Yang Liu, Jie Kang, Yazhen Su, Xiuying Fan, Dan Ma, Zewen Wu, Xueyan Gong, Junkang Zhao, Liyun Zhang
Background
Primary Sjögren's syndrome (pSS) is an autoimmune disease characterized by the destruction of exocrine glands primarily via T-cell-mediated B-cell over-activation and cytokine production. This leads to pronounced dryness of the mouth and eyes and can result in multi-systemic involvement affecting the kidneys, lungs, and blood. In recent years, there has been increasing attention on the role of immune cells in pSS. However, studies investigating the causal role of immune cells in pSS have been relatively limited.
Methods
In this study, we employed a two-way two-sample Mendelian randomization approach to assess the causal relationship between immune cells and pSS. Utilizing publicly available genome-wide association study (GWAS) data, we explored the causal links between 731 immunophenotypically labeled immune cells and the risk of pSS.
Results
Through the use of instrumental variables derived from GWAS data and corrected for false discovery rate (FDR), we identified three immune cells with increased levels that were causally associated with pSS risk (FDR < 0.05). These included IgD+ CD38br AC B cells, CD27 on IgD+ CD38− unswitched memory B cells, and Granulocyte % leukocyte. Additionally, three immune cells with reduced levels were found to be causally associated with pSS risk, namely CD4+ CD8dim %lymphocyte, CD4+ CD8dim %leukocyte, and CD28 on activated and secreting regulatory T cells (Tregs). Furthermore, the development of pSS was associated with elevated levels of CD33br HLA DR+ CD14− % CD33br HLA DR+ in myeloid cells.
Conclusion
This study demonstrates that immune responses influence the progression of pSS in a complex pattern. Our findings may provide new insights into the immunology of pSS pathogenesis and more experimental studies should be conducted to further explore the potential mechanisms between identified immune features and pSS risk, which may provide a basis for exploring early intervention methods for pSS and developing targeted therapeutic strategies or even reshaping immune homeostasis.
{"title":"The causal role of immune cells in primary Sjögren's syndrome: A two-sample Mendelian randomization","authors":"Yang Liu, Jie Kang, Yazhen Su, Xiuying Fan, Dan Ma, Zewen Wu, Xueyan Gong, Junkang Zhao, Liyun Zhang","doi":"10.1111/1756-185X.15350","DOIUrl":"https://doi.org/10.1111/1756-185X.15350","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Primary Sjögren's syndrome (pSS) is an autoimmune disease characterized by the destruction of exocrine glands primarily via T-cell-mediated B-cell over-activation and cytokine production. This leads to pronounced dryness of the mouth and eyes and can result in multi-systemic involvement affecting the kidneys, lungs, and blood. In recent years, there has been increasing attention on the role of immune cells in pSS. However, studies investigating the causal role of immune cells in pSS have been relatively limited.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In this study, we employed a two-way two-sample Mendelian randomization approach to assess the causal relationship between immune cells and pSS. Utilizing publicly available genome-wide association study (GWAS) data, we explored the causal links between 731 immunophenotypically labeled immune cells and the risk of pSS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Through the use of instrumental variables derived from GWAS data and corrected for false discovery rate (FDR), we identified three immune cells with increased levels that were causally associated with pSS risk (FDR < 0.05). These included IgD+ CD38br AC B cells, CD27 on IgD+ CD38− unswitched memory B cells, and Granulocyte % leukocyte. Additionally, three immune cells with reduced levels were found to be causally associated with pSS risk, namely CD4+ CD8dim %lymphocyte, CD4+ CD8dim %leukocyte, and CD28 on activated and secreting regulatory T cells (Tregs). Furthermore, the development of pSS was associated with elevated levels of CD33br HLA DR+ CD14− % CD33br HLA DR+ in myeloid cells.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This study demonstrates that immune responses influence the progression of pSS in a complex pattern. Our findings may provide new insights into the immunology of pSS pathogenesis and more experimental studies should be conducted to further explore the potential mechanisms between identified immune features and pSS risk, which may provide a basis for exploring early intervention methods for pSS and developing targeted therapeutic strategies or even reshaping immune homeostasis.</p>\u0000 </section>\u0000 </div>","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"27 11","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142666049","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}
Ludmila V. Bregel, Alla E. Matunova, Qing Zhou, Mikhail M. Kostik
{"title":"Case Report of Unusual Manifestation of Mevalonate Kinase Deficiency Syndrome Mimicking Juvenile Idiopathic Arthritis With Systemic Onset","authors":"Ludmila V. Bregel, Alla E. Matunova, Qing Zhou, Mikhail M. Kostik","doi":"10.1111/1756-185X.15416","DOIUrl":"10.1111/1756-185X.15416","url":null,"abstract":"","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"27 11","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142647616","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}
<p>Psoriatic arthritis (PsA) is a heterogeneous autoimmune, inflammatory disease, with inflammatory skin, articular and extra-musculoskeletal involvement, [<span>1, 2</span>]. PsA occurs in about one-third of psoriasis patients [<span>3</span>]. Dysregulation of several molecules, including proinflammatory interleukins (ILs), interferons (IFNs), growth factors, and colony-stimulating factors (CSF) act as ligands for receptors connected to intracytoplasmic Janus kinases (JAKs). Consequently, JAKs activate signal transducer and activator of transcription (STAT) proteins, which translocate to the nucleus and induce the expression of inflammatory nuclear factor. The JAK/STAT pathways play a central role in the development and pathogenesis of PsA [<span>4, 5</span>]. Several studies have reported increased activation of JAK1/STAT3/STAT1, which may contribute to the articular inflammatory process characterized by the expansion of Th17 effector cells in the synovial fluid of patients with active PsA [<span>6, 7</span>]. By targeting these JAK/STAT pathways, JAK inhibitors effectively reduce inflammatory responses [<span>7-9</span>].</p><p>JAK inhibitors, including tofacitinib and upadacitinib, effectively target these pathways to reduce inflammation and improve symptoms in PsA patients. However, emerging evidence has raised concerns regarding the cardiovascular (CV) risks associated with JAK inhibitors. Both clinical trials and post-marketing surveillance have reported higher incidences of thromboembolism and other CV events in patients using these therapies, particularly at higher doses. Given the growing use of JAK inhibitors in PsA management, understanding the underlying mechanisms of CV risk is crucial for optimizing patient safety.</p><p>Since the publication of the Oral Surveillance Study [<span>10</span>], the use of JAK inhibitors has raised concerns regarding the potential increased risk of CV disease (CVD) in PsA patients. All known cytokines and chemokines create a unique network of self-interactions, activation and regulatory loops. Theoretically, inhibition of JAK should translate into reduction of both autoimmune diseases' activity and reduction of prothrombotic risk. However, JAK inhibitors are less specific than biologics due to the inherent redundancy of the JAK/STAT pathway and therefore may display an increase in off-target effects, which may be related to the association of increased Major Adverse Cardiovascular Events (MACE) and Venous Thromboembolism (VTE) observed [<span>11</span>].</p><p>The etiology of thrombotic tendency in PsA may also be linked to other mechanisms and causal factors, including antiphospholipid antibodies, hyperhomocysteinemia, and inflammation. JAKs in various combinations bind to cytokine receptors that transmit prothrombotic and proinflammatory signals from a wide range of cytokines. With the exception of IL-10, IFNβ and IFNλ that exert anti-thrombotic potential, signaling downstream of these cytokines cre
{"title":"JAK Inhibitors and Cardiovascular Disease in Psoriatic Arthritis: Friends or Foe?","authors":"Jenny Lin-Hong Shi, Wei-Yuan Chuang, Lai-Shan Tam","doi":"10.1111/1756-185X.15419","DOIUrl":"10.1111/1756-185X.15419","url":null,"abstract":"<p>Psoriatic arthritis (PsA) is a heterogeneous autoimmune, inflammatory disease, with inflammatory skin, articular and extra-musculoskeletal involvement, [<span>1, 2</span>]. PsA occurs in about one-third of psoriasis patients [<span>3</span>]. Dysregulation of several molecules, including proinflammatory interleukins (ILs), interferons (IFNs), growth factors, and colony-stimulating factors (CSF) act as ligands for receptors connected to intracytoplasmic Janus kinases (JAKs). Consequently, JAKs activate signal transducer and activator of transcription (STAT) proteins, which translocate to the nucleus and induce the expression of inflammatory nuclear factor. The JAK/STAT pathways play a central role in the development and pathogenesis of PsA [<span>4, 5</span>]. Several studies have reported increased activation of JAK1/STAT3/STAT1, which may contribute to the articular inflammatory process characterized by the expansion of Th17 effector cells in the synovial fluid of patients with active PsA [<span>6, 7</span>]. By targeting these JAK/STAT pathways, JAK inhibitors effectively reduce inflammatory responses [<span>7-9</span>].</p><p>JAK inhibitors, including tofacitinib and upadacitinib, effectively target these pathways to reduce inflammation and improve symptoms in PsA patients. However, emerging evidence has raised concerns regarding the cardiovascular (CV) risks associated with JAK inhibitors. Both clinical trials and post-marketing surveillance have reported higher incidences of thromboembolism and other CV events in patients using these therapies, particularly at higher doses. Given the growing use of JAK inhibitors in PsA management, understanding the underlying mechanisms of CV risk is crucial for optimizing patient safety.</p><p>Since the publication of the Oral Surveillance Study [<span>10</span>], the use of JAK inhibitors has raised concerns regarding the potential increased risk of CV disease (CVD) in PsA patients. All known cytokines and chemokines create a unique network of self-interactions, activation and regulatory loops. Theoretically, inhibition of JAK should translate into reduction of both autoimmune diseases' activity and reduction of prothrombotic risk. However, JAK inhibitors are less specific than biologics due to the inherent redundancy of the JAK/STAT pathway and therefore may display an increase in off-target effects, which may be related to the association of increased Major Adverse Cardiovascular Events (MACE) and Venous Thromboembolism (VTE) observed [<span>11</span>].</p><p>The etiology of thrombotic tendency in PsA may also be linked to other mechanisms and causal factors, including antiphospholipid antibodies, hyperhomocysteinemia, and inflammation. JAKs in various combinations bind to cytokine receptors that transmit prothrombotic and proinflammatory signals from a wide range of cytokines. With the exception of IL-10, IFNβ and IFNλ that exert anti-thrombotic potential, signaling downstream of these cytokines cre","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"27 11","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1756-185X.15419","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142647747","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}
Jinlin Miao, Bei Zhang, Haoyang Sun, Peiyan Zhang, Haomiao Shen, Jiawei Wang, Junfeng Jia, Kui Zhang, Zhaohui Zheng, Ping Zhu
Aim
Rheumatoid arthritis (RA) is a prevalent autoimmune disease characterized by immune dysegulation, including an immune imbalance due to abnormal activation of non-classical Th1 cells (CD161+ Th1). This study investigated the effects of CCR5 on the activation and proliferation of CD161+ Th1 and their pathogenicity in patients with RA.
Methods
The study was conducted on 53 patients with RA and 32 age- and sex-matched healthy controls (HC). The cell phenotype was assessed by flow cytometry and the cytokine levels in the supernatant were detected by ELISA.
Results
We demonstrate a marked increase in CD161+ Th1 cells in the synovial fluid of RA patients. These cells exhibit a hyperactivated and hyperproliferative state alongside elevated CCR5 expression. Furthermore, the levels of CD161+ Th1 cells, CD25, and CCR5 in RA synovial fluid show a positive correlation with the disease activity. Additionally, our study reveals that CCR5 facilitates the activation, proliferation, and cytokine production of CD161+ Th1 cells through the pZAP70/NFAT signaling pathway.
Conclusion
These findings contribute to a deeper understanding of RA pathogenesis and uncover a novel mechanism that regulates non-classical CD161+ Th1 responses in RA, which may provide a potential therapeutic target.
{"title":"CCR5 mediates rheumatoid arthritis progression by promoting the activation and proliferation of non-classical Th1 cells","authors":"Jinlin Miao, Bei Zhang, Haoyang Sun, Peiyan Zhang, Haomiao Shen, Jiawei Wang, Junfeng Jia, Kui Zhang, Zhaohui Zheng, Ping Zhu","doi":"10.1111/1756-185X.15370","DOIUrl":"https://doi.org/10.1111/1756-185X.15370","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Aim</h3>\u0000 \u0000 <p>Rheumatoid arthritis (RA) is a prevalent autoimmune disease characterized by immune dysegulation, including an immune imbalance due to abnormal activation of non-classical Th1 cells (CD161<sup>+</sup> Th1). This study investigated the effects of CCR5 on the activation and proliferation of CD161<sup>+</sup> Th1 and their pathogenicity in patients with RA.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>The study was conducted on 53 patients with RA and 32 age- and sex-matched healthy controls (HC). The cell phenotype was assessed by flow cytometry and the cytokine levels in the supernatant were detected by ELISA.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We demonstrate a marked increase in CD161<sup>+</sup> Th1 cells in the synovial fluid of RA patients. These cells exhibit a hyperactivated and hyperproliferative state alongside elevated CCR5 expression. Furthermore, the levels of CD161<sup>+</sup> Th1 cells, CD25, and CCR5 in RA synovial fluid show a positive correlation with the disease activity. Additionally, our study reveals that CCR5 facilitates the activation, proliferation, and cytokine production of CD161<sup>+</sup> Th1 cells through the pZAP70/NFAT signaling pathway.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>These findings contribute to a deeper understanding of RA pathogenesis and uncover a novel mechanism that regulates non-classical CD161<sup>+</sup> Th1 responses in RA, which may provide a potential therapeutic target.</p>\u0000 </section>\u0000 </div>","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"27 11","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142666130","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}
{"title":"Relationship between proton pump inhibitor and acute gout attacks in patients with acute upper gastrointestinal bleeding combined with gout","authors":"Qiaoli Xu","doi":"10.1111/1756-185X.15403","DOIUrl":"10.1111/1756-185X.15403","url":null,"abstract":"","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"27 11","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142647748","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}
Our objective was to investigate real-world outcomes and treatment strategies in individuals affected by DADA2 using over 10-year period real-life experience.
Methods
This descriptive analysis encompassed all adult/pediatric patients with DADA2 from our Vasculitis Research Center prospective database. Patients on anti-TNF therapy have been specifically examined, analyzing the treatment's duration, indications, and outcomes. Treatment responses were based on physicians' assessments and categorized as full response (symptom-free with normal acute phase reactants) or partial/no response.
Results
Totally 32 patients (Adult/Childhood age: 19/13) were analyzed. Anti-TNF agents were prescribed to 27 of the 32 patients. Over a median follow-up of 58 months on anti-TNF therapy, 10 patients (35.7%) exhibited a complete response, predominantly in cases with nervous system or skin involvement. Partial responses were observed in the other 10 patients (35.7%). Currently, 20/ 27 patients remain on anti-TNF treatment. Among the seven who are not on anti-TNF now: five died (four of them with a late diagnosis, one could not continue due to cardiomyopathy), one refused treatment and one had a cure after bone marrow transplantation. We have become aware that four patients increased their dose interval and one returned to the normal interval after an increase in CRP. The first patient was diagnosed in 2013 and over the last 10 years, 6/32 (18.8%) of the patients died.
Conclusion
Anti-TNF treatment is beneficial for vasculitic and inflammatory lesions. The clinical course of patients is diverse, especially if the diagnosis is delayed, with a mortality rate of up to 20% over a 10-year period.
{"title":"Long-term follow-up of anti-TNF treatment in adult and pediatric DADA2 patients: Insights from real-world data","authors":"Gizem Ayan, Ozge Basaran, Busra Firlatan, Levent Kilic, Yelda Bilginer, Mehmet Alikasifoglu, Omer Karadag, Seza Ozen","doi":"10.1111/1756-185X.15377","DOIUrl":"https://doi.org/10.1111/1756-185X.15377","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background/Purpose</h3>\u0000 \u0000 <p>Our objective was to investigate real-world outcomes and treatment strategies in individuals affected by DADA2 using over 10-year period real-life experience.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This descriptive analysis encompassed all adult/pediatric patients with DADA2 from our Vasculitis Research Center prospective database. Patients on anti-TNF therapy have been specifically examined, analyzing the treatment's duration, indications, and outcomes. Treatment responses were based on physicians' assessments and categorized as full response (symptom-free with normal acute phase reactants) or partial/no response.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Totally 32 patients (Adult/Childhood age: 19/13) were analyzed. Anti-TNF agents were prescribed to 27 of the 32 patients. Over a median follow-up of 58 months on anti-TNF therapy, 10 patients (35.7%) exhibited a complete response, predominantly in cases with nervous system or skin involvement. Partial responses were observed in the other 10 patients (35.7%). Currently, 20/ 27 patients remain on anti-TNF treatment. Among the seven who are not on anti-TNF now: five died (four of them with a late diagnosis, one could not continue due to cardiomyopathy), one refused treatment and one had a cure after bone marrow transplantation. We have become aware that four patients increased their dose interval and one returned to the normal interval after an increase in CRP. The first patient was diagnosed in 2013 and over the last 10 years, 6/32 (18.8%) of the patients died.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Anti-TNF treatment is beneficial for vasculitic and inflammatory lesions. The clinical course of patients is diverse, especially if the diagnosis is delayed, with a mortality rate of up to 20% over a 10-year period.</p>\u0000 </section>\u0000 </div>","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"27 11","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142666026","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}
Background: Retroperitoneal Fibrosis (RPF) is an interesting yet daunting rare clinical encounter, especially one that is associated with SLE. It was first described by Albarran in 1905, subsequently by Ormond in 1948, which involves the formation of fibrous bands around the abdominal aorta and its surrounding retroperitoneal organs. SLE coronary vasculitis is also another underappreciated entity, one that is occasionally encountered among young individuals in clinical practice, but not widely discussed in the literature till date.
Case Presentation: We describe a young 38-year-old Malaysian female who was diagnosed with SLE and RPF after she presented with a 6-month history of a left lower quadrant abdominal pain and a worsening stable angina. She had undergone a recent coronary arterial bypass grafting (CABG) for a triple coronary arterial disease 1 year prior to this presentation. Her Computed Tomography Scan with Angiogram demonstrated periaortitis, RPF and a mural thrombus extending from the renal hilar region down to the common iliac arteries. Her CT coronary angiogram demonstrated microaneurysms and beadings. Her IgG4 level, cANCA and pANCA were negative. There were clinical and radiological improvements with intravenous (IV) Cyclophosphamide and high dose corticosteroids.
Conclusion: Corticosteroids and immunosuppressants remain the preferred treatment modalities for RPF across case studies, with an optimistic prognosis. Relapses have been reported, especially ones that are associated with a positive anti-nuclear antigen (ANA) level. Therefore, patients should be educated and followed up closely.
Tey S.1; Ahmad A.1; Samat S.1; Mohd Perdaus A.21KPJ Rawang 专科医院; 2KPJ Damansara 专科医院背景：腹膜后纤维化（RPF）是一种有趣而又令人生畏的罕见临床病，尤其是与系统性红斑狼疮相关的疾病。腹膜后纤维化最早由阿尔巴兰（Albarran）于1905年描述，随后由奥蒙德（Ormond）于1948年描述，它涉及腹主动脉及其周围腹膜后器官周围纤维带的形成。系统性红斑狼疮冠状血管炎也是另一种未被充分重视的疾病，在临床实践中偶尔会在年轻人中遇到，但迄今为止在文献中并未得到广泛讨论：我们描述了一名 38 岁的年轻马来西亚女性，她因 6 个月的左下腹痛和不断恶化的稳定型心绞痛病史而被诊断为系统性红斑狼疮和冠状动脉血管炎。一年前，她曾因三支冠状动脉疾病接受过冠状动脉旁路移植术（CABG）。她的计算机断层扫描和血管造影显示她患有大动脉周围炎、RPF 和壁状血栓，血栓从肾门区一直延伸到髂总动脉。她的 CT 冠状动脉造影显示出微动脉瘤和血珠。她的 IgG4 水平、cANCA 和 pANCA 均为阴性。静脉注射环磷酰胺和大剂量皮质类固醇后，她的临床和放射学状况均有所改善：结论：在所有病例研究中，皮质类固醇和免疫抑制剂仍是治疗 RPF 的首选方法，预后乐观。有复发的报道，尤其是与抗核抗原（ANA）阳性水平相关的复发。因此，应对患者进行教育和密切随访：系统性红斑狼疮 腹膜后纤维化 冠状动脉血管炎
{"title":"Poster Abstracts Part B","authors":"","doi":"10.1111/1756-185X.15346","DOIUrl":"10.1111/1756-185X.15346","url":null,"abstract":"<p><span>Tey S.</span><sup>1</sup>; <span>Ahmad A.</span><sup>1</sup>; Samat S.<sup>1</sup>; Mohd Perdaus A.<sup>2</sup></p><p><sup>1</sup>KPJ Rawang Specialist Hospital; <sup>2</sup>KPJ Damansara Specialist Hospital</p><p><b>Background:</b> Retroperitoneal Fibrosis (RPF) is an interesting yet daunting rare clinical encounter, especially one that is associated with SLE. It was first described by Albarran in 1905, subsequently by Ormond in 1948, which involves the formation of fibrous bands around the abdominal aorta and its surrounding retroperitoneal organs. SLE coronary vasculitis is also another underappreciated entity, one that is occasionally encountered among young individuals in clinical practice, but not widely discussed in the literature till date.</p><p><b>Case Presentation:</b> We describe a young 38-year-old Malaysian female who was diagnosed with SLE and RPF after she presented with a 6-month history of a left lower quadrant abdominal pain and a worsening stable angina. She had undergone a recent coronary arterial bypass grafting (CABG) for a triple coronary arterial disease 1 year prior to this presentation. Her Computed Tomography Scan with Angiogram demonstrated periaortitis, RPF and a mural thrombus extending from the renal hilar region down to the common iliac arteries. Her CT coronary angiogram demonstrated microaneurysms and beadings. Her IgG4 level, cANCA and pANCA were negative. There were clinical and radiological improvements with intravenous (IV) Cyclophosphamide and high dose corticosteroids.</p><p><b>Conclusion:</b> Corticosteroids and immunosuppressants remain the preferred treatment modalities for RPF across case studies, with an optimistic prognosis. Relapses have been reported, especially ones that are associated with a positive anti-nuclear antigen (ANA) level. Therefore, patients should be educated and followed up closely.</p><p><b>Key Words</b>: SLE, Retroperitoneal Fibrosis, Coronary Vasculitis</p>","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"27 S3","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1756-185X.15346","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142620451","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}
{"title":"Verification of flare definition of rheumatoid arthritis based on SDAI and CDAI","authors":"Ichiro Yoshii, Naoya Sawada, Tatsumi Chijiwa","doi":"10.1111/1756-185X.15411","DOIUrl":"10.1111/1756-185X.15411","url":null,"abstract":"","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"27 11","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142620445","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}
Shumin Zhang, Dongxia Liu, Cuiyan Wang, Hongsheng Sun
{"title":"Attention should be paid to screening for cardiac diseases in patients with ANCA-associated vasculitis","authors":"Shumin Zhang, Dongxia Liu, Cuiyan Wang, Hongsheng Sun","doi":"10.1111/1756-185X.15382","DOIUrl":"10.1111/1756-185X.15382","url":null,"abstract":"","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"27 11","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142620273","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}
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