 patients with primary ITP showed that the incidence of AID (81.2% vs. 52.5%) and the risk of progression (a 16.8-fold increase) we significantly higher in the ANA-positive group than in the ANA-negative group. ANA positivity, anti-SSB antibodies, and high C3 levels were identified as independent predictors of disease progression in the ANA-positive group [<span>4</span>]. Furthermore, the 10-year CTD conversion risk exceeds 40% in individuals with both high-titer ANA and low complement levels. The treatment effect and prognosis of ANA-positive ITP patients were negatively correlated with titer values, and the risk of thrombotic events and bleeding was significantly higher than in the negative group.</p><p>The 2019 International ITP Working Group guidelines suggest that ANA may be considered as an early warning indicator of ITP translational risk, but ANA is stil","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"28 12","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1756-185x.70512","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145739723","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}