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A retrospective study of neonatal and pregnancy outcomes in pregnant women suffering from inflammatory arthropathy treated with adalimumab 阿达木单抗治疗炎症性关节病孕妇新生儿和妊娠结局的回顾性研究
Pub Date : 2024-07-01 DOI: 10.1016/j.rcreue.2024.07.003
Navid Najarpour, Elham Rajaei, Karim Mowla, Alireza Ghanbaran

Introduction

Anti-tumor necrosis factor-alpha (TNF-α) treatments have been available for over two decades to treat inflammatory arthropathies (IA). Most of these disorders are common among women of reproductive age, which emphasizes the need to evaluate their safety in pregnancy.

Objective

This study aims to scrutinize neonatal and pregnancy outcomes in pregnant IA patients treated with adalimumab.

Materials and methods

The current cross-sectional work was conducted by reviewing the medical files of pregnant IA patients (n = 30) receiving adalimumab referred to Golestan Hospital in Ahvaz (Iran) from 2014 to 2017, followed by extracting demographic profiles as well as neonatal and pregnancy outcomes.

Results

Noteworthy among the findings were PsA (n = 13), RA (n = 5), IBD (n = 4), AS (n = 3), uveitis (n = 2), Behcet's disease (n = 2), and panuveitis (n = 1). The mean age of subjects, duration of illness, and duration of treatment were estimated at 29.53 ± 5.88, 2.85 ± 1.15, and 1.96 ± .90 years, respectively. No delivery outcome was found for 27 (90%) cases, and delivery outcomes observed in three (10%) patients were abortion (n = 2) and preterm complications (n = 1). No neonatal complication was found for 28 (93.3%) cases and neonatal IUGR outcome was reported in 2 (6.7%) cases. Cesarean section was a delivery method in 7 (23.3%) cases and natural method in 21 (70%) cases. There were no significant differences for the prevalence of cesarean section and neonatal outcomes based on the type of disease, but differences were observed for the outcome of delivery based on the type of disease.

Conclusion

According to our findings, definitive conclusions on the safety of adalimumab during pregnancy were impossible and there is a need for further research with a larger sample size.

导言抗肿瘤坏死因子α(TNF-α)治疗炎症性关节病(IA)已有二十多年的历史。这些疾病大多常见于育龄妇女,因此有必要对其在妊娠期的安全性进行评估。本研究旨在仔细研究接受阿达木单抗治疗的妊娠期炎症性关节病患者的新生儿和妊娠结局。材料和方法本次横断面研究通过回顾2014年至2017年期间转诊至伊朗阿瓦士戈勒斯坦医院接受阿达木单抗治疗的妊娠IA患者(n = 30)的医疗档案,然后提取人口统计学特征以及新生儿和妊娠结局。结果值得注意的是PsA(n = 13)、RA(n = 5)、IBD(n = 4)、AS(n = 3)、葡萄膜炎(n = 2)、白塞氏病(n = 2)和泛葡萄膜炎(n = 1)。受试者的平均年龄、病程和治疗时间分别为(29.53 ± 5.88)年、(2.85 ± 1.15)年和(1.96 ± 0.90)年。27例(90%)患者未发现分娩结果,3例(10%)患者的分娩结果为流产(2例)和早产并发症(1例)。28例(93.3%)未发现新生儿并发症,2例(6.7%)报告了新生儿IUGR结果。7例(23.3%)采用剖宫产,21例(70%)采用自然分娩。根据疾病类型,剖宫产率和新生儿结局无明显差异,但根据疾病类型,分娩结局存在差异。
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引用次数: 0
Haematological complication with pulmonary impact in a patient with Sjögren’s syndrome 一名斯约格伦综合征患者的血液并发症对肺部的影响
Pub Date : 2024-04-01 DOI: 10.1016/j.rcreue.2023.02.013
Laura Gallego , Jhon Buitrago , Diana Guavita-Navarro , Jairo Cajamarca-Barón , Ana María Arredondo , José Fernando Polo Nieto , Juan Pablo Castañeda-González , Alejandro Escobar

Sjögren’s Syndrome (SS) is an autoimmune pathology with glandular and/or extraglandular compromise, secondary to the infiltration of lymphoid cells. The clinical course varies depending on genetic susceptibility, comorbidities, patient’s age, and environmental risk factors. Lymphoid proliferation and differentiation are key factors in the progression of SS to haematological malignancies or amyloidosis. Amyloidosis is a secondary entity to the aberrant accumulation of soluble plasma proteins, derived from chronic infectious, inflammatory, neoplastic and haematolymphoid processes. The clinical manifestations vary and depend on the constitutive protein and the age of the patient; and may have glandular or extraglandular, local, or systemic compromise. Among the affected organs, pulmonary involvement poses a diagnostic and therapeutic challenge due to its variable course and clinical manifestation. The following is a case report of a woman over 70 years old, with SS and amyloidosis with glandular and extra glandular manifestations at pulmonary level.

斯约格伦综合征(SS)是一种继发于淋巴细胞浸润的腺体和/或腺外损害的自身免疫性疾病。临床病程因遗传易感性、合并症、患者年龄和环境风险因素而异。淋巴细胞的增殖和分化是 SS 演变为血液恶性肿瘤或淀粉样变性疾病的关键因素。淀粉样变性是慢性感染、炎症、肿瘤和血液淋巴过程中产生的可溶性血浆蛋白异常积累的继发性疾病。淀粉样变性的临床表现各不相同,取决于构成蛋白和患者的年龄;可能有腺体或腺外、局部或全身损害。在受影响的器官中,肺部受累因其病程和临床表现多变而成为诊断和治疗的难题。以下病例报告了一名 70 多岁的女性患者,她患有 SS 和淀粉样变性,在肺部有腺体和腺体外表现。
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引用次数: 0
Outcomes with the use of rituximab in patients with refractory lupus nephritis in a Colombian cohort 哥伦比亚队列中难治性狼疮肾炎患者使用利妥昔单抗的疗效
Pub Date : 2024-04-01 DOI: 10.1016/j.rcreue.2022.07.006

Introduction/Objective

To describe the safety and response to treatment with RTX, estimating its impact on the health state utility (HSU) of patients with refractory lupus nephritis (LN) treated in referral centres in several cities in Colombia.

Materials and methods

A registry-based follow-up study. Patients aged between 16 and 75 years, who were refractory to first-line management and had ISN / RPS class III-IV (+/- V) LN, were included. Our primary outcome was total or partial response to treatment; secondary outcomes were HSU measured with the EQ-5D-3 L, and safety of treatment with RTX. The impact analysis of response to RTX on HSU were performed by mean difference estimated by robust regression.

Results

Forty-six patients (44 women) were included, with a median age of 34 years (IQR = 13), the median SDI was 1 (IQR = 1) and the median activity measured by SLEDAI was 4.5 (IQR = 5.9). Response to RTX was observed in 27 (58.7%) patients. Adjusted for SLEDAI and co-interventions, the patients who responded to RTX obtained a higher mean HSU by 0.162 (95% CI 0.006–0.317). Which is equivalent to 1.9 (95% CI 0.2–3.8) more months lived in ideal health conditions for each year with refractory LN. In 54.3% of the patients, RTX had adequate safety.

Conclusion

From the patient's perspective, the response to treatment with RTX in patients with refractory LN implies a significant impact on their quality of life.

引言/目的描述在哥伦比亚多个城市的转诊中心接受治疗的难治性狼疮性肾炎(LN)患者使用 RTX 治疗的安全性和反应,并估计其对健康状态效用(HSU)的影响。研究对象包括年龄在 16 至 75 岁之间、对一线治疗无效且患有 ISN / RPS III-IV 级(+/- V 级)LN 的患者。我们的主要结果是对治疗的全部或部分反应;次要结果是用 EQ-5D-3 L 测量的 HSU 以及 RTX 治疗的安全性。结果纳入了 46 名患者(44 名女性),中位年龄为 34 岁(IQR = 13),中位 SDI 为 1(IQR = 1),SLEDAI 测量的中位活动度为 4.5(IQR = 5.9)。27例(58.7%)患者对RTX有反应。根据SLEDAI和联合干预调整后,对RTX有反应的患者的平均HSU提高了0.162(95% CI 0.006-0.317)。这相当于难治性 LN 患者每多活一年,就能在理想的健康状况下多活 1.9 个月(95% CI 0.2-3.8)。结论从患者的角度来看,难治性 LN 患者对 RTX 治疗的反应意味着他们的生活质量会受到显著影响。
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引用次数: 0
Dose tapering of biologic therapy in psoriasis. Is this achievable? 银屑病生物疗法的剂量递减。可以实现吗?
Pub Date : 2024-04-01 DOI: 10.1016/j.rcreue.2024.05.005
Juan Raúl Castro Ayarza , Manuel Darío Franco-Franco
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引用次数: 0
Interstitial lung disease in autoimmune diseases 自身免疫性疾病中的间质性肺病
Pub Date : 2024-04-01 DOI: 10.1016/j.rcreue.2023.12.002
Vivek Nagaraja , Isabel Mira-Avendano , Alejandro Diaz-Arumir , Michael Gotway , Ana C. Zamora

Approximately forty percent of patients with autoimmune diseases suffer from interstitial lung disease (ILD). There are currently no specific screening guidelines for these patients. ILD causes substantial morbidity and mortality; early recognition and diagnosis are essential to avoid treatment delays. The gold standard for management incorporates a multidisciplinary approach (MMD) with input from various specialties, such as pulmonary, rheumatology, radiology, and pathology, to reach a consensus regarding diagnosis and treatment. In this article, we will discuss the most common forms of ILD that affect patients with autoimmune diseases, as well as how to promptly and effectively diagnose and treat these conditions.

约有 40% 的自身免疫性疾病患者患有间质性肺病 (ILD)。目前还没有针对这些患者的具体筛查指南。间质性肺病会导致严重的发病率和死亡率;为避免延误治疗,早期识别和诊断至关重要。治疗的黄金标准是采用多学科方法 (MMD),由肺科、风湿免疫科、放射科和病理科等多个专科共同参与,就诊断和治疗达成共识。在本文中,我们将讨论影响自身免疫性疾病患者的最常见 ILD 形式,以及如何及时有效地诊断和治疗这些疾病。
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引用次数: 0
SARS-CoV-2 and post-COVID-19 syndrome in paediatric rheumatology: A scoping review 儿童风湿病学中的 SARS-CoV-2 和后 COVID-19 综合征:范围审查
Pub Date : 2024-04-01 DOI: 10.1016/j.rcreue.2024.06.001
Eduardo Tuta Quintero , Angela C. Mosquera Pongutá , Erika Alexandra Barroso da Silva , Juan Olivella , Andrea Alexandra Silvera , Camila Aragón , Lorena Vásquez , Estefanía Collazos , Karla Olivares Vigles , Karol Martínez , Mateo León Machicado , Yeimy N. Díaz Pérez , Juan Pimentel

An increasing number of musculoskeletal clinical manifestations, the onset of diseases and rheumatological manifestations have been seen in the paediatric population surviving COVID-19, however, the medical literature on the subject is limited.

The objective is to explore the available evidence on musculoskeletal symptoms and autoimmune diseases in the paediatric population with post-COVID syndrome.

Scoping systematic review in PubMed and Scopus through search strategies. Observational and experimental studies are included in populations under 21 years of age with and without autoimmune diseases, without time limit in English and Spanish.

The 28 documents included: case reports (n = 6), cross-sectional studies (n = 5), prospective cohort studies (n = 5), retrospective cohort (n = 6), case series (n = 6), ambidirectional section (n = 1), randomized controlled trial (n = 1), and longitudinal section study (n = 1). The total study population was 56,738 patients. The most frequent symptoms presented in long COVID-19 are myalgias and arthralgias. The evidence showing a relationship between SARS-CoV-2 infection in the development of musculoskeletal symptoms and autoimmune diseases in the convalescent period is limited.

As a conclusion, myalgias and arthralgias are the most frequent symptoms in long COVID. Patients with SARS-CoV-2 infection and a history of rheumatic disease who are undergoing immunomodulatory treatment do not have a dangerous risk of developing severe presentations and/or complications of the disease.

在 COVID-19 存活的儿科人群中,出现了越来越多的肌肉骨骼临床表现、疾病发病和风湿病表现,然而,有关该主题的医学文献却很有限。本研究旨在探索有关 COVID 后综合征儿科人群中肌肉骨骼症状和自身免疫性疾病的现有证据。28篇文献包括:病例报告(6篇)、横断面研究(5篇)、前瞻性队列研究(5篇)、回顾性队列研究(6篇)、病例系列研究(6篇)、双向切面研究(1篇)、随机对照试验(1篇)和纵向切面研究(1篇)。研究总人数为 56 738 人。长 COVID-19 中最常见的症状是肌痛和关节痛。有证据表明,SARS-CoV-2 感染与康复期肌肉骨骼症状和自身免疫性疾病的发展之间的关系是有限的。正在接受免疫调节治疗的 SARS-CoV-2 感染者和有风湿病史的患者不会有出现严重症状和/或并发症的危险。
{"title":"SARS-CoV-2 and post-COVID-19 syndrome in paediatric rheumatology: A scoping review","authors":"Eduardo Tuta Quintero ,&nbsp;Angela C. Mosquera Pongutá ,&nbsp;Erika Alexandra Barroso da Silva ,&nbsp;Juan Olivella ,&nbsp;Andrea Alexandra Silvera ,&nbsp;Camila Aragón ,&nbsp;Lorena Vásquez ,&nbsp;Estefanía Collazos ,&nbsp;Karla Olivares Vigles ,&nbsp;Karol Martínez ,&nbsp;Mateo León Machicado ,&nbsp;Yeimy N. Díaz Pérez ,&nbsp;Juan Pimentel","doi":"10.1016/j.rcreue.2024.06.001","DOIUrl":"10.1016/j.rcreue.2024.06.001","url":null,"abstract":"<div><p>An increasing number of musculoskeletal clinical manifestations, the onset of diseases and rheumatological manifestations have been seen in the paediatric population surviving COVID-19, however, the medical literature on the subject is limited.</p><p>The objective is to explore the available evidence on musculoskeletal symptoms and autoimmune diseases in the paediatric population with post-COVID syndrome.</p><p>Scoping systematic review in PubMed and Scopus through search strategies. Observational and experimental studies are included in populations under 21 years of age with and without autoimmune diseases, without time limit in English and Spanish.</p><p>The 28 documents included: case reports (n = 6), cross-sectional studies (n = 5), prospective cohort studies (n = 5), retrospective cohort (n = 6), case series (n = 6), ambidirectional section (n = 1), randomized controlled trial (n = 1), and longitudinal section study (n = 1). The total study population was 56,738 patients. The most frequent symptoms presented in long COVID-19 are myalgias and arthralgias. The evidence showing a relationship between SARS-CoV-2 infection in the development of musculoskeletal symptoms and autoimmune diseases in the convalescent period is limited.</p><p>As a conclusion, myalgias and arthralgias are the most frequent symptoms in long COVID. Patients with SARS-CoV-2 infection and a history of rheumatic disease who are undergoing immunomodulatory treatment do not have a dangerous risk of developing severe presentations and/or complications of the disease.</p></div>","PeriodicalId":101099,"journal":{"name":"Revista Colombiana de Reumatología (English Edition)","volume":"31 2","pages":"Pages 223-233"},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141636783","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
Interstitial lung disease in systemic lupus erythematosus: State of the art search 系统性红斑狼疮的间质性肺病:最新研究成果
Pub Date : 2024-04-01 DOI: 10.1016/j.rcreue.2022.10.006
Juan Camilo Santacruz, Marta Juliana Mantilla, Gustavo Rodríguez-Salas, Igor Rueda, Ana María Santos, John Londoño

Interstitial lung disease in lupus is an entity that occurs infrequently and tends to progress slowly in most cases. Despite this, the therapeutic approach for moderate to severe cases is largely unknown because most of the evidence comes from case reports, many of which predate the advent of today’s known treatments for lupus. Additionally, little progress has been made in understanding its pathophysiology and current concepts come from other connective tissue diseases such as systemic sclerosis or are grouped within the group of interstitial pneumonias with autoimmune characteristics. This, to an extent, has been an obstacle for research in this field, and to date there is no unified diagnostic and therapeutic approach. Therefore we conducted a state-of-the-art search of the best evidence available to date, in terms of diagnostic methods and emerging therapies, to offer the clinician a practical vision for a comprehensive approach.

狼疮间质性肺病是一种不常发生的疾病,在大多数病例中往往进展缓慢。尽管如此,中度至重度病例的治疗方法在很大程度上仍是未知数,因为大多数证据都来自病例报告,其中许多都是在当今已知的狼疮治疗方法出现之前。此外,人们对狼疮病理生理学的认识进展甚微,目前的概念来自于其他结缔组织疾病(如系统性硬化症)或具有自身免疫特征的间质性肺炎。这在一定程度上阻碍了该领域的研究,迄今为止还没有统一的诊断和治疗方法。因此,我们从诊断方法和新兴疗法两方面对迄今为止的最佳证据进行了一次最先进的搜索,以便为临床医生提供一种切实可行的综合方法。
{"title":"Interstitial lung disease in systemic lupus erythematosus: State of the art search","authors":"Juan Camilo Santacruz,&nbsp;Marta Juliana Mantilla,&nbsp;Gustavo Rodríguez-Salas,&nbsp;Igor Rueda,&nbsp;Ana María Santos,&nbsp;John Londoño","doi":"10.1016/j.rcreue.2022.10.006","DOIUrl":"10.1016/j.rcreue.2022.10.006","url":null,"abstract":"<div><p>Interstitial lung disease in lupus is an entity that occurs infrequently and tends to progress slowly in most cases. Despite this, the therapeutic approach for moderate to severe cases is largely unknown because most of the evidence comes from case reports, many of which predate the advent of today’s known treatments for lupus. Additionally, little progress has been made in understanding its pathophysiology and current concepts come from other connective tissue diseases such as systemic sclerosis or are grouped within the group of interstitial pneumonias with autoimmune characteristics. This, to an extent, has been an obstacle for research in this field, and to date there is no unified diagnostic and therapeutic approach. Therefore we conducted a state-of-the-art search of the best evidence available to date, in terms of diagnostic methods and emerging therapies, to offer the clinician a practical vision for a comprehensive approach.</p></div>","PeriodicalId":101099,"journal":{"name":"Revista Colombiana de Reumatología (English Edition)","volume":"31 2","pages":"Pages 234-242"},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141636785","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
Is it possible to optimize biologic therapy in patients with psoriasis? 能否优化银屑病患者的生物疗法?
Pub Date : 2024-04-01 DOI: 10.1016/j.rcreue.2022.10.005
Ángela María Londoño-García , María Fernanda Suárez-Giraldo , Lina María Colmenares-Roldán , Juliana Madrigal-Cadavid , Jorge Estrada , Paulo Giraldo , Daniel Jaramillo-Arroyave

Introduction/Objective

The purpose of this study was to evaluate patients in clinical remission of psoriasis for at least for one year, who maintained therapeutic goals after initiating optimization of biologic therapy.

Materials and methods

A descriptive, observational study was conducted on patients with a diagnosis of moderate–severe psoriasis in treatment with biologic therapy who were started on optimization of biologic therapy.

Results

A total of 29 patients started therapeutic optimization, of these, 27 patients were in the target range with absolute PASI less than 3. Only one patient failed therapeutic optimization with final PASI 3.6 and there was a case of a patient who lost continuity of management due to an accident and had a final PASI 3.8. Most of the patients were male, with an average age of 53 years, married, employed, residing in urban areas, with psoriasis of more than ten years of evolution, without associated morbidities, and without previous biologic treatment, the most frequently used being etanercept and adalimumab.

Conclusion

Optimizing biologic therapy in patients with moderate–severe psoriasis may be viable. We seek to share this experience to propose a protocol to reduce the possibility of adverse events due to the prolonged use of this type of therapy, preserving clinical response and reducing costs to the health system.

导言/目的本研究的目的是评估银屑病临床缓解至少一年的患者在开始优化生物疗法后是否保持了治疗目标。只有一名患者治疗优化失败,最终 PASI 为 3.6,还有一名患者因意外失去了治疗的连续性,最终 PASI 为 3.8。大多数患者为男性,平均年龄 53 岁,已婚,有工作,居住在城市地区,银屑病病程超过 10 年,无相关疾病,既往未接受过生物制剂治疗,最常用的是依那西普(etanercept)和阿达木单抗。我们希望分享这一经验,提出一种方案,以减少因长期使用这种疗法而出现不良反应的可能性,保持临床反应,降低医疗系统的成本。
{"title":"Is it possible to optimize biologic therapy in patients with psoriasis?","authors":"Ángela María Londoño-García ,&nbsp;María Fernanda Suárez-Giraldo ,&nbsp;Lina María Colmenares-Roldán ,&nbsp;Juliana Madrigal-Cadavid ,&nbsp;Jorge Estrada ,&nbsp;Paulo Giraldo ,&nbsp;Daniel Jaramillo-Arroyave","doi":"10.1016/j.rcreue.2022.10.005","DOIUrl":"10.1016/j.rcreue.2022.10.005","url":null,"abstract":"<div><h3>Introduction/Objective</h3><p>The purpose of this study was to evaluate patients in clinical remission of psoriasis for at least for one year, who maintained therapeutic goals after initiating optimization of biologic therapy.</p></div><div><h3>Materials and methods</h3><p>A descriptive, observational study was conducted on patients with a diagnosis of moderate–severe psoriasis in treatment with biologic therapy who were started on optimization of biologic therapy.</p></div><div><h3>Results</h3><p>A total of 29 patients started therapeutic optimization, of these, 27 patients were in the target range with absolute PASI less than 3. Only one patient failed therapeutic optimization with final PASI 3.6 and there was a case of a patient who lost continuity of management due to an accident and had a final PASI 3.8. Most of the patients were male, with an average age of 53 years, married, employed, residing in urban areas, with psoriasis of more than ten years of evolution, without associated morbidities, and without previous biologic treatment, the most frequently used being etanercept and adalimumab.</p></div><div><h3>Conclusion</h3><p>Optimizing biologic therapy in patients with moderate–severe psoriasis may be viable. We seek to share this experience to propose a protocol to reduce the possibility of adverse events due to the prolonged use of this type of therapy, preserving clinical response and reducing costs to the health system.</p></div>","PeriodicalId":101099,"journal":{"name":"Revista Colombiana de Reumatología (English Edition)","volume":"31 2","pages":"Pages 166-170"},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141638632","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
Scleromyxoedema with extracutaneous pulmonary manifestation: A case report and review of the literature 伴有皮外肺部表现的硬肌水肿:病例报告和文献综述
Pub Date : 2024-04-01 DOI: 10.1016/j.rcreue.2024.02.001

Scleromyxoedema is a cutaneous fibromucinosis of unknown aetiology. It is associated with haematological dyscrasias and quite diverse manifestations. Pulmonary vascular involvement is rare and requires a differential diagnosis approach with systemic sclerosis. The case of a patient with scleromyxoedema with an extracutaneous pulmonary manifestation is described.

硬化性粘液性水肿是一种病因不明的皮肤纤维瘤病。它与血液学异常和多种多样的表现有关。肺血管受累很少见,需要与系统性硬化症进行鉴别诊断。本病例描述了一名伴有皮外肺部表现的硬肌水肿患者。
{"title":"Scleromyxoedema with extracutaneous pulmonary manifestation: A case report and review of the literature","authors":"","doi":"10.1016/j.rcreue.2024.02.001","DOIUrl":"10.1016/j.rcreue.2024.02.001","url":null,"abstract":"<div><p>Scleromyxoedema is a cutaneous fibromucinosis of unknown aetiology. It is associated with haematological dyscrasias and quite diverse manifestations. Pulmonary vascular involvement is rare and requires a differential diagnosis approach with systemic sclerosis. The case of a patient with scleromyxoedema with an extracutaneous pulmonary manifestation is described.</p></div>","PeriodicalId":101099,"journal":{"name":"Revista Colombiana de Reumatología (English Edition)","volume":"31 2","pages":"Pages 243-248"},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141399920","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
Antibodies related to the presence, and putatively, development of interstitial lung disease in patients with anti-synthetase syndrome: A systematic literature review and meta-analysis 与抗合成酶综合征患者间质性肺病的存在和发展有关的抗体:系统文献综述和荟萃分析
Pub Date : 2024-04-01 DOI: 10.1016/j.rcreue.2024.05.003
Alejandra García-Rueda , María Paula Uchima-Vera , Jorge Bruce Florez-Suarez , Olga Milena García , Gerardo Quintana-López

Introduction

Anti-synthetase syndrome is a recently characterized entity whose morbidity and mortality are mainly determined by interstitial lung involvement. For this reason, it is considered important to identify the association between the presence of anti-synthetase antibodies and the presence and putatively, the development of a specific radiological pattern of interstitial lung disease.

Objective

To determine the association between the antibodies present at the time of diagnosis of anti-synthetase syndrome and the presence of interstitial lung disease.

Materials and methods

Systematic review of the literature and meta-analysis. The search strategy was carried out in: EMBASE, LILACS, PUBMED, CENTRAL (Cochrane), and Grey Literature. The primary outcomes were the detection of the different radiological patterns of interstitial lung disease, and the reported specific anti-synthetase antibody.

Results

One hundred seventy-six patients were identified; Jo-1 in combination with NSIP was the most frequent pattern. Quantitative analysis suggests that PL-7 expression is associated with the presence of UIP and NSIP. For obstructive pneumonitis, a relationship was observed with the presence of anti EJ, while the expression of PL-7 was negatively associated. Also, EJ had a negative association with the presence of NSIP. The observed associations were corroborated with the subgroup analysis carried out using the two retrospective observational studies identified.

Conclusion

Despite the limitations, PL-7 and EJ showed significant associations with the presence of specific patterns of interstitial lung disease. Jo-1 did not have a significant specific association. Studies of higher methodological quality are required to generate recommendations that affect clinical practice.

导言抗合成酶综合征是最近才出现的一种疾病,其发病率和死亡率主要取决于肺间质受累。因此,确定抗合成酶抗体的存在与间质性肺疾病的存在和可能的特定放射学模式的发展之间的关系非常重要。材料与方法系统回顾文献并进行荟萃分析。检索策略包括EMBASE、LILACS、PUBMED、CENTRAL(Cochrane)和灰色文献。主要结果是检测间质性肺病的不同放射学模式,以及报告的特异性抗合成酶抗体。定量分析表明,PL-7 的表达与 UIP 和 NSIP 的存在有关。就阻塞性肺炎而言,抗 EJ 的存在与 PL-7 的表达呈负相关。此外,EJ 与 NSIP 的存在也呈负相关。结论尽管存在局限性,但 PL-7 和 EJ 与间质性肺病的特定模式存在显著关联。Jo-1没有明显的特异性关联。要提出影响临床实践的建议,需要进行方法学质量更高的研究。
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引用次数: 0
期刊
Revista Colombiana de Reumatología (English Edition)
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