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Anti-TIF1γ-positive dermatomyositis with interstitial lung involvement: Report of two cases 抗tif1 γ阳性累及肺间质性皮肌炎2例报告
Pub Date : 2025-10-01 DOI: 10.1016/j.rcreue.2025.06.012
Pablo Arango Guerra , Santiago Monsalve Yepes , Andrés Chavarriaga Restrepo , Carlos Jaime Velásquez

Introduction

The association of certain myositis-specific antibodies (MSA) with the development of interstitial lung disease (ILD) in relation to inflammatory myopathies, such as antisynthetase syndrome and anti-MDA5, is well known. However, the potential relationship with the anti-TIF1γ antibody is not well understood, as the reported cases to date are few and have not allowed for solid confirmation of this association. Most of the literature has focused primarily on the association with malignant neoplasms. We aim to include two new cases in the existing literature to support the assertion of the link despite its infrequency.

Objective

To report two cases of ILD associated with anti-TIF1γ positive dermatomyositis (DM).

Materials and methods

A description of the clinical characteristics of two cases of anti-TIF1γ positive DM with ILD and a comparison with analogous cases.

Results

Two cases are described, the first of a 47-year-old man and the second of a 57-year-old woman, both diagnosed with anti-TIF1γ positive DM with the presence of ILD. Underlying malignancy was ruled out due to the presence of the MSA in question, and other potential causes of ILD were excluded, leading to the initiation of immunosuppressive treatment in both cases.

Conclusion

It is essential to further investigate the relationship between inflammatory myopathy and how different MSAs, other than antisynthetase and anti-MDA5 are also associated with ILD. Finding two cases related to anti-TIF1γ and knowing that there are analogous cases generates this hypothesis.
某些肌炎特异性抗体(MSA)与炎性肌病(如抗合成酶综合征和抗mda5)相关的间质性肺疾病(ILD)的发展之间的关联是众所周知的。然而,与抗tif1γ抗体的潜在关系尚不清楚,因为迄今为止报道的病例很少,而且还没有得到这种关联的确凿证实。大多数文献主要集中在与恶性肿瘤的联系上。我们的目标是在现有文献中包括两个新的案例,以支持这种联系的断言,尽管它不常见。目的报告2例ILD合并抗tif1γ阳性皮肌炎(DM)。材料与方法对2例抗tif1γ阳性DM合并ILD的临床特点进行描述,并与类似病例进行比较。结果报告了两例患者,第一例为47岁男性,第二例为57岁女性,均诊断为抗tif1γ阳性DM并伴有ILD。由于MSA的存在,排除了潜在的恶性肿瘤,并排除了其他可能导致ILD的原因,导致两例患者开始免疫抑制治疗。结论炎性肌病与除抗合成酶和抗mda5外不同msa与ILD的关系有待进一步研究。找到两个与anti-TIF1γ相关的病例,并知道存在类似的病例,从而产生了这一假设。
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引用次数: 0
The coexistence of rheumatoid arthritis and ankylosing spondylitis: Case series and literature review 类风湿关节炎和强直性脊柱炎共存:病例系列和文献回顾
Pub Date : 2025-10-01 DOI: 10.1016/j.rcreue.2025.09.004
Hanene Lassoued Ferjani , Ben Ayed Hiba , Dorra Ben Nessib , Dhia Kaffel , Kaouther Maatallah , Wafa Hamdi

Introduction

Rheumatoid arthritis and ankylosing spondylitis are among the most common rheumatic diseases. The coexistence of both affections in a single patient is highly infrequent but seems to be underestimated in clinical practice.

Materials and methods

We described the clinical, biological, and radiological characteristics of 12 patients with concomitant rheumatoid arthritis and ankylosing spondylitis diagnosed at our hospital. The 28 disease activity score (DAS28), the Bath ankylosing spondylitis disease activity index (BASDAI), and the Ankylosing Spondylitis Disease Activity Score (ASDAS) were used as outcome measures.

Results

Twelve patients with a male-to-female ratio of 0.71 and a mean age of 62.1 ± 12.8 years were included. Rheumatoid arthritis was the first disease diagnosed in seven patients. The mean duration of rheumatoid arthritis diagnosis at the time of ankylosing spondylitis diagnosis was 20.2 ± 25 months. The first sign of ankylosing spondylitis in rheumatoid arthritis patients was incidental radiological sacroiliitis in four patients, inflammatory low back pain in three patients, and distal interphalangeal joint involvement in hands radiographs in two patients. Rheumatoid arthritis was seronegative in four patients. Erosions were observed on radiographs of the hands and/or feet in 66% of the cases and almost all the patients (11/12) had sacroiliitis on imaging studies. The mean values of the DAS28, ASDAS, and BASDAI scores at the initial diagnosis of rheumatoid arthritis/ankylosing spondylitis were 4.54 ± 1.22, 3.1 ± 0.72, and 4.1 ± 0.5, respectively.

Conclusion

The coexistence of Rheumatoid arthritis and ankylosing spondylitis is uncommon but should be considered. We could not draw a conclusion about whether the association of both disease conditions confers different characteristics.
类风湿关节炎和强直性脊柱炎是最常见的风湿病。两种情感共存于一个病人是非常罕见的,但在临床实践中似乎被低估了。材料和方法我们对我院诊断的12例类风湿关节炎合并强直性脊柱炎患者的临床、生物学和放射学特征进行了描述。采用28疾病活动性评分(DAS28)、巴斯强直性脊柱炎疾病活动性指数(BASDAI)和强直性脊柱炎疾病活动性评分(ASDAS)作为结局指标。结果纳入12例患者,男女比0.71,平均年龄62.1±12.8岁。类风湿关节炎是7名患者确诊的第一种疾病。强直性脊柱炎诊断时的平均类风湿关节炎诊断时间为20.2±25个月。类风湿关节炎患者强直性脊柱炎的第一个征象是4例患者的偶发性骶髂炎,3例患者的炎症性腰痛,2例患者的手部x线片显示远端指间关节受累。4例类风湿关节炎患者血清阴性。66%的病例在x线片上观察到手和/或脚的侵蚀,几乎所有的患者(11/12)在影像学检查中都有骶髂炎。类风湿关节炎/强直性脊柱炎初诊时DAS28、ASDAS和BASDAI评分的平均值分别为4.54±1.22、3.1±0.72和4.1±0.5。结论类风湿关节炎与强直性脊柱炎共存并不常见,但应予以重视。我们无法得出结论,这两种疾病的关联是否赋予了不同的特征。
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引用次数: 0
Systematic review and meta-analysis of the low-dose versus high-dose cyclophosphamide regimen for induction treatment in lupus nephritis patients, evidence update 2010–2023 低剂量与高剂量环磷酰胺方案诱导治疗狼疮性肾炎患者的系统评价和荟萃分析,证据更新2010-2023
Pub Date : 2025-10-01 DOI: 10.1016/j.rcreue.2025.08.001
Pedro Arbey Quevedo Mayorga , Javier Mauricio Mora Méndez , Alejandro Aristizábal , Lilian Marcela Estupiñan Moya , Diana Muñoz

Introduction

Lupus nephritis is a common complication in patients with systemic lupus erythematosus. Cyclophosphamide in induction treatment has a good efficacy profile, but greater toxicity.

Objective

To perform a systematic review and meta-analysis, updating the literature that compares the efficacy and safety of cyclophosphamide at high and low doses.

Materials and methods

PRISMA-P methodology, PROSPERO register number CRD42023485477, only randomized clinical trials were included from 2010 to October 2023, in patients over 16 years of age with lupus nephritis class III, IV, V, or V + III, V + IV. Complete response, partial response, relapses, infections, leukopenia, and amenorrhoea were measured, the RR was estimated for a 95% CI, based on the Mantel-Haenszel method, and heterogeneity by I2 and Q test.

Results

415 articles were found, only three were included with 406 patients, 217 for the high dose group and 189 low doses. There were no statistically significant differences with respect to complete response (RR = 0.77, 95% CI 0.60–1.01, p = 0.06), partial response (RR = 0.88, 95% CI 0.67–1.16, p = 0.35), relapses (RR = 2.16, 95% CI 0.18–24.7, p = 0.52) and infections (RR = 0.68, 95% CI 0.45–1.04, p = 0.08), while leukopenia and amenorrhoea were significantly uncommon in the low dose group (RR = 0.41, 95% CI 0.22–0.77, p = 0.01) and (RR = 0.40, 95% CI 0.25–0.64, p = 0.002).

Conclusions

The use of low-dose cyclophosphamide is as effective as the high-dose regimen and the probability of leukopenia and amenorrhoea is lower.
狼疮肾炎是系统性红斑狼疮患者常见的并发症。环磷酰胺在诱导治疗中具有良好的疗效,但毒性较大。目的进行系统回顾和荟萃分析,更新比较高剂量和低剂量环磷酰胺疗效和安全性的文献。材料和方法采用sprima - p方法,PROSPERO注册号CRD42023485477,仅纳入2010年至2023年10月16岁以上狼疮肾炎III、IV、V或V + III, V + IV患者的随机临床试验。测量完全缓解、部分缓解、复发、感染、白细胞减少和闭经,根据Mantel-Haenszel方法估计95% CI的RR,并通过I2和Q检验估计异质性。结果共检索到文献415篇,仅纳入3篇,406例患者,其中高剂量组217篇,低剂量组189篇。没有统计上显著的差异对完全缓解(RR = 0.77,95%可信区间0.60 - -1.01,p = 0.06),局部反应(RR = 0.88,95%可信区间0.67 - -1.16,p = 0.35),复发(RR = 2.16,95%可信区间0.18 - -24.7,p = 0.52)和感染(RR = 0.68,95%可信区间0.45 - -1.04,p = 0.08),而白血球减少症和闭经明显罕见低剂量组(RR = 0.41,95%可信区间0.22 - -0.77,p = 0.01)和(RR = 0.40,95%可信区间0.25 - -0.64,p = 0.002)。结论低剂量环磷酰胺与高剂量环磷酰胺治疗效果相当,发生白细胞减少和闭经的概率较低。
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引用次数: 0
Use of thickened-handled spoon in improving feeding performance in patients with rheumatoid arthritis 加粗柄勺在改善类风湿关节炎患者喂养性能中的应用
Pub Date : 2025-10-01 DOI: 10.1016/j.rcreue.2025.07.002
Kaori Mitchell Gil-Rivasplata, Valeria Katiuska Rojas-Del Aguila, Carlos Manuel Escobar-Galindo, Jesús Martin Trinidad-López

Introduction/objective

Rheumatoid arthritis (RA) primarily affects the hand joints, limiting independence for feeding and requiring the use of assistive technology. The thickening of the handles on the spoon is used as a treatment strategy. However, few studies address how much it helps in RA. Therefore, the study aimed to determine the effect of the use of thickened handle spoons on time, performance quality, and perceived hand effort in the improvement of feeding in users simulating RA with restricted hand movements.

Materials and methods

A quasi-experimental design was used in which RA was simulated with a simulator glove, comparing feeding experiences with regular spoons.

Results

Using spoons with thickened handles had statistically significant effects on the reduction of perceived effort when eating different foods (p = .005); however, there was no significant difference between the number of times food was dropped and the time to complete the activity (p = .78).

Conclusions

Spoons with thickened handles reduce the level of perceived effort, which allows for better joint protection and pain management. It also equals the time and efficiency of eating with a conventional spoon.
类风湿性关节炎(RA)主要影响手部关节,限制进食的独立性,需要使用辅助技术。加厚勺子上的把手是一种治疗策略。然而,很少有研究表明它对类风湿关节炎有多大帮助。因此,本研究旨在确定使用加厚手柄勺子对时间、表现质量和感知手部努力的影响,以改善模拟RA的手部运动受限用户的喂养。材料与方法采用准实验设计,用模拟手套模拟RA,比较常规调羹的喂养体验。结果使用粗柄勺子对减少吃不同食物时的感知努力有统计学意义(p = .005);然而,在食物掉落的次数和完成活动的时间之间没有显著差异(p = .78)。结论:手柄加厚的勺子可以减少感知力度,从而更好地保护关节和控制疼痛。它也相当于用传统勺子吃饭的时间和效率。
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引用次数: 0
Errata in articles published in the journal Revista Colombiana de Reumatología in 2023 and 2024 2023年和2024年发表在《哥伦比亚评论》Reumatología杂志上的文章的勘误表
Pub Date : 2025-10-01 DOI: 10.1016/j.rcreue.2025.07.003
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引用次数: 0
Protective effect of propolis against oxidative stress induced by hydrogen peroxide in vitro peripheral blood mononuclear cells 蜂胶对过氧化氢诱导的体外外周血单核细胞氧化应激的保护作用
Pub Date : 2025-10-01 DOI: 10.1016/j.rcreue.2025.06.001
Luz Elena Triana Vidal, Armando Lucumi Moreno, Ivonne Valeria Ortiz

Introduction

Oxidative stress occurs when there is an imbalance between the production of reactive oxygen species (ROS) and cellular antioxidant activity. Overproduction of ROS causes oxidative damage to major macromolecules, alters homeostasis and leads to the generation of different pathologies. At present, there is no totally effective treatment to counteract these diseases involved with oxidative stress, so it is necessary to investigate new treatment alternatives that suppress the ROS generated. One promising alternative is the use of plant extracts, which have demonstrated a potent antioxidant effect. Efficient cells for the study of pathological diseases related to reactive oxygen species are T lymphocytes, which share systems with neurons such as: the dopaminergic system, death signaling and survival. In vitro lymphocytes are an optimal model for the evaluation of oxidative mechanisms.

Objective

To determine the protective effect of ethanolic extract of propolis against oxidative damage induced by hydrogen peroxide (H2O2) in human lymphocytes in vitro, by means of the cell viability test with trypan blue.

Results

Anova test shows that the concentrations of 0.0225 and 0.045 mg/mL of ethanolic extract of propolis, present protective activity against cell damage caused by H2O2.

Conclusion

This study proposes the ethanolic extract of propolis as a potential pharmacological, useful for treatments against autoinmune disorders.
当活性氧(ROS)的产生和细胞抗氧化活性之间存在不平衡时,就会发生氧化应激。ROS的过量产生导致主要大分子的氧化损伤,改变体内平衡,导致不同病理的产生。目前还没有完全有效的治疗方法来对抗这些与氧化应激有关的疾病,因此有必要研究新的治疗方案来抑制ROS的产生。一种有希望的替代方法是使用植物提取物,它已被证明具有强大的抗氧化作用。研究与活性氧有关的病理疾病的有效细胞是T淋巴细胞,它与神经元共享系统,如:多巴胺能系统、死亡信号和生存。体外淋巴细胞是评价氧化机制的最佳模型。目的通过台盼蓝细胞活力试验,探讨蜂胶醇提物对过氧化氢(H2O2)诱导的体外人淋巴细胞氧化损伤的保护作用。结果sanova实验表明,0.0225和0.045 mg/mL浓度的蜂胶乙醇提取物对H2O2对细胞的损伤具有保护作用。结论蜂胶乙醇提取物具有潜在的药理作用,可用于治疗自身免疫性疾病。
{"title":"Protective effect of propolis against oxidative stress induced by hydrogen peroxide in vitro peripheral blood mononuclear cells","authors":"Luz Elena Triana Vidal,&nbsp;Armando Lucumi Moreno,&nbsp;Ivonne Valeria Ortiz","doi":"10.1016/j.rcreue.2025.06.001","DOIUrl":"10.1016/j.rcreue.2025.06.001","url":null,"abstract":"<div><h3>Introduction</h3><div>Oxidative stress occurs when there is an imbalance between the production of reactive oxygen species (ROS) and cellular antioxidant activity. Overproduction of ROS causes oxidative damage to major macromolecules, alters homeostasis and leads to the generation of different pathologies. At present, there is no totally effective treatment to counteract these diseases involved with oxidative stress, so it is necessary to investigate new treatment alternatives that suppress the ROS generated. One promising alternative is the use of plant extracts, which have demonstrated a potent antioxidant effect. Efficient cells for the study of pathological diseases related to reactive oxygen species are T lymphocytes, which share systems with neurons such as: the dopaminergic system, death signaling and survival. <em>In vitro</em> lymphocytes are an optimal model for the evaluation of oxidative mechanisms.</div></div><div><h3>Objective</h3><div>To determine the protective effect of ethanolic extract of propolis against oxidative damage induced by hydrogen peroxide (H<sub>2</sub>O<sub>2</sub>) in human lymphocytes <em>in vitro</em>, by means of the cell viability test with trypan blue.</div></div><div><h3>Results</h3><div>Anova test shows that the concentrations of 0.0225 and 0.045 mg/mL of ethanolic extract of propolis, present protective activity against cell damage caused by H<sub>2</sub>O<sub>2</sub>.</div></div><div><h3>Conclusion</h3><div>This study proposes the ethanolic extract of propolis as a potential pharmacological, useful for treatments against autoinmune disorders.</div></div>","PeriodicalId":101099,"journal":{"name":"Revista Colombiana de Reumatología (English Edition)","volume":"32 4","pages":"Pages 315-320"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145335267","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A practical perspective on gastrointestinal manifestations of systemic lupus erythematosus 系统性红斑狼疮胃肠道表现的临床观察
Pub Date : 2025-10-01 DOI: 10.1016/j.rcreue.2025.08.003
Juan Camilo Santacruz , Marta Juliana Mantilla , Sandra Pulido , Ferney Africano , Carlos Alberto Agudelo , Rubén Darío Mantilla , Ángelo Arzuaga , John Londoño
Systemic lupus erythematosus is an autoimmune disease with a tendency to wax and wane. Unlike other autoimmune conditions, disease activity in the gastrointestinal system is relatively rare, and its approach can vary, according to different points of view. Despite its low prevalence, gastrointestinal manifestations can be potentially fatal, as occurs in cases of intestinal vasculitis and the consequent mesenteric ischaemia. The spectrum of gastrointestinal involvement in this disease is broad, and includes disease activity, infection associated with immunosuppression, adverse effects of some medications, or other intercurrent processes such as lupus cystitis. The clinical symptoms and laboratory findings are highly diverse, which leads to a delay in diagnosis with consequent organ dysfunction. Early diagnosis can be a challenge because a large proportion of patients present with nonspecific constitutional symptoms associated with nausea, emesis, and abdominal pain. These numerous factors have been an obstacle to further research in this field, making a unified diagnostic and therapeutic approach difficult. Therefore, an advanced search of the literature will be undertaken to obtain the most evidence available on the diagnostic methods and treatments currently available, providing the clinician with more tools to achieve a comprehensive approach.
系统性红斑狼疮是一种自身免疫性疾病,有起起落落的趋势。与其他自身免疫性疾病不同,胃肠道系统的疾病活动相对罕见,其方法可以根据不同的观点而变化。尽管发病率很低,但胃肠道表现可能是致命的,如肠血管炎和随后的肠系膜缺血。此病的胃肠道受累范围很广,包括疾病活动性、免疫抑制相关感染、某些药物的不良反应或其他并发过程,如狼疮膀胱炎。临床症状和实验室结果是高度多样化的,这导致延误诊断和随之而来的器官功能障碍。早期诊断可能是一个挑战,因为很大一部分患者存在与恶心、呕吐和腹痛相关的非特异性体质症状。这些因素已经成为该领域进一步研究的障碍,使统一的诊断和治疗方法变得困难。因此,我们将对文献进行深入的检索,以获得目前可用的诊断方法和治疗的最多证据,为临床医生提供更多的工具来实现全面的方法。
{"title":"A practical perspective on gastrointestinal manifestations of systemic lupus erythematosus","authors":"Juan Camilo Santacruz ,&nbsp;Marta Juliana Mantilla ,&nbsp;Sandra Pulido ,&nbsp;Ferney Africano ,&nbsp;Carlos Alberto Agudelo ,&nbsp;Rubén Darío Mantilla ,&nbsp;Ángelo Arzuaga ,&nbsp;John Londoño","doi":"10.1016/j.rcreue.2025.08.003","DOIUrl":"10.1016/j.rcreue.2025.08.003","url":null,"abstract":"<div><div>Systemic lupus erythematosus is an autoimmune disease with a tendency to wax and wane. Unlike other autoimmune conditions, disease activity in the gastrointestinal system is relatively rare, and its approach can vary, according to different points of view. Despite its low prevalence, gastrointestinal manifestations can be potentially fatal, as occurs in cases of intestinal vasculitis and the consequent mesenteric ischaemia. The spectrum of gastrointestinal involvement in this disease is broad, and includes disease activity, infection associated with immunosuppression, adverse effects of some medications, or other intercurrent processes such as lupus cystitis. The clinical symptoms and laboratory findings are highly diverse, which leads to a delay in diagnosis with consequent organ dysfunction. Early diagnosis can be a challenge because a large proportion of patients present with nonspecific constitutional symptoms associated with nausea, emesis, and abdominal pain. These numerous factors have been an obstacle to further research in this field, making a unified diagnostic and therapeutic approach difficult. Therefore, an advanced search of the literature will be undertaken to obtain the most evidence available on the diagnostic methods and treatments currently available, providing the clinician with more tools to achieve a comprehensive approach.</div></div>","PeriodicalId":101099,"journal":{"name":"Revista Colombiana de Reumatología (English Edition)","volume":"32 4","pages":"Pages 385-393"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145335172","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Agreement in quantifying the extension of autoimmune-associated interstitial lung disease using the Goh method 使用Goh方法量化自身免疫相关间质性肺疾病扩展的一致性
Pub Date : 2025-10-01 DOI: 10.1016/j.rcreue.2025.09.005
Luis Javier Cajas Santana , Santiago Cuero , Gabriela Guerrero , Mayelin Ceballos , María Carolina Torres , Diana Rocio Gil , Yimy F. Medina , Ana Milena Callejas , Javier Leonardo Galindo , Cesar Riascos , Wilmer Aponte , Diana Ochoa , Jennifer Delgadillo

Introduction

Interstitial lung disease (ILD) is one of the leading causes of mortality in autoimmune diseases. The extent of the disease is a determining factor in the prognosis and treatment initiation and monitoring. Quantification using the Goh method is the most commonly used method; however, it is subjective. So far, no studies have evaluated the level of agreement among various readers.

Objective

The study's objective is to determine the interobserver and intra-observer variability in using ILD quantification among physicians from various specialties and levels of experience.

Methods

Images from chest computed tomography of patients with rheumatoid arthritis (RA) or systemic sclerosis (SSc) and ILD were collected. The five necessary cuts described by Goh were extracted to be evaluated by pulmonologists, rheumatologists, radiologists, fellows, and a thoracic radiologist (gold standard). The interobserver and intra-observer variability values were calculated using the intraclass correlation coefficient test or Cohen's Kappa test, depending on the nature of the variable, between each group of medical specialties and in comparison with the gold standard.

Results

Seventy-nine patients were selected, primarily women, 56% having SSc. A total of 1098 CT scans were performed. The intraclass correlation coefficient was .75 (95% CI: .67–.81), including all nine readers. The best correlation with the gold standard was found among pulmonologists (CCI .83) and rheumatologists (CCI .81). According to severity (more significant or less than 20% extension), the Kappa coefficient was .64 among the nine readers. The intraclass correlation coefficient for the average intra-observer correlation of all readers was .89 (95% CI: .81–.93), and the Kappa coefficient was .82.

Conclusion

The Goh method is valuable and highly correlated among a diverse group of specialties that manage ILD, making it a practical tool for assessing the extent of the disease.
间质性肺疾病(ILD)是导致自身免疫性疾病死亡的主要原因之一。疾病的程度是预后和治疗开始和监测的决定性因素。采用Goh法进行定量是最常用的方法;然而,这是主观的。到目前为止,还没有研究评估不同读者的认同程度。本研究的目的是确定来自不同专业和经验水平的医生在使用ILD量化时的观察者之间和观察者内部的变异性。方法收集类风湿关节炎(RA)、系统性硬化症(SSc)和ILD患者的胸部ct图像。Goh描述的五个必要切口被提取出来,由肺科医生、风湿科医生、放射科医生、研究员和一位胸科放射科医生(金标准)进行评估。根据变量的性质,使用类内相关系数检验或Cohen’s Kappa检验计算每组医学专业之间的观察者间和观察者内变异性值,并与金标准进行比较。结果入选患者79例,以女性为主,56%患有SSc。共进行1098次CT扫描。类内相关系数为。75 (95% ci: 0.67)。81),包括所有9个读者。与金标准相关性最好的是肺科医师(CCI)。83)和风湿病学家(CCI .81)。根据严重程度(扩展更显著或小于20%),Kappa系数为。9位读者中有64位。所有读者平均观察者内相关的类内相关系数为。89 (95% ci: 0.81)。93), Kappa系数为0.82。结论:Goh方法在处理ILD的不同专科中是有价值的,并且高度相关,使其成为评估疾病程度的实用工具。
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引用次数: 0
Analysis of inequalities and correction model of the Goh method used in quantifying interstitial lung disease 间质性肺疾病定量Goh法的不等式分析及校正模型
Pub Date : 2025-10-01 DOI: 10.1016/j.rcreue.2025.06.003
Luis Javier Cajas Santana , Daniela Rojas Carvajal , Wendy Torres Chazatar , Laura Mendoza Galindo , Yensi Romero Díaz , Daniel Ramírez Muñoz , Gisella Fernández Salamanca

Introduction

Interstitial lung disease is a significant complication in autoimmune diseases. The extent of involvement is crucial in determining both onset and treatment response. While there are computational methods for this calculation, manual methods, such as the Goh method, remain the most commonly used. However, it has some disadvantages, such as presuming that areas across the lung are equivalent and thus influence the calculation equally. The aim of this study is to analyse these difficulties and find a mathematical correction method.

Materials and methods

High-resolution chest tomography images were obtained from patients with and without ILD. Pulmonary areas were analysed and compared in the slices referenced in the Goh method. Through geometric analysis of lung structure, the slice closest to the average of its areas was identified for comparison with other measurements. Based on this, a correction equation was formulated, and through simulation of various interstitial involvement patterns, the degree of variation was determined.

Results

Images from 80 patients were analysed, mostly women diagnosed with systemic sclerosis. It was found that apical areas (slices 1 and 2) and the sum of left-sided pulmonary areas were 20% smaller compared to basal and right-sided areas, respectively. Simulation with different involvement components found that apical or left-sided disease overestimates extension by 5% to 10%, especially when it exceeds 15%. The third slice was determined to be closest to the average of the areas, and a correction formula was devised based on this.

Conclusions

The Goh method incorrectly assumes the equivalence of pulmonary areas, which can impact calculations. We propose a mathematical correction for this purpose.
间质性肺疾病是自身免疫性疾病的重要并发症。受累程度是决定发病和治疗反应的关键。虽然有许多计算方法,但手工方法,如Goh方法,仍然是最常用的方法。然而,它也有一些缺点,比如假设横跨肺部的面积是相等的,从而对计算产生同样的影响。本研究的目的是分析这些困难,并找到一种数学修正方法。材料和方法对有ILD和无ILD的患者进行高分辨率胸部断层扫描。对Goh方法所参考的切片进行肺面积分析和比较。通过对肺结构的几何分析,确定了最接近其面积平均值的切片,以便与其他测量结果进行比较。在此基础上,建立了修正方程,并通过模拟各种间隙累及模式,确定了变异程度。结果分析了80例患者的图像,其中大多数为女性,诊断为系统性硬化症。结果发现,与基底区和右侧区相比,根尖区(切片1和2)和左侧肺面积之和分别小20%。不同受累成分的模拟发现,顶端或左侧疾病高估了5%至10%的延伸,特别是当它超过15%时。第三个切片被确定为最接近平均值的区域,并在此基础上设计了一个修正公式。结论Goh法不正确地假设肺面积相等,影响计算。为此,我们提出一个数学修正。
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引用次数: 0
Blau syndrome as a cause of fever of unknown origin in adults: A case report Blau综合征作为成人不明原因发热的病因:1例报告
Pub Date : 2025-10-01 DOI: 10.1016/j.rcreue.2025.06.002
Edgar Santiago Castro Prieto , Carlos Mauricio Martínez Montalvo , Sandra Ximena Ramírez , Valentina Ramírez Vega , Carlos Ernesto Artega Unigarro , Cristian Ivan Aparicio Neisa

Introduction

We present the first case reported in Colombia of Blau syndrome manifested in adults associated with a de novo mutation in the NOD2 gene in the context of a case of fever of unknown origin.

Case summary

44-year-old female patient presenting with a condition of approximately 4 years of evolution consisting of cyclical episodes of quantified fever lasting approximately 20 days, with remission intervals of 6–8 months, accompanied by generalized abdominal pain, polymyalgia, polyarthralgia, and general discomfort. Her medical history included treated liver tuberculosis (TB), anterior uveitis, appearance of erythema nodosum in the lower limbs with spontaneous remission, and an episode of peripheral facial paralysis. During the aetiological studies, hepatic granulomas were documented, which were taken to biopsy where multiple non-caseating granulomas were found. Angiotensin-converting enzyme levels were measured, which were found within normal limits, and molecular and microbiological limits for tuberculosis in the biopsy were negative. Subsequently, an autoinflammatory syndrome was considered a suspected diagnostic diagnosis given the persistence of the condition, so genetic studies were performed where a de novo heterozygous mutation was detected in the NOD2 gene, which is associated with Blau syndrome.

Conclusions

Autoinflammatory syndromes, although they occur mostly in childhood, should not be ruled out in adults. In our country there are no known cases of Blau syndrome manifesting in adulthood, so this case report will help us inform the scientific community about it.
我们报告了哥伦比亚报告的第一例Blau综合征,在成人中表现为NOD2基因的新生突变,并伴有不明原因发热。病例总结:44岁女性患者,病情发展约4年,包括持续约20天的周期性定量发热,缓解间隔为6-8个月,伴有全身腹痛、多肌痛、多关节痛和全身不适。她的病史包括治疗过的肝结核、葡萄膜前炎、下肢结节性红斑的出现并自行缓解,以及周围性面瘫的发作。在病因学研究中,肝肉芽肿被记录下来,活检时发现了多个非干酪化肉芽肿。测量血管紧张素转换酶水平,发现其在正常范围内,活检中结核的分子和微生物限度为阴性。随后,考虑到病情的持续性,自体炎症综合征被认为是一种可疑的诊断诊断,因此进行了遗传学研究,在NOD2基因中检测到新生杂合突变,这与Blau综合征有关。结论自身炎症综合征虽然多发生于儿童期,但不应排除其在成人中的发生。在我国还没有已知的成年期出现布劳综合征的病例,因此本病例报告将有助于我们向科学界通报这一情况。
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引用次数: 0
期刊
Revista Colombiana de Reumatología (English Edition)
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