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Efficacy and safety of avacopan in antineutrophil cytoplasmic autoantibody-associated vasculitis: a retrospective cohort study in Japan.
IF 2.1 Q3 RHEUMATOLOGY Pub Date : 2025-01-23 DOI: 10.1186/s41927-025-00456-4
Genri Tagami, Makoto Yamaguchi, Hirokazu Sugiyama, Hiroshi Kinashi, Kentaro Imai, Keisuke Kamiya, Takayuki Katsuno, Takahiro Imaizumi, Shogo Banno, Yasuhiko Ito, Takuji Ishimoto

Background: Avacopan, an oral C5a receptor antagonist, demonstrated efficacy as an alternative to glucocorticoid therapy in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in the phase 3 ADVOCATE trial. However, limited real-world data exist on the outcomes and experiences associated with avacopan use for AAV in Japan.

Methods: We performed a single-centre retrospective analysis and evaluated 21 patients with newly diagnosed or relapsed AAV who received avacopan. The co-primary outcomes were clinical remission at 6 and 12 months.

Results: Among the 21 patients, 20 (95.2%) achieved clinical remission at 6 months, and 19 (90.4%) sustained remission at 12 months. The median time from initiation of immunosuppressive therapy to the start of avacopan was 12 days (interquartile range, 5-26). Adverse events were reported in 10 patients (47.6%), with elevated liver enzyme levels observed in eight patients (38.1%) as the most frequent complication. Avacopan was discontinued in nine patients (42.9%). Despite early discontinuation, these patients achieved comparable rates of clinical remission at 6 months, sustained remission at 12 months, and experienced a reduction in glucocorticoid doses relative to those who continued avacopan.

Conclusions: A high incidence of adverse events, particularly liver enzyme elevation, and frequent early discontinuations of avacopan were observed. Nevertheless, favourable clinical outcomes and reduced glucocorticoid doses were achieved regardless of avacopan discontinuation. Further studies are warranted to validate the optimal use of avacopan in clinical practice.

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引用次数: 0
Use of disease modifying anti-rheumatic drugs and risk of multiple myeloma in US Veterans with rheumatoid arthritis. 美国类风湿关节炎退伍军人使用改善疾病的抗风湿药物和多发性骨髓瘤的风险
IF 2.1 Q3 RHEUMATOLOGY Pub Date : 2025-01-17 DOI: 10.1186/s41927-025-00457-3
Kate Tokareva, Alexander C Peterson, Aaron Baraff, Sarah P Chung, Jennifer Barton, Joshua F Baker, Bryant R England, Ted R Mikuls, Nicholas L Smith, David G Coffey, Noel S Weiss, Namrata Singh

Background: Biologic (b) and targeted synthetic (ts) disease-modifying anti-rheumatic drugs (DMARDs) used in the management of rheumatoid arthritis (RA) target inflammatory pathways implicated in the pathogenesis of multiple myeloma (MM). It is unknown whether use of b/tsDMARDs affects the incidence of MM.

Methods: In this cohort study using Veterans Health Administration (VHA) data, we identified Veterans newly diagnosed with RA from 1/1/2002 to 12/31/2018 using diagnostic codes and medication fills. DMARD exposure was categorized as follows: conventional synthetic (cs)DMARDs; bDMARDs, which included tumor necrosis factor inhibitors (TNFi), non-TNFi; and a tsDMARD, tofacitinib. A Cox proportional hazards model with time-varying exposure was used to estimate the hazard ratio for developing MM among those who received b/tsDMARD medications relative to b/tsDMARD-naïve persons.

Results: 27,540 veterans with RA met eligibility criteria of whom 8322 (30%) took a b/tsDMARD during follow-up. There were 77 incident cases of MM over 192,000 person-years of follow-up. The age-adjusted incidence rate (IR) of MM among b/tsDMARD-naïve patients was 0.37 (95% CI 0.28-0.49) per 1000 person-years and 0.42 among current or former b/tsDMARD users (95% CI 0.25-0.65). Adjusting for age and other demographic characteristics, the hazard ratio for MM associated with use of b/tsDMARDs was 1.32 (95% CI 0.78, 2.26).

Conclusion: In this study of Veterans with RA, the rate of MM did not differ between b/tsDMARD and csDMARD users. The relatively short duration of follow-up and few events limited our power to detect treatment-related differences in MM risk.

背景:生物制剂(b)和靶向合成(ts)改善疾病的抗风湿药物(DMARDs)用于治疗类风湿性关节炎(RA)的目标炎症途径,涉及多发性骨髓瘤(MM)的发病机制。方法:在这项队列研究中,我们使用退伍军人健康管理局(VHA)的数据,对2002年1月1日至2018年12月31日期间新诊断为RA的退伍军人进行了诊断代码和药物填充。DMARD暴露分类如下:常规合成(cs)DMARD;bDMARDs,包括肿瘤坏死因子抑制剂(TNFi)、非TNFi;和一个tsDMARD,用于facitinib。使用时变暴露的Cox比例风险模型来估计接受b/tsDMARD药物的人相对于b/tsDMARD-naïve人发生MM的风险比。结果:27540例RA退伍军人符合入选标准,其中8322例(30%)在随访期间服用了b/tsDMARD。在192,000人年的随访中,有77例MM病例。b/tsDMARD-naïve患者中MM的年龄调整发病率(IR)为每1000人年0.37 (95% CI 0.28-0.49),目前或曾经使用b/tsDMARD的患者为0.42 (95% CI 0.25-0.65)。调整年龄和其他人口统计学特征后,与b/tsDMARDs使用相关的MM风险比为1.32 (95% CI 0.78, 2.26)。结论:在本研究中患有RA的退伍军人中,b/tsDMARD和csDMARD使用者的MM率没有差异。相对较短的随访时间和较少的事件限制了我们检测治疗相关的MM风险差异的能力。
{"title":"Use of disease modifying anti-rheumatic drugs and risk of multiple myeloma in US Veterans with rheumatoid arthritis.","authors":"Kate Tokareva, Alexander C Peterson, Aaron Baraff, Sarah P Chung, Jennifer Barton, Joshua F Baker, Bryant R England, Ted R Mikuls, Nicholas L Smith, David G Coffey, Noel S Weiss, Namrata Singh","doi":"10.1186/s41927-025-00457-3","DOIUrl":"https://doi.org/10.1186/s41927-025-00457-3","url":null,"abstract":"<p><strong>Background: </strong>Biologic (b) and targeted synthetic (ts) disease-modifying anti-rheumatic drugs (DMARDs) used in the management of rheumatoid arthritis (RA) target inflammatory pathways implicated in the pathogenesis of multiple myeloma (MM). It is unknown whether use of b/tsDMARDs affects the incidence of MM.</p><p><strong>Methods: </strong>In this cohort study using Veterans Health Administration (VHA) data, we identified Veterans newly diagnosed with RA from 1/1/2002 to 12/31/2018 using diagnostic codes and medication fills. DMARD exposure was categorized as follows: conventional synthetic (cs)DMARDs; bDMARDs, which included tumor necrosis factor inhibitors (TNFi), non-TNFi; and a tsDMARD, tofacitinib. A Cox proportional hazards model with time-varying exposure was used to estimate the hazard ratio for developing MM among those who received b/tsDMARD medications relative to b/tsDMARD-naïve persons.</p><p><strong>Results: </strong>27,540 veterans with RA met eligibility criteria of whom 8322 (30%) took a b/tsDMARD during follow-up. There were 77 incident cases of MM over 192,000 person-years of follow-up. The age-adjusted incidence rate (IR) of MM among b/tsDMARD-naïve patients was 0.37 (95% CI 0.28-0.49) per 1000 person-years and 0.42 among current or former b/tsDMARD users (95% CI 0.25-0.65). Adjusting for age and other demographic characteristics, the hazard ratio for MM associated with use of b/tsDMARDs was 1.32 (95% CI 0.78, 2.26).</p><p><strong>Conclusion: </strong>In this study of Veterans with RA, the rate of MM did not differ between b/tsDMARD and csDMARD users. The relatively short duration of follow-up and few events limited our power to detect treatment-related differences in MM risk.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"7"},"PeriodicalIF":2.1,"publicationDate":"2025-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11740324/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142999366","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Correction: Benefits of a Juvenile Arthritis Support Program (JASP-1) for children recently diagnosed with Juvenile Idiopathic Arthritis and their parents. 更正:青少年关节炎支持计划(JASP-1)对最近诊断为青少年特发性关节炎的儿童及其父母的益处。
IF 2.1 Q3 RHEUMATOLOGY Pub Date : 2025-01-15 DOI: 10.1186/s41927-024-00443-1
Karina Mördrup, Johanna Granhagen Jungner, Eva Broström, Karin Palmblad, Cecilia Bartholdson
{"title":"Correction: Benefits of a Juvenile Arthritis Support Program (JASP-1) for children recently diagnosed with Juvenile Idiopathic Arthritis and their parents.","authors":"Karina Mördrup, Johanna Granhagen Jungner, Eva Broström, Karin Palmblad, Cecilia Bartholdson","doi":"10.1186/s41927-024-00443-1","DOIUrl":"https://doi.org/10.1186/s41927-024-00443-1","url":null,"abstract":"","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"6"},"PeriodicalIF":2.1,"publicationDate":"2025-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11734426/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142999365","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Antimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohort. 抗黑色素瘤分化相关基因5(抗mda5)抗体阳性的皮肌炎:不同种族患者队列的临床特征和结果
IF 2.1 Q3 RHEUMATOLOGY Pub Date : 2025-01-09 DOI: 10.1186/s41927-025-00455-5
Sai K Koyoda, Fatema Ezzy, Dawn Wahezi, Anand Kumthekar, Xianhong Xie, Clement E Tagoe, Bibi Ayesha

Background: The anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis is known for its association with rapidly progressive interstitial lung disease (RP-ILD) and ulcerative skin lesions, often presenting with or without muscle involvement. The aim of this study was to identify distinct clinical and laboratory features that could be used to evaluate disease progression in an ethnically diverse cohort of anti-MDA5 dermatomyositis patients at a U.S. academic center.

Methods: A retrospective chart review was conducted on dermatomyositis patients hospitalized at our institution between January 2014 and June 2023. The data were analyzed via Fischer's exact test and a t test.

Results: Among the 195 dermatomyositis patients reviewed, 22 tested positive for the MDA5 antibody, comprising of thirteen adults and nine pediatric patients. Myositis was significantly more common in pediatric patients than in adult patients (p = 0.002). RP-ILD was more frequently observed in adult patients of African ancestry (including both Black Hispanic and Black non-Hispanic individuals) (p = 0.04). There was a significant association noted between Raynaud's phenomenon and ILD (p = 0.02). The overall mortality rate of 13.6% was more favorable than the previously reported rates of 40-60%.

Conclusion: This study enhances our understanding of the disease by emphasizing potential racial and demographic variations, as well as delineating the similarities and differences between adult and pediatric populations.

背景:抗黑色素瘤分化相关基因5(抗mda5)抗体阳性的皮肌炎因其与快速进展的间质性肺疾病(RP-ILD)和溃疡性皮肤病变相关而闻名,通常伴有或不伴有肌肉受累。本研究的目的是在美国一个学术中心确定不同种族的抗mda5皮肌炎患者的不同临床和实验室特征,以评估疾病进展。方法:对2014年1月至2023年6月在我院住院的皮肌炎患者进行回顾性图表分析。数据通过Fischer精确检验和t检验进行分析。结果:在195例皮肌炎患者中,22例MDA5抗体检测阳性,包括13例成人和9例儿科患者。肌炎在儿童患者中比在成人患者中更常见(p = 0.002)。RP-ILD更常见于非洲血统的成年患者(包括西班牙裔黑人和非西班牙裔黑人)(p = 0.04)。雷诺氏现象与ILD之间存在显著相关性(p = 0.02)。13.6%的总死亡率比先前报道的40-60%的死亡率更有利。结论:本研究通过强调潜在的种族和人口差异,以及描述成人和儿科人群之间的异同,增强了我们对该疾病的理解。
{"title":"Antimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohort.","authors":"Sai K Koyoda, Fatema Ezzy, Dawn Wahezi, Anand Kumthekar, Xianhong Xie, Clement E Tagoe, Bibi Ayesha","doi":"10.1186/s41927-025-00455-5","DOIUrl":"10.1186/s41927-025-00455-5","url":null,"abstract":"<p><strong>Background: </strong>The anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis is known for its association with rapidly progressive interstitial lung disease (RP-ILD) and ulcerative skin lesions, often presenting with or without muscle involvement. The aim of this study was to identify distinct clinical and laboratory features that could be used to evaluate disease progression in an ethnically diverse cohort of anti-MDA5 dermatomyositis patients at a U.S. academic center.</p><p><strong>Methods: </strong>A retrospective chart review was conducted on dermatomyositis patients hospitalized at our institution between January 2014 and June 2023. The data were analyzed via Fischer's exact test and a t test.</p><p><strong>Results: </strong>Among the 195 dermatomyositis patients reviewed, 22 tested positive for the MDA5 antibody, comprising of thirteen adults and nine pediatric patients. Myositis was significantly more common in pediatric patients than in adult patients (p = 0.002). RP-ILD was more frequently observed in adult patients of African ancestry (including both Black Hispanic and Black non-Hispanic individuals) (p = 0.04). There was a significant association noted between Raynaud's phenomenon and ILD (p = 0.02). The overall mortality rate of 13.6% was more favorable than the previously reported rates of 40-60%.</p><p><strong>Conclusion: </strong>This study enhances our understanding of the disease by emphasizing potential racial and demographic variations, as well as delineating the similarities and differences between adult and pediatric populations.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"5"},"PeriodicalIF":2.1,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11715331/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142944719","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Real-world effectiveness of intravenous belimumab in adults with systemic lupus erythematosus: results of the observational OBSErve study in the Russian Federation. 静脉注射贝利单抗治疗成人系统性红斑狼疮的实际疗效:俄罗斯联邦观察性研究的结果。
IF 2.1 Q3 RHEUMATOLOGY Pub Date : 2025-01-08 DOI: 10.1186/s41927-024-00452-0
Alexander Mikhailovich Lila, Elena Aleksandrovna Aseeva, Alyona Igorevna Zagrebneva, Irina Borisovna Vinogradova, Ruzana Ramilovna Samigullina, Munther Khamashta, Tamer Elfishawy, Lindsey Teichman, Debora Dos Santos, Juliana Queiroz, Larisa Alexandrovna Kniazeva, Saeed Noibi

Background: The real-world effectiveness of intravenous (IV) belimumab in treating systemic lupus erythematosus (SLE) has been demonstrated in various countries through the OBSErve (evaluation Of use of Belimumab in clinical practice SEttings) program. Here we describe the clinical effectiveness of IV belimumab for treating SLE in real-world clinical practice in the Russian Federation.

Methods: In the retrospective, observational OBSErve Russia study (GSK Study 215349), eligible physicians enrolled adults with SLE receiving IV belimumab as part of their standard care. De-identified data were collected from patient medical records from September 2021 to March 2022. The primary outcome was the physician-assessed overall clinical response at 6 months post-index versus index (belimumab initiation) among patients receiving belimumab for ≥6 months. Other endpoints included change in Safety of Estrogens in Lupus Erythematosus National Assessment - SLE Disease Activity Index (SELENA-SLEDAI) score and glucocorticoid use.

Results: Overall, 59 patients initiated IV belimumab, mainly due to the previous regimen not being effective and to decrease glucocorticoid use (76.3% each); 15.3% of patients started belimumab within the first year of SLE diagnosis. Only 13.6% of patients discontinued belimumab within the first 6 months, mainly due to loss to follow-up and loss of insurance/reimbursement. At 6 months post-index, among patients who completed ≥6 months of belimumab therapy (full analysis set, n = 53), 90.6% and 60.4% had an overall clinical improvement of ≥20% and ≥50%, respectively. Mean (standard deviation, SD) change in SELENA-SLEDAI score from index to 6 months post-index was -5.9 (4.3). Mean (SD) glucocorticoid dose decreased from 12.2 (7.3) mg/day at index to 8.6 (5.1) mg/day at 6 months post-index (n = 50).

Conclusions: Patients with SLE receiving IV belimumab for 6 months in real-world settings in the Russian Federation experienced overall clinical improvements and reductions in glucocorticoid use, which is an important long-term strategy of SLE treatment.

背景:静脉注射(IV)贝利姆单抗治疗系统性红斑狼疮(SLE)的实际有效性已在多个国家通过观察(临床实践环境中贝利姆单抗使用评估)项目得到证实。在这里,我们描述了在俄罗斯联邦现实世界的临床实践中,静脉注射贝利单抗治疗SLE的临床效果。方法:在回顾性、观察性的俄罗斯研究(GSK研究215349)中,符合条件的医生招募了接受静脉注射贝利单抗作为标准治疗一部分的SLE成人患者。从2021年9月至2022年3月的患者医疗记录中收集去识别数据。主要结局是在接受贝利单抗治疗≥6个月的患者中,医生评估的指数与指数(贝利单抗起始)后6个月的总体临床反应。其他终点包括红斑狼疮国家评估-SLE疾病活动指数(SELENA-SLEDAI)评分和糖皮质激素使用的雌激素安全性的变化。结果:总体而言,59例患者开始静脉注射贝利单抗,主要是由于先前的方案无效和减少糖皮质激素的使用(各占76.3%);15.3%的患者在SLE诊断的一年内开始使用贝利单抗。只有13.6%的患者在前6个月内停药,主要是由于随访失败和保险/报销损失。在指数后6个月,在完成≥6个月贝利姆单抗治疗的患者中(完整分析集,n = 53), 90.6%和60.4%的患者分别有≥20%和≥50%的总体临床改善。SELENA-SLEDAI评分从指数到指数后6个月的平均(标准差,SD)变化为-5.9(4.3)。平均(SD)糖皮质激素剂量从指数时的12.2 (7.3)mg/天降至指数后6个月时的8.6 (5.1)mg/天(n = 50)。结论:在俄罗斯联邦的现实环境中,接受6个月静脉注射贝利单抗的SLE患者总体临床改善,糖皮质激素的使用减少,这是SLE治疗的重要长期策略。
{"title":"Real-world effectiveness of intravenous belimumab in adults with systemic lupus erythematosus: results of the observational OBSErve study in the Russian Federation.","authors":"Alexander Mikhailovich Lila, Elena Aleksandrovna Aseeva, Alyona Igorevna Zagrebneva, Irina Borisovna Vinogradova, Ruzana Ramilovna Samigullina, Munther Khamashta, Tamer Elfishawy, Lindsey Teichman, Debora Dos Santos, Juliana Queiroz, Larisa Alexandrovna Kniazeva, Saeed Noibi","doi":"10.1186/s41927-024-00452-0","DOIUrl":"10.1186/s41927-024-00452-0","url":null,"abstract":"<p><strong>Background: </strong>The real-world effectiveness of intravenous (IV) belimumab in treating systemic lupus erythematosus (SLE) has been demonstrated in various countries through the OBSErve (evaluation Of use of Belimumab in clinical practice SEttings) program. Here we describe the clinical effectiveness of IV belimumab for treating SLE in real-world clinical practice in the Russian Federation.</p><p><strong>Methods: </strong>In the retrospective, observational OBSErve Russia study (GSK Study 215349), eligible physicians enrolled adults with SLE receiving IV belimumab as part of their standard care. De-identified data were collected from patient medical records from September 2021 to March 2022. The primary outcome was the physician-assessed overall clinical response at 6 months post-index versus index (belimumab initiation) among patients receiving belimumab for ≥6 months. Other endpoints included change in Safety of Estrogens in Lupus Erythematosus National Assessment - SLE Disease Activity Index (SELENA-SLEDAI) score and glucocorticoid use.</p><p><strong>Results: </strong>Overall, 59 patients initiated IV belimumab, mainly due to the previous regimen not being effective and to decrease glucocorticoid use (76.3% each); 15.3% of patients started belimumab within the first year of SLE diagnosis. Only 13.6% of patients discontinued belimumab within the first 6 months, mainly due to loss to follow-up and loss of insurance/reimbursement. At 6 months post-index, among patients who completed ≥6 months of belimumab therapy (full analysis set, n = 53), 90.6% and 60.4% had an overall clinical improvement of ≥20% and ≥50%, respectively. Mean (standard deviation, SD) change in SELENA-SLEDAI score from index to 6 months post-index was -5.9 (4.3). Mean (SD) glucocorticoid dose decreased from 12.2 (7.3) mg/day at index to 8.6 (5.1) mg/day at 6 months post-index (n = 50).</p><p><strong>Conclusions: </strong>Patients with SLE receiving IV belimumab for 6 months in real-world settings in the Russian Federation experienced overall clinical improvements and reductions in glucocorticoid use, which is an important long-term strategy of SLE treatment.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"4"},"PeriodicalIF":2.1,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11708191/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142944731","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Is middle East pain syndrome (MEPS) a variant of fibromyalgia syndrome or a distinct disease? 中东疼痛综合征(MEPS)是纤维肌痛综合征的一种变体还是一种独特的疾病?
IF 2.1 Q3 RHEUMATOLOGY Pub Date : 2025-01-08 DOI: 10.1186/s41927-024-00428-0
Mona H Elhamamy, Adel A Elbeialy, Maha S Mohamed, Sabah E Abdelraheem, Hala M Elzomor

Background: Fibromyalgia Syndrome (FMS) is a chronic disabling musculoskeletal condition of unknown aetiology characterized by generalized musculoskeletal pain, extreme fatigue, mood disturbance, impaired cognition, and lack of refreshing sleep. Middle East pain syndrome (MEPS) is a newly described pollution-induced syndrome of hyperparathyroidism and fibromyalgia mimicking rheumatoid arthritis, characterized by the radiological presence of spur-like excrescences in terminal phalanges. This study aimed to explore the inflammatory nature of Middle East pain and Fibromyalgia syndromes.

Methods: Eighty primary fibromyalgia patients were included in this study. They were divided into two groups, group [1] 1 of 40 FMS patients with low vitamin D levels and secondary hyperparathyroidism, which were diagnosed as MEPS, and group [2] of 40 primary FMS patients. They were subjected to full medical history taking, clinical examination and laboratory assessment including serum IL-17 by enzyme-linked immunosorbent assay technique, as well as assessment of Madrid Sonographic Enthesitis Index (MASEI) using musculoskeletal ultrasound and nailfold capillaroscopic pattern assessment. Plain X-ray films for hands were done on all patients.

Results: There was a statistically significant elevation of serum IL17 in the MEPS group (median = 58.3 ng/L) compared to the FMS group (median = 45.7 ng/L) as the p-value is < 0.05. Capillaroscopic examination revealed a statistically significant difference between MEPS and FMS groups regarding angiogenesis as the p-value is < 0.05. The ultrasonographic examination also showed a statistically significant difference between MEPS and FMS groups as regards MASEI score as the p-value is < 0.05. Hands X-rays evidenced the exclusive existence of tuft spur-like excrescences in MEPS patients only.

Conclusion: Elevated IL-17 levels, non-scleroderma pattern capillaroscopic and enthesopathy findings in both MEPS and FMS patients are strongly supportive that inflammatory mechanisms participate in the pathogenesis of both diseases. The significant increase of these findings in MEPS than FMS patients as well as the presence of hand tufts spur-like excrescences, confirm that the newly discovered MEPS is a different disease although it involves fibromyalgia symptoms and signs.

背景:纤维肌痛综合征(FMS)是一种慢性致残性肌肉骨骼疾病,病因不明,以全身肌肉骨骼疼痛、极度疲劳、情绪障碍、认知障碍和缺乏提神睡眠为特征。中东疼痛综合征(MEPS)是一种由污染引起的类似类风湿关节炎的甲状旁腺功能亢进和纤维肌痛综合征,其特征是末梢指骨放射学上存在刺状赘生物。本研究旨在探讨中东疼痛和纤维肌痛综合征的炎症性质。方法:选取80例原发性纤维肌痛患者作为研究对象。将患者分为两组,[1]组(1)和[2]组(40例原发性FMS患者,分别为维生素D水平低和继发性甲状旁腺功能亢进,诊断为MEPS)。对患者进行完整的病史记录、临床检查和实验室评估,包括采用酶联免疫吸附法测定血清IL-17,以及采用肌肉骨骼超声和甲襞毛细血管镜评估马德里超声腹膜炎指数(MASEI)。所有患者均行手部x线平片。结果:MEPS组血清IL-17水平升高(中位数= 58.3 ng/L)较FMS组(中位数= 45.7 ng/L)有统计学意义,p值为。结论:MEPS和FMS患者血清IL-17水平升高、非硬皮病型毛细血管镜及骨髓瘤病变结果均有力支持炎症机制参与两种疾病的发病机制。这些发现在MEPS患者中比FMS患者显著增加,以及手簇刺样赘生物的存在,证实了新发现的MEPS是一种不同的疾病,尽管它涉及纤维肌痛的症状和体征。
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引用次数: 0
Assessing the relation between systemic lupus erythematosus and metabolic syndrome in Syria: a cross-sectional study. 评估叙利亚系统性红斑狼疮和代谢综合征之间的关系:一项横断面研究。
IF 2.1 Q3 RHEUMATOLOGY Pub Date : 2025-01-03 DOI: 10.1186/s41927-024-00453-z
Noura Mallouhi, Ahmad Nabil Alhouri, Naram Khalayli, Hasan Nabil Alhouri, Mayssoun Kudsi, Younes Kabalan

Background: Systemic Lupus Erythematosus (SLE) affects all organ systems. As a result, fat intake and sedentary life are evident in the modern world. The prevalence of metabolic syndrome, with its components, increased, leading to increased mortality. We aimed to investigate the prevalence of metabolic syndrome in SLE and its relationship with disease activity.

Methods: A cross-sectional study was conducted on 70 SLE patients at Al Mouwasat University Hospitals in Damascus, Syria, between November 2021 and November 2022. The patients were divided into two groups based on the presence or absence of metabolic syndrome. The SLE Disease Activity Index (SLEDAI) was assessed in each group and compared with different disease parameters.

Results: Out of the 70 patients, 65 were females. The mean age was 32.19 ± 7.15 years, and the mean disease duration was 4.4 ± 2.96 years. Metabolic syndrome was found in 32 patients (45.7%). Metabolic syndrome in SLE patients was associated with a higher disease activity index, older age, delayed age at first diagnosis, longer disease duration, higher frequency of renal involvement, and use of cyclophosphamide.

Conclusion: Our study highlights the importance of evaluating and treating metabolic syndrome and its components in patients with SLE, as it may play a role in controlling disease activity. We recommend conducting larger studies in the future to overcome the limitations of this research, such as including a larger number of patients, conducting multicenter studies to generalize the results, and including a healthy control group.

背景:系统性红斑狼疮(SLE)影响所有器官系统。因此,脂肪摄入和久坐生活在现代社会是显而易见的。代谢综合征及其组成部分的患病率上升,导致死亡率上升。我们的目的是调查代谢综合征在SLE中的患病率及其与疾病活动度的关系。方法:在2021年11月至2022年11月期间,对叙利亚大马士革Al Mouwasat大学医院的70例SLE患者进行了横断面研究。根据有无代谢综合征将患者分为两组。评估各组SLE疾病活动指数(SLEDAI),并与不同疾病参数进行比较。结果:70例患者中,女性65例。平均年龄32.19±7.15岁,平均病程4.4±2.96年。代谢综合征32例(45.7%)。SLE患者的代谢综合征与较高的疾病活动指数、年龄较大、首次诊断年龄延迟、病程较长、肾脏受累频率较高以及使用环磷酰胺相关。结论:我们的研究强调了评估和治疗SLE患者代谢综合征及其组成部分的重要性,因为它可能在控制疾病活动中发挥作用。我们建议在未来进行更大规模的研究,以克服本研究的局限性,例如纳入更多的患者,开展多中心研究以推广结果,并纳入健康对照组。
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引用次数: 0
Association between red blood cell distribution width and genetic risk in patients with rheumatoid arthritis: a prospective cohort study and Mendelian randomization analysis. 类风湿关节炎患者红细胞分布宽度与遗传风险的关系:一项前瞻性队列研究和孟德尔随机化分析
IF 2.1 Q3 RHEUMATOLOGY Pub Date : 2025-01-02 DOI: 10.1186/s41927-024-00451-1
Mingyang Chen, Jing Lei, Zhenqiu Liu, Renjia Zhao, Yanfeng Jiang, Kelin Xu, Tiejun Zhang, Chen Suo, Xingdong Chen

Objective: Elevated red blood cell distribution width (RDW) is associated with increased risk of rheumatoid arthritis (RA), but the potential interactions of RDW with genetic risk of incident RA remain unclear. This study aimed to investigate the associations between RDW, genetics, and the risk of developing RA.

Methods: We analysed data from 145,025 healthy participants at baseline in the UK Biobank. The endpoint was diagnosed rheumatoid arthritis (ICD-10 codes M05 and M06). Using previously reported results, we constructed a polygenic risk score for RA to evaluate the joint effects of RDW and RA-related genetic risk. Two-sample mendelian randomization and bayesian colocalization were used to infer the causal relation between them.

Results: A total of 675 patients with RA were enrolled and had a median followed up of 5.1 years, with an incidence rate of 0.57/1000 person-years. The hazard ratio of RA was 1.89 (95% CI: 1.45, 2.47) in highest RDW quartile group compared with the lowest RDW quartile group. Individuals within the top quintile of PRS showed a significantly high risk of RA. Moreover, Participants with high genetic risk and those in highest RDW group exhibited a significantly elevated hazard ratio (7.67, 95% CI: 3.98, 14.81), as opposed to participants with low genetic risk and those in lowest RDW group. Interactions between PRS and RDW on the multiplicative and additive scale were observed. Mendelian randomization provided suggestive evidence of a bi-directional causal relationship between RDW and RA. Loci near IL6R, IL1RN, FADS1/FADS2, UBE2L3 and HELZ2 showed colocalization.

Conclusion: Increased RDW is associated with elevated risk of incident RA especially in the high genetic risk populations, but only suggestive evidence supports a causal relationship between them.

目的:红细胞分布宽度(RDW)升高与类风湿关节炎(RA)风险增加相关,但RDW与RA发生遗传风险的潜在相互作用尚不清楚。本研究旨在探讨RDW、遗传学和RA发病风险之间的关系。方法:我们分析了英国生物银行145,025名健康参与者的基线数据。终点诊断为类风湿关节炎(ICD-10代码M05和M06)。根据先前报道的结果,我们构建了RA的多基因风险评分,以评估RDW和RA相关遗传风险的联合影响。采用双样本孟德尔随机化和贝叶斯共定位来推断两者之间的因果关系。结果:共有675例RA患者入组,中位随访时间为5.1年,发病率为0.57/1000人年。RDW最高四分位数组RA的风险比为1.89 (95% CI: 1.45, 2.47), RDW最低四分位数组RA的风险比为1.89。PRS排名前五分之一的人患RA的风险明显较高。此外,与低遗传风险组和最低RDW组相比,高遗传风险组和最高RDW组的风险比显著升高(7.67,95% CI: 3.98, 14.81)。观察了PRS和RDW在乘法和加性尺度上的相互作用。孟德尔随机化提供了RDW和RA之间双向因果关系的暗示证据。IL6R、IL1RN、FADS1/FADS2、UBE2L3和HELZ2附近的位点存在共定位。结论:RDW的增加与RA发病风险的增加有关,特别是在高遗传风险人群中,但只有暗示的证据支持两者之间的因果关系。
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引用次数: 0
Evaluation of hematological markers as prognostic tools in rheumatoid arthritis. 血液学指标作为类风湿关节炎预后工具的评价。
IF 2.1 Q3 RHEUMATOLOGY Pub Date : 2024-12-26 DOI: 10.1186/s41927-024-00444-0
Maryam Masoumi, Maryam Bozorgi, Zahra Nourmohammadi, Mohammad Javad Mousavi, Aref Shariati, Jafar Karami

Background: Reducing inflammation is central to the management of RA. However, commonly used markers such as CRP and ESR, along with the DAS-28 score, have shown limitations. Hematologic indices, such as platelet-lymphocyte ratio (PLR), lymphocyte-monocyte ratio (LMR), and neutrophil-lymphocyte ratio (NLR), show potential as reliable indicators of inflammation in RA. This study evaluates these markers across different RA activity levels to identify effective biomarkers for distinguishing active RA from remission.

Materials and methods: 305 RA patients were enrolled in our study, diagnosed by ACR/EULAR 2010 criteria, and divided into four groups according to the DAS28-ESR score. 8 ml of blood were taken for a CBC test and serological tests such as rheumatoid factor (RF), anticyclic citrullinated peptide antibodies (anti-CCP), anti-nuclear antibodies (ANA), and C-reactive protein (CRP). Platelet-lymphocyte ratio (PLR), lymphocyte-monocyte ratio (LMR), and neutrophil-lymphocyte ratio (NLR) were assessed as potential markers of inflammation.

Results: The mean age of RA patients was 51.7 years and a disease duration of 56.7 months. Significant differences in tender and swollen joints were observed between RA groups. Laboratory findings revealed higher CRP and ESR in active RA patients, while hemoglobin, hematocrit, and lymphocyte counts were higher in remission group. ROC analysis showed ESR, CRP, NLR, and PLR as potential markers for distinguishing active from remission RA, with ESR demonstrating the highest diagnostic accuracy. LMR could not differentiate between active and inactive forms of RA disease.

Conclusion: The NLR and PLR markers were significantly correlated with traditional inflammatory markers like CRP and ESR. These novel markers could be useful tools for evaluating RA activity, offering a cost-effective alternative to imaging techniques. Further research is needed to confirm their clinical utility.

背景:减轻炎症是治疗类风湿性关节炎的核心。然而,常用的标志物,如CRP和ESR,以及DAS-28评分,已经显示出局限性。血液学指标,如血小板-淋巴细胞比率(PLR)、淋巴细胞-单核细胞比率(LMR)和中性粒细胞-淋巴细胞比率(NLR),显示出作为RA炎症可靠指标的潜力。本研究评估了不同RA活动水平的这些标记物,以确定区分活动期RA和缓解期RA的有效生物标志物。材料和方法:我们的研究纳入305例RA患者,按照ACR/EULAR 2010标准诊断,根据DAS28-ESR评分分为四组。取血8ml进行CBC检测和血清学检测,如类风湿因子(RF)、抗环瓜氨酸肽抗体(anti-CCP)、抗核抗体(ANA)、c反应蛋白(CRP)。将血小板-淋巴细胞比率(PLR)、淋巴细胞-单核细胞比率(LMR)和中性粒细胞-淋巴细胞比率(NLR)作为炎症的潜在标志物进行评估。结果:RA患者平均年龄51.7岁,病程56.7个月。类风湿关节炎组间关节压痛和肿胀有显著差异。实验室结果显示活动期RA患者的CRP和ESR较高,而缓解组的血红蛋白、红细胞压积和淋巴细胞计数较高。ROC分析显示ESR、CRP、NLR和PLR是区分活动性RA和缓解性RA的潜在标志物,其中ESR的诊断准确性最高。LMR不能区分活动性和非活动性RA疾病。结论:NLR、PLR标志物与CRP、ESR等传统炎症标志物有显著相关性。这些新的标记物可能是评估RA活性的有用工具,提供了一种具有成本效益的替代成像技术。需要进一步的研究来证实它们的临床应用。
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引用次数: 0
The association between composite dietary antioxidant index and rheumatoid arthritis: evidence from NHANES 2001-2020. 膳食复合抗氧化指数与类风湿关节炎之间的关系:来自NHANES 2001-2020的证据
IF 2.1 Q3 RHEUMATOLOGY Pub Date : 2024-12-24 DOI: 10.1186/s41927-024-00447-x
Guangbin Ma, Shulin Zhang, Yiyan Luo, Chengcheng Zhang, Weina Xu, Liyan Wang

Background: This study aimed to investigate the relationship between the composite dietary antioxidant index (CDAI) and rheumatoid arthritis (RA) using data from the National Health and Nutrition Examination Survey (NHANES) spanning from 2001 to 2020.

Methods: CDAI is based on the intake of vitamins A, C, E, manganese, selenium, and zinc from the diet. RA patients were identified through questionnaire responses. Weighted multivariate regression analysis was employed to examine the association between CDAI and RA. Additionally, restricted cubic splines were utilized to assess potential non-linear relationships. Subgroup analyses were used to explore whether the relationship between CDAI and RA remained consistent across subgroups (e.g., sex, age, smoking status, etc.). We also used interaction terms to assess whether these subgroup variables influence the relationship between CDAI and RA risk. Finally, we also performed sensitivity analyses to assess the robustness of the main findings after excluding patients with a history of diabetes.

Results: The study included a total of 11,266 patients. After adjusting for all covariates, the multivariate logistic regression analysis showed that each unit increase in CDAI was associated with a 4% reduction in the odds of RA (odds ratio = 0.96, 95% confidence interval = 0.94-0.99). The incidence of RA was found to decrease as CDAI levels increased (P for trend < 0.05). In the restricted cubic spline analysis, a linear relationship between CDAI and RA was observed. Subgroup analyses and interactions demonstrated that the negative association between CDAI and RA was consistent across all subgroups and was influenced by smoking.

Conclusion: This study indicates a negative correlation between CDAI and RA, suggesting that CDAI may serve as a valuable and convenient marker for reducing the risk of RA in US adults.

Clinical trial number: Not applicable.

背景:本研究旨在利用2001 - 2020年美国国家健康与营养调查(NHANES)的数据,探讨膳食复合抗氧化指数(CDAI)与类风湿关节炎(RA)的关系。方法:CDAI是基于从饮食中摄取维生素A、C、E、锰、硒和锌。通过问卷调查确定RA患者。采用加权多元回归分析检验CDAI与RA的相关性。此外,限制三次样条用于评估潜在的非线性关系。采用亚组分析来探讨CDAI与RA之间的关系是否在不同亚组(如性别、年龄、吸烟状况等)中保持一致。我们还使用相互作用项来评估这些亚组变量是否影响CDAI和RA风险之间的关系。最后,在排除有糖尿病病史的患者后,我们还进行了敏感性分析,以评估主要发现的稳健性。结果:该研究共纳入11,266例患者。在对所有协变量进行调整后,多变量logistic回归分析显示,CDAI每增加一个单位,RA的几率降低4%(优势比= 0.96,95%可信区间= 0.94-0.99)。结论:本研究提示CDAI与RA呈负相关,提示CDAI可作为降低美国成人RA风险的有价值且方便的标志物。临床试验号:不适用。
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引用次数: 0
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BMC Rheumatology
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