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Association of systemic lupus erythematosus standard of care immunosuppressants with glucocorticoid use and disease outcomes: a multicentre cohort study. 系统性红斑狼疮标准治疗免疫抑制剂与糖皮质激素使用和疾病预后的关系:一项多中心队列研究。
IF 2 4区 医学 Q3 RHEUMATOLOGY Pub Date : 2024-05-08 DOI: 10.1186/s42358-024-00366-y
Ricardo Azêdo de Luca Montes, Molla Huq, Timothy Godfrey, Shereen Oon, Alicia Calderone, Rangi Kandane-Rathnayake, Worawit Louthrenoo, Shue-Fen Luo, Yeong-Jian Jan Wu, Vera Golder, Aisha Lateef, Sandra V Navarra, Leonid Zamora, Laniyati Hamijoyo, Sargunan Sockalingam, Yuan An, Zhanguo Li, Yasuhiro Katsumata, Masayoshi Harigai, Madelynn Chan, Fiona Goldblatt, Sean O'Neill, Chak Sing Lau, Jiacai Cho, Alberta Hoi, Chetan S Karyekar, Eric F Morand, Mandana Nikpour

Background: This study examines the association of standard-of-care systemic lupus erythematosus (SLE) medications with key outcomes such as low disease activity attainment, flares, damage accrual, and steroid-sparing, for which there is current paucity of data.

Methods: The Asia Pacific Lupus Collaboration (APLC) prospectively collects data across numerous sites regarding demographic and disease characteristics, medication use, and lupus outcomes. Using propensity score methods and panel logistic regression models, we determined the association between lupus medications and outcomes.

Results: Among 1707 patients followed over 12,689 visits for a median of 2.19 years, 1332 (78.03%) patients achieved the Lupus Low Disease Activity State (LLDAS), 976 (57.18%) experienced flares, and on most visits patients were taking an anti-malarial (69.86%) or immunosuppressive drug (76.37%). Prednisolone, hydroxychloroquine and azathioprine were utilised with similar frequency across all organ domains; methotrexate for musculoskeletal activity. There were differences in medication utilisation between countries, with hydroxychloroquine less frequently, and calcineurin inhibitors more frequently, used in Japan. More patients taking leflunomide, methotrexate, chloroquine/hydroxychloroquine, azathioprine, and mycophenolate mofetil/mycophenolic acid were taking ≤ 7.5 mg/day of prednisolone (compared to > 7.5 mg/day) suggesting a steroid-sparing effect. Patients taking tacrolimus were more likely (Odds Ratio [95% Confidence Interval] 13.58 [2.23-82.78], p = 0.005) to attain LLDAS. Patients taking azathioprine (OR 0.67 [0.53-0.86], p = 0.001) and methotrexate (OR 0.68 [0.47-0.98], p = 0.038) were less likely to attain LLDAS. Patients taking mycophenolate mofetil were less likely to experience a flare (OR 0.79 [0.64-0.97], p = 0.025). None of the drugs was associated with a reduction in damage accrual.

Conclusions: This study suggests a steroid-sparing benefit for most commonly used standard of care immunosuppressants used in SLE treatment, some of which were associated with an increased likelihood of attaining LLDAS, or reduced incidence of flares. It also highlights the unmet need for effective treatments in lupus.

背景:本研究探讨了系统性红斑狼疮(SLE)标准治疗用药与低疾病活动度、复发、损害累积和类固醇节省等主要结果之间的关联,目前这方面的数据还很少:方法:亚太狼疮合作组织(APLC)在众多研究机构中前瞻性地收集了有关人口和疾病特征、药物使用和狼疮治疗效果的数据。我们采用倾向评分法和面板逻辑回归模型,确定了狼疮药物治疗与疗效之间的关联:在中位数为 2.19 年的 12689 次随访中,1707 名患者中有 1332 人(78.03%)达到了狼疮低疾病活动状态 (LLDAS),976 人(57.18%)出现了病情复发,大多数患者都在服用抗疟疾药物(69.86%)或免疫抑制剂(76.37%)。泼尼松龙、羟氯喹和硫唑嘌呤在所有器官领域的使用频率相似;甲氨蝶呤用于肌肉骨骼活动。不同国家在药物使用方面存在差异,在日本,羟氯喹的使用频率较低,而钙神经蛋白抑制剂的使用频率较高。更多服用来氟米特、甲氨蝶呤、氯喹/羟氯喹、硫唑嘌呤和霉酚酸酯/霉酚酸的患者每天服用的泼尼松龙剂量低于 7.5 毫克(而不是 > 7.5 毫克/天),这表明存在类固醇节省效应。服用他克莫司的患者更有可能达到 LLDAS(Odds Ratio [95% Confidence Interval] 13.58 [2.23-82.78],p = 0.005)。服用硫唑嘌呤(OR 0.67 [0.53-0.86],p = 0.001)和甲氨蝶呤(OR 0.68 [0.47-0.98],p = 0.038)的患者获得 LLDAS 的可能性较低。服用霉酚酸酯(mycophenolate mofetil)的患者出现复发的可能性较低(OR 0.79 [0.64-0.97],p = 0.025)。没有一种药物与损害累积的减少有关:这项研究表明,系统性红斑狼疮治疗中最常用的标准疗法免疫抑制剂具有节省类固醇的益处,其中一些药物与达到LLDAS的可能性增加或复发率降低有关。这也凸显了狼疮患者对有效治疗的需求尚未得到满足。
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引用次数: 0
IgG4-related disease—rare but you should not forget it IgG4 相关疾病--罕见但不应忘记
IF 2.3 4区 医学 Q3 RHEUMATOLOGY Pub Date : 2024-05-03 DOI: 10.1186/s42358-024-00374-y
Frederico Augusto Gurgel Pinheiro, Ivanio Alves Pereira, Alexandre Wagner Silva de Souza, Henrique Ayres Mayrink Giardini, Rafael Alves Cordeiro
Immunoglobulin G4-related disease is a systemic immune-mediated disease with insidious evolution characterized by fibroinflammatory lesions over virtually any organ system. Despite the remarkable progression of knowledge, its etiology remains undefined. Due to its relapse-remitting pattern, it could accumulate irreversible damage, increasing comorbidities and mortality. This paper emphasizes key concepts for diagnosing and treating patients with this condition.
免疫球蛋白 G4 相关疾病是一种全身性免疫介导疾病,以几乎所有器官系统的纤维炎性病变为特征,具有隐匿性。尽管人们对该病的认识有了长足的进步,但其病因仍未确定。由于其复发-缓解模式,它可能会累积不可逆转的损害,增加并发症和死亡率。本文强调了诊断和治疗这种疾病患者的关键概念。
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引用次数: 0
Hyperuricemia and intravenous fat emulsion are risk factors for gout flares during active gastrointestinal bleeding: a case control study 高尿酸血症和静脉注射脂肪乳剂是活动性消化道出血期间痛风复发的风险因素:一项病例对照研究
IF 2.3 4区 医学 Q3 RHEUMATOLOGY Pub Date : 2024-05-03 DOI: 10.1186/s42358-024-00376-w
Yujie Jiang, Xuelian Hong, Bingtian Xia, Hongwei Du
It is well-established that patients with a history of gout are more susceptible to experiencing gastrointestinal bleeding. Gout flare during active gastrointestinal bleeding poses a significant challenge due to the gastrointestinal side effects of anti-inflammatory therapy. This study sought to investigate the risk factors associated with gout flares during episodes of gastrointestinal bleeding. We conducted a retrospective observational study involving 94 patients who experienced active gastrointestinal bleeding and had a history of gout. This study was conducted at Jinhua Municipal Central Hospital from January 2019 to October 2022. We collected and recorded demographic information and clinical characteristics. Among the gout flare patients, hyperuricemia and intravenous fat emulsion therapy were more prevalent compared to those who remained stable (81.6% vs. 57.8% and 46.9% vs. 24.4%, p < 0.05). Multivariate logistic regression analysis revealed that both hyperuricemia (odds ratio 2.741, 95% CI 1.014–7.413, p = 0.047) and intravenous fat emulsion therapy (odds ratio 2.645, 95% CI 1.046–6.686, p = 0.040) were independent predictors of gout flares. Furthermore, gout attacks occurred sooner in patients receiving intravenous fat emulsion therapy compared to those not receiving it (median: 4 days (interquartile range: 2) vs. median: 5 days (interquartile range: 2.25), p = 0.049). Our study revealed a high incidence of gout flares during episodes of active gastrointestinal bleeding, with patients undergoing intravenous fat emulsion therapy and those with hyperuricemia being at increased risk.
众所周知,有痛风病史的患者更容易出现消化道出血。由于抗炎治疗的胃肠道副作用,在活动性胃肠道出血期间痛风复发是一项重大挑战。本研究旨在调查胃肠道出血发作期间痛风复发的相关风险因素。我们进行了一项回顾性观察研究,涉及 94 名活动性消化道出血且有痛风病史的患者。这项研究于2019年1月至2022年10月在金华市中心医院进行。我们收集并记录了人口统计学信息和临床特征。在痛风发作患者中,高尿酸血症和静脉注射脂肪乳剂治疗的发病率高于病情稳定者(81.6% vs. 57.8%,46.9% vs. 24.4%,P < 0.05)。多变量逻辑回归分析显示,高尿酸血症(几率比2.741,95% CI 1.014-7.413,p = 0.047)和静脉注射脂肪乳剂治疗(几率比2.645,95% CI 1.046-6.686,p = 0.040)是痛风发作的独立预测因素。此外,与未接受静脉注射脂肪乳剂治疗的患者相比,接受静脉注射脂肪乳剂治疗的患者痛风发作的时间更早(中位数为 4 天(四分位间范围为 1.5 天)):4天(四分位数间距:2)对中位数:5天(四分位数间距:2.25),P = 0.049)。我们的研究显示,在活动性消化道出血期间痛风复发的发生率很高,接受静脉注射脂肪乳剂治疗的患者和高尿酸血症患者的风险更高。
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引用次数: 0
Subclinical joint inflammation in rheumatoid arthritis: comparing thermal and ultrasound imaging at the metacarpophalangeal joint 类风湿性关节炎的亚临床关节炎症:掌指关节热成像与超声波成像的比较
IF 2.3 4区 医学 Q3 RHEUMATOLOGY Pub Date : 2024-05-03 DOI: 10.1186/s42358-024-00377-9
York Kiat Tan, Gek Hsiang Lim
While ultrasound and MRI are both superior to clinical examination in the detection of joint inflammation, there is presently a lack of data whether thermography may be similarly useful in the assessment of joint inflammation in patients with RA. Our study aims to evaluate the use of thermography in detecting subclinical joint inflammation at clinically quiescent (non-tender and non-swollen) metacarpophalangeal joints (MCPJs) in patients with rheumatoid arthritis (RA). The outcomes from thermography in our study will be compared with ultrasonography (which is a more established imaging tool used for joint inflammation assessment in RA). The minimum (Tmin), average (Tavg) and maximum (Tmax) temperatures at the 10 MCPJs of each patient were summed to obtain the Total Tmin, Total Tavg and Total Tmax, respectively. Ultrasound grey-scale (GS) and power Doppler (PD) joint inflammation (scored semi-quantitatively, 0–3) at the 10 MCPJs were summed up to derive the respective TGS and TPD scores per patient. Pearson’s correlation and simple linear regression were respectively used to assess correlation and characterize relationships between thermographic parameters (Total Tmin, Total Tavg and Total Tmax) and ultrasound imaging parameters (TGS, TPD and the number of joint(s) with PD ≥ 1 or GS ≥ 2). In this cross-sectional study, 420 clinically non-swollen and non-tender MCPJs from 42 RA patients were examined. All thermographic parameters (Total Tmin, Total Tavg and Total Tmax) correlated significantly (P-values ranging from 0.001 to 0.0012) with TGS score (correlation coefficient ranging from 0.421 to 0.430), TPD score (correlation coefficient ranging from 0.383 to 0.424), and the number of joint(s) with PD ≥ 1 or GS ≥ 2 (correlation coefficient ranging from 0.447 to 0.465). Similarly, simple linear regression demonstrated a statistically significant relationship (P-values ranging from 0.001 to 0.005) between all thermographic parameters (Total Tmin, Total Tavg and Total Tmax) and ultrasound imaging parameters (TPD and TGS). For the first time, thermographic temperatures were shown to correlate with ultrasound-detected joint inflammation at clinically quiescent MCPJs. The use of thermography in the detection of subclinical joint inflammation in RA appears promising and warrants further investigation.
虽然超声波和核磁共振成像在检测关节炎症方面均优于临床检查,但目前尚缺乏数据显示热成像在评估RA患者关节炎症方面是否同样有用。我们的研究旨在评估热成像技术在检测类风湿关节炎(RA)患者临床静止期(无触痛和无肿胀)掌指关节(MCPJ)亚临床关节炎症中的应用。我们研究中的热成像结果将与超声波成像(用于评估类风湿性关节炎关节炎症的更成熟的成像工具)进行比较。将每位患者 10 个 MCPJ 的最低温度(Tmin)、平均温度(Tavg)和最高温度(Tmax)相加,分别得出总 Tmin、总 Tavg 和总 Tmax。将 10 个 MCPJ 的超声灰度(GS)和功率多普勒(PD)关节炎症(半定量评分,0-3 分)相加,得出每位患者各自的 TGS 和 TPD 分数。皮尔逊相关性和简单线性回归分别用于评估热成像参数(总Tmin、总Tavg和总Tmax)与超声成像参数(TGS、TPD和PD≥1或GS≥2的关节数)之间的相关性和特征。在这项横断面研究中,对 42 名 RA 患者的 420 个临床无肿胀、无触痛的 MCPJ 关节进行了检查。所有热成像参数(总 Tmin、总 Tavg 和总 Tmax)均与 TGS 评分(相关系数为 0.421 至 0.430)、TPD 评分(相关系数为 0.383 至 0.424)以及 PD ≥ 1 或 GS ≥ 2 的关节数量(相关系数为 0.447 至 0.465)显著相关(P 值为 0.001 至 0.0012)。同样,简单线性回归显示,所有热成像参数(总 Tmin、总 Tavg 和总 Tmax)与超声成像参数(TPD 和 TGS)之间均存在显著的统计学关系(P 值范围为 0.001 至 0.005)。该研究首次证明,热成像温度与超声波检测到的临床静止 MCPJ 关节炎症相关。热成像技术在检测RA亚临床关节炎症方面的应用前景广阔,值得进一步研究。
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引用次数: 0
Effectiveness of secukinumab in patients with psoriasis and psoriatic arthritis in a Saudi real-world setting secukinumab在沙特真实世界环境中对银屑病和银屑病关节炎患者的疗效
IF 2.3 4区 医学 Q3 RHEUMATOLOGY Pub Date : 2024-04-29 DOI: 10.1186/s42358-024-00371-1
Ibrahim A. Al-Homood, Mohammed Alajlan, Majid Alberdisi, Mohammad Alturki, Ahmed Ali Ahmed, Nancy Zakaria
Psoriasis (PsO) is an immune-mediated chronic inflammatory disease that results in severe outcomes that impact the patient’s quality of life and work productivity. We investigated the effectiveness of secukinumab in patients with chronic plaque psoriasis and psoriatic arthritis (PsA) over a 12-month period. This was a longitudinal, retrospective study of the medical records of 81 patients with psoriasis and/or psoriatic arthritis who had been treated with secukinumab for at least 12 weeks. The Psoriasis Area Severity Index (PASI), Body Surface Area (BSA) percentage, and Dermatology Quality of Life Index (DLQI) among patients with PsO and PsO-PsA showed a statistically significant decrease from baseline over 12 months by approximately 9.86, 19.3%, and 9.7, respectively (p values < 0.001 for each). Moreover, there was a statistically significant decrease in the overall Disease Activity in Psoriatic Arthritis score (DAPSA) by approximately 22.35 from baseline over 12 months of treatment (p < 0.001). Considering the patients who started secukinumab 12 months or more prior to the study cutoff date, the 12-month retention rate was 85%. In a Saudi real-world setting, secukinumab proved to be an efficient medication with high efficacy and retention rates.
银屑病(PsO)是一种免疫介导的慢性炎症性疾病,严重影响患者的生活质量和工作效率。我们调查了 secukinumab 在 12 个月内对慢性斑块状银屑病和银屑病关节炎(PsA)患者的疗效。这是一项纵向回顾性研究,研究对象是81名接受secukinumab治疗至少12周的银屑病和/或银屑病关节炎患者的病历。银屑病和银屑病关节炎患者的银屑病面积严重指数(PASI)、体表面积(BSA)百分比和皮肤病生活质量指数(DLQI)在12个月内分别比基线值下降了约9.86%、19.3%和9.7%,差异有统计学意义(P值均小于0.001)。此外,在12个月的治疗期间,银屑病关节炎疾病活动性总评分(DAPSA)比基线值下降了约22.35(P值<0.001),具有统计学意义。考虑到在研究截止日期前 12 个月或更早开始使用 secukinumab 的患者,12 个月的保留率为 85%。在沙特的真实世界环境中,secukinumab被证明是一种高效药物,具有很高的疗效和保留率。
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引用次数: 0
Effect size varies based on calculation method and may affect interpretation of treatment effect: an illustration using randomised clinical trials in osteoarthritis 疗效大小因计算方法而异,可能影响治疗效果的解释:以骨关节炎随机临床试验为例说明
IF 2.3 4区 医学 Q3 RHEUMATOLOGY Pub Date : 2024-04-22 DOI: 10.1186/s42358-024-00358-y
Thomas J. Schnitzer, Philip G. Conaghan, Francis Berenbaum, Lucy Abraham, Joseph C. Cappelleri, Andrew G. Bushmakin, Lars Viktrup, Ruoyong Yang, Mark T. Brown
To illustrate how (standardised) effect sizes (ES) vary based on calculation method and to provide considerations for improved reporting. Data from three trials of tanezumab in subjects with osteoarthritis were analyzed. ES of tanezumab versus comparator for WOMAC Pain (outcome) was defined as least squares difference between means (mixed model for repeated measures analysis) divided by a pooled standard deviation (SD) of outcome scores. Three approaches to computing the SD were evaluated: Baseline (the pooled SD of WOMAC Pain values at baseline [pooled across treatments]); Endpoint (the pooled SD of these values at the time primary endpoints were assessed); and Median (the median pooled SD of these values based on the pooled SDs across available timepoints). Bootstrap analyses were used to compute 95% confidence intervals (CI). ES (95% CI) of tanezumab 2.5 mg based on Baseline, Endpoint, and Median SDs in one study were − 0.416 (− 0.796, − 0.060), − 0.195 (− 0.371, − 0.028), and − 0.196 (− 0.373, − 0.028), respectively; negative values indicate pain improvement. This pattern of ES differences (largest with Baseline SD, smallest with Endpoint SD, Median SD similar to Endpoint SD) was consistent across all studies and doses of tanezumab. Differences in ES affect interpretation of treatment effect. Therefore, we advocate clearly reporting individual elements of ES in addition to its overall calculation. This is particularly important when ES estimates are used to determine sample sizes for clinical trials, as larger ES will lead to smaller sample sizes and potentially underpowered studies. Clinicaltrials.gov NCT02697773, NCT02709486, and NCT02528188.
说明(标准化)效应大小(ES)如何因计算方法而异,并提供改进报告的注意事项。我们分析了在骨关节炎患者中进行的三项坦珠单抗试验的数据。在WOMAC疼痛(结果)方面,他尼珠单抗与对比药的ES定义为均值之间的最小二乘差(重复测量分析的混合模型)除以结果评分的集合标准差(SD)。对三种计算标准差的方法进行了评估:基线(基线时 WOMAC 疼痛值的集合标差[各治疗方法的集合标差]);终点(评估主要终点时这些值的集合标差);中位数(根据各可用时间点的集合标差计算这些值的集合标差中位数)。采用 Bootstrap 分析法计算 95% 置信区间 (CI)。在一项研究中,基于基线、终点和中位数标样的坦珠单抗 2.5 mg ES(95% CI)分别为- 0.416(- 0.796,- 0.060)、- 0.195(- 0.371,- 0.028)和- 0.196(- 0.373,- 0.028);负值表示疼痛有所改善。这种ES差异模式(基线SD最大,终点SD最小,中位SD与终点SD相似)在所有研究和坦珠单抗剂量中都是一致的。ES差异会影响治疗效果的解释。因此,我们主张除了整体计算ES外,还应明确报告ES的各个要素。当使用ES估计值来确定临床试验的样本量时,这一点尤为重要,因为较大的ES会导致较小的样本量,并有可能导致研究动力不足。Clinicaltrials.gov NCT02697773、NCT02709486 和 NCT02528188。
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引用次数: 0
Characterization of the patterns of care, access, and direct cost of systemic lupus erythematosus in Brazil: findings from the Macunaíma study 巴西系统性红斑狼疮的护理模式、就医途径和直接费用的特点:Macunaíma 研究的发现
IF 2.3 4区 医学 Q3 RHEUMATOLOGY Pub Date : 2024-04-19 DOI: 10.1186/s42358-024-00369-9
Mirhelen Mendes de Abreu, Odirlei Andre Monticielo, Vander Fernandes, Dalianna Luise Andrade Souto Rodrigues, Cristhiane Almeida Leite da Silva, Alexandre Cristovão Maiorano, Fernando dos Santos Beserra, Flavia Rachel Moreira Lamarão, Bruna Medeiros Gonçalves de Veras, Nathalie David, Magda Araújo, Marcelly Cristinny Ribeiro Alves, Matheus Amaral Stocco, Fernando Mello Lima, Emilly Borret, Andrese Aline Gasparin, Gustavo Flores Chapacais, Guilherme Andrade Bulbol, Diogo da Silva Lima, Natália Jardim Martins da Silva, Marta Maria Costa Freitas, Blanca Elena Rios Gomes Bica, Domingos Sávio Nunes de Lima, Marta Maria das Chagas Medeiros
A cost of illness (COI) study aims to evaluate the socioeconomic burden that an illness imposes on society as a whole. This study aimed to describe the resources used, patterns of care, direct cost, and loss of productivity due to systemic lupus erythematosus (SLE) in Brazil. This 12-month, cross-sectional, COI study of patients with SLE (ACR 1997 Classification Criteria) collected data using patient interviews (questionnaires) and medical records, covering: SLE profile, resources used, morbidities, quality of life (12-Item Short Form Survey, SF-12), and loss of productivity. Patients were excluded if they were retired or on sick leave for another illness. Direct resources included health-related (consultations, tests, medications, hospitalization) or non-health-related (transportation, home adaptation, expenditure on caregivers) hospital resources.Costs were calculated using the unit value of each resource and the quantity consumed. A gamma regression model explored cost predictors for patients with SLE. Overall, 300 patients with SLE were included (92.3% female,mean [standard deviation (SD)] disease duration 11.8 [7.9] years), of which 100 patients (33.3%) were on SLE-related sick leave and 46 patients (15.3%) had stopped schooling. Mean (SD) travel time from home to a care facility was 4.4 (12.6) hours. Antimalarials were the most commonly used drugs (222 [74.0%]). A negative correlation was observed between SF-12 physical component and SLE Disease Activity Index (− 0.117, p = 0.042), Systemic Lupus International CollaboratingClinics/AmericanCollegeofRheumatology Damage Index (− 0.115, p = 0.046), medications/day for multiple co-morbidities (− 0.272, p < 0.001), SLE-specific drugs/day (− 0.113, p = 0.051), and lost productivity (− 0.570, p < 0.001). For the mental component, a negative correlation was observed with medications/day for multiple co-morbidities (− 0.272, p < 0.001), SLE-specific medications/day (− 0.113, p = 0.051), and missed appointments (− 0.232, p < 0.001). Mean total SLE cost was US$3,123.53/patient/year (median [interquartile range (IQR)] US$1,618.51 [$678.66, $4,601.29]). Main expenditure was medication, with a median (IQR) cost of US$910.62 ($460, $4,033.51). Mycophenolate increased costs by 3.664 times (p < 0.001), and inflammatory monitoring (erythrocyte sedimentation rate or C-reactive protein) reduced expenditure by 0.381 times (p < 0.001). These results allowed access to care patterns, the median cost for patients with SLE in Brazil, and the differences across regions driven by biological, social, and behavioral factors. The cost of SLE provides an updated setting to support the decision-making process across the country.
疾病成本(COI)研究旨在评估一种疾病给整个社会带来的社会经济负担。这项研究旨在描述巴西系统性红斑狼疮(SLE)患者所使用的资源、护理模式、直接成本和生产力损失。这项为期 12 个月的横断面系统性红斑狼疮患者 COI 研究(ACR 1997 分类标准)通过患者访谈(问卷调查)和医疗记录收集数据,内容包括研究内容包括系统性红斑狼疮概况、所用资源、发病率、生活质量(12 项简表调查,SF-12)和生产力损失。已退休或因其他疾病休病假的患者不包括在内。直接资源包括与健康相关的(咨询、检查、药物、住院)或与健康无关的(交通、家庭适应、护理人员支出)医院资源。伽马回归模型探讨了系统性红斑狼疮患者的成本预测因素。研究共纳入了 300 名系统性红斑狼疮患者(92.3% 为女性,平均[标准差(SD)]病程为 11.8 [7.9] 年),其中 100 名患者(33.3%)休过与系统性红斑狼疮相关的病假,46 名患者(15.3%)停学。从家到医疗机构的平均(标清)旅行时间为 4.4(12.6)小时。抗疟药是最常用的药物(222 [74.0%])。SF-12身体部分与系统性红斑狼疮疾病活动指数(- 0.117,p = 0.042)、系统性红斑狼疮国际合作诊所/美国风湿病学院损害指数(- 0.115,p = 0.046)、多种并发症药物/天(- 0.272,p < 0.001)、系统性红斑狼疮特异性药物/天(- 0.113,p = 0.051)和生产力损失(- 0.570,p < 0.001)。在精神方面,多种并发症的用药/天(- 0.272,p < 0.001)、系统性红斑狼疮特异性用药/天(- 0.113,p = 0.051)和失约(- 0.232,p < 0.001)呈负相关。系统性红斑狼疮患者的平均总费用为 3,123.53 美元/人/年(中位数[四分位数间距(IQR)]为 1,618.51 美元[678.66 美元,4,601.29 美元])。主要支出为药物费用,中位数(IQR)为 910.62 美元(460 美元至 4,033.51 美元)。霉酚酸盐使费用增加了 3.664 倍(P < 0.001),而炎症监测(红细胞沉降率或 C 反应蛋白)使费用减少了 0.381 倍(P < 0.001)。这些结果有助于了解巴西系统性红斑狼疮患者的就医模式、费用中位数以及生物、社会和行为因素导致的地区间差异。系统性红斑狼疮的费用为全国的决策过程提供了最新的支持。
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引用次数: 0
Unveiling the clinical spectrum of relapsing polychondritis: insights into its pathogenesis, novel monogenic causes, and therapeutic strategies 揭示复发性多软骨炎的临床表现:对其发病机制、新的单基因病因和治疗策略的见解
IF 2.3 4区 医学 Q3 RHEUMATOLOGY Pub Date : 2024-04-16 DOI: 10.1186/s42358-024-00365-z
Blanca E R G Bica, Alexandre Wagner S de Souza, Ivânio Alves Pereira
Relapsing polychondritis is a rare multisystem disease involving cartilaginous and proteoglycan-rich structures. The diagnosis of this disease is mainly suggested by the presence of flares of inflammation of the cartilage, particularly in the ears, nose or respiratory tract, and more rarely, in the presence of other manifestations. The spectrum of clinical presentations may vary from intermittent episodes of painful and often disfiguring auricular and nasal chondritis to an occasional organ or even life-threatening manifestations such as lower airway collapse. There is a lack of awareness about this disease is mainly due to its rarity. In 2020, VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, a novel autoinflammatory syndrome, was described. VEXAS syndrome is attributed to somatic mutations in methionine-41 of UBA1, the major E1 enzyme that initiates ubiquitylation. This new disease entity connects seemingly unrelated conditions: systemic inflammatory syndromes (relapsing chondritis, Sweet’s syndrome, and neutrophilic dermatosis) and hematologic disorders (myelodysplastic syndrome or multiple myeloma). Therefore, this article reviews the current literature on both disease entities.
复发性多软骨炎是一种罕见的多系统疾病,累及软骨和富含蛋白多糖的结构。诊断这种疾病的主要依据是软骨炎症的发作,尤其是在耳朵、鼻子或呼吸道,更罕见的是出现其他表现。该病的临床表现多种多样,从间歇性发作的疼痛、往往是毁容性的耳软骨炎和鼻软骨炎,到偶尔出现的器官甚至危及生命的表现,如下呼吸道塌陷。人们对这种疾病缺乏认识,主要是因为它很少见。2020 年,一种新型自身炎症综合征 VEXAS(空泡、E1 酶、X-连锁、自身炎症、体质)综合征被描述出来。VEXAS 综合征归因于 UBA1(启动泛素化的主要 E1 酶)蛋氨酸-41 的体细胞突变。这一新的疾病实体将看似不相关的疾病联系在一起:全身性炎症综合征(复发性软骨炎、斯威特综合征和嗜中性粒细胞皮肤病)和血液系统疾病(骨髓增生异常综合征或多发性骨髓瘤)。因此,本文回顾了有关这两种疾病实体的现有文献。
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引用次数: 0
Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin 嗜血细胞淋巴组织细胞增多症和巨噬细胞活化综合征:同一枚毁灭性硬币的罕见两面
IF 2.3 4区 医学 Q3 RHEUMATOLOGY Pub Date : 2024-04-16 DOI: 10.1186/s42358-024-00370-2
Flavio Sztajnbok, Adriana Rodrigues Fonseca, Leonardo Rodrigues Campos, Kátia Lino, Marta Cristine Félix Rodrigues, Rodrigo Moulin Silva, Rozana Gasparello de Almeida, Sandro Félix Perazzio, Margarida de Fátima Fernandes Carvalho
Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes of secondary HLH, such as infections and malignancy. In this article, we reviewed the concepts, epidemiology, clinical and laboratory features, diagnosis, differential diagnosis, prognosis, and treatment of HLH and MAS. We also reviewed the presence of MAS in the most common autoimmune diseases that affect children. Both are severe diseases that require prompt diagnosis and treatment to avoid morbidity and mortality.
嗜血细胞淋巴组织细胞增生症(HLH)是一种罕见的遗传性高炎症综合征,早年即会发病。巨噬细胞活化综合征(MAS)通常是指与自身免疫相关的继发性HLH,但继发性HLH也有其他病因,如感染和恶性肿瘤。本文回顾了 HLH 和 MAS 的概念、流行病学、临床和实验室特征、诊断、鉴别诊断、预后和治疗。我们还回顾了 MAS 在影响儿童的最常见自身免疫性疾病中的存在。这两种疾病都很严重,需要及时诊断和治疗,以避免发病和死亡。
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引用次数: 0
Prevalence of spondyloarthritis in inflammatory bowel disease according ASAS and ultrassonography and its correlation with plasma calprotectin 根据 ASAS 和超声波检查得出的炎症性肠病脊柱关节炎患病率及其与血浆钙蛋白的相关性
IF 2.3 4区 医学 Q3 RHEUMATOLOGY Pub Date : 2024-04-15 DOI: 10.1186/s42358-023-00348-6
Míriam Küster Huber, Valeria Valim, Érica Vieira Serrano, José Alexandre Mendonça, Rafael Burgomeister Lourenço, Thaisa Moraes Ribeiro Espírito Santo, Hilde Nordal, Maria de Fátima Bissoli, Maria Bernadete Renoldi de Oliveira Gavi
Enteropathic spondyloarthritis is underdiagnosed and inflammatory biomarkers and ultrasonography (US) could be useful for screening inflammatory bowel disease (IBD) patients. The objective of this study was to evaluate the prevalence of spondyloarthritis (SpA) in IBD patients, according to the Assessment of SpondyloArthritis International Society (ASAS) criteria and the correlation of results of US of entheses and joints with plasma calprotectin levels. This was an observational cross-sectional study. Patients from the IBD outpatient clinic of a reference center were evaluated according to ASAS criteria classification, results of US of entheses and joints, and inflammatory biomarker measurements (erythrocyte sedimentation rates, C-reactive protein levels, fecal and plasma calprotectin levels). A p value lower than 0.05 was considered significant. A total of 30.5% of the studied sample (n = 118) of patients with IBD presented at least one inflammatory musculoskeletal manifestation. The overall prevalence of enteropathic SpA was 13.55%, with 10.16% axial SpA and 4.23% peripheral SpA according to the ASAS criteria. A total of 42.1% of patients had an MASEI score greater than 18, 35.2% had synovitis, and 14.7% had tenosynovitis on US, increasing the frequency of diagnosis of enteropathic SpA to 22.8%. Plasma calprotectin levels were similar to those in healthy controls, and correlated only with the fecal calprotectin level (p 0.041). A total of 13.5% of patients met the criteria in accordance with the ASAS criteria for enteropathic SpA, which increased to 22.8% with the addition of US. The prevalence of enthesitis, synovitis and tenosynovitis by US of symptomatic joints and entheses were 42%, 35% and 14.7% respectively. Plasma calprotectin was correlated with fecal calprotectin but not with inflammatory biomarkers or US or ASAS criteria.
肠病性脊柱关节炎诊断率低,而炎症生物标志物和超声波检查(US)可用于筛查炎症性肠病(IBD)患者。本研究的目的是根据脊柱关节炎国际协会(ASAS)的评估标准,评估脊柱关节炎(SpondyloArthritis,SPA)在 IBD 患者中的患病率,以及粘膜和关节超声检查结果与血浆钙蛋白水平的相关性。这是一项观察性横断面研究。参照中心的 IBD 门诊根据 ASAS 标准分类、内膜和关节 US 检测结果以及炎症生物标志物测量结果(红细胞沉降率、C 反应蛋白水平、粪便和血浆钙蛋白水平)对患者进行了评估。P 值低于 0.05 即为显著。在所研究的样本(n = 118)中,共有 30.5% 的 IBD 患者至少有一种炎症性肌肉骨骼表现。根据 ASAS 标准,肠病性 SpA 的总发病率为 13.55%,其中 10.16% 为轴向 SpA,4.23% 为周围 SpA。共有42.1%的患者的MASEI评分大于18分,35.2%的患者患有滑膜炎,14.7%的患者在US检查中发现了腱鞘炎,这使得肠病性斯巴炎的诊断率增加到22.8%。血浆钙蛋白水平与健康对照组相似,仅与粪便钙蛋白水平相关(P 0.041)。根据 ASAS 标准,共有 13.5% 的患者符合肠病性脊柱炎的标准,加上 US 标准后,这一比例增至 22.8%。通过对有症状的关节和夹缝进行US检查,发现夹缝炎、滑膜炎和腱鞘炎的发病率分别为42%、35%和14.7%。血浆钙蛋白与粪便钙蛋白相关,但与炎症生物标志物或 US 或 ASAS 标准无关。
{"title":"Prevalence of spondyloarthritis in inflammatory bowel disease according ASAS and ultrassonography and its correlation with plasma calprotectin","authors":"Míriam Küster Huber, Valeria Valim, Érica Vieira Serrano, José Alexandre Mendonça, Rafael Burgomeister Lourenço, Thaisa Moraes Ribeiro Espírito Santo, Hilde Nordal, Maria de Fátima Bissoli, Maria Bernadete Renoldi de Oliveira Gavi","doi":"10.1186/s42358-023-00348-6","DOIUrl":"https://doi.org/10.1186/s42358-023-00348-6","url":null,"abstract":"Enteropathic spondyloarthritis is underdiagnosed and inflammatory biomarkers and ultrasonography (US) could be useful for screening inflammatory bowel disease (IBD) patients. The objective of this study was to evaluate the prevalence of spondyloarthritis (SpA) in IBD patients, according to the Assessment of SpondyloArthritis International Society (ASAS) criteria and the correlation of results of US of entheses and joints with plasma calprotectin levels. This was an observational cross-sectional study. Patients from the IBD outpatient clinic of a reference center were evaluated according to ASAS criteria classification, results of US of entheses and joints, and inflammatory biomarker measurements (erythrocyte sedimentation rates, C-reactive protein levels, fecal and plasma calprotectin levels). A p value lower than 0.05 was considered significant. A total of 30.5% of the studied sample (n = 118) of patients with IBD presented at least one inflammatory musculoskeletal manifestation. The overall prevalence of enteropathic SpA was 13.55%, with 10.16% axial SpA and 4.23% peripheral SpA according to the ASAS criteria. A total of 42.1% of patients had an MASEI score greater than 18, 35.2% had synovitis, and 14.7% had tenosynovitis on US, increasing the frequency of diagnosis of enteropathic SpA to 22.8%. Plasma calprotectin levels were similar to those in healthy controls, and correlated only with the fecal calprotectin level (p 0.041). A total of 13.5% of patients met the criteria in accordance with the ASAS criteria for enteropathic SpA, which increased to 22.8% with the addition of US. The prevalence of enthesitis, synovitis and tenosynovitis by US of symptomatic joints and entheses were 42%, 35% and 14.7% respectively. Plasma calprotectin was correlated with fecal calprotectin but not with inflammatory biomarkers or US or ASAS criteria.","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"40 1","pages":""},"PeriodicalIF":2.3,"publicationDate":"2024-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140583870","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}
引用次数: 0
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Advances in Rheumatology
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