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Systemic sclerosis associated myopathy: how to treat. 系统性硬化症相关肌病:如何治疗?
Q4 Medicine Pub Date : 2023-12-01 Epub Date: 2023-07-19 DOI: 10.1007/s40674-023-00206-y
A Selva-O'Callaghan, A Guillen-Del-Castillo, A Gil-Vila, E Trallero-Araguás, A Matas-García, J C Milisenda, I Pinal-Fernández, C Simeón-Aznar

Purpose of review: Systemic sclerosis (SSc) and myositis are two different entities that may coexist as an overlap syndrome. Immunological biomarkers such as anti-PM/Scl or anti-Ku reinforce the syndrome. This review is focused on the treatment of different and characteristic manifestations of this syndrome.

Recent findings: Among the different phenotypes of muscle involvement in patients with SSc, the fibrotic pattern and the sporadic inclusion body myositis must be identified early to avoid a futile immunosuppressive treatment. Other forms such as dermatomyositis, non-specific myositis and immune-mediated necrotizing myopathy need to receive conventional immunosuppressive therapy considering that high dose of glucocorticoids may induce a scleroderma renal crisis in patients with SSc. Physicians must be aware of the existence of a "double trouble" association of hereditary myopathy with an autoimmune phenomenon. Several autoantibodies, mainly anti-PM/Scl and anti-Ku may help to define specific phenotypes with characteristic clinical manifestations that need a more specific therapy. Vasculopathy is one of the underlying mechanisms that link SSc and myositis. Recent advances in this topic are reviewed.

Summary: Current treatment of SSc associated myopathy must be tailored to specific organs involved. Identifying the specific clinical, pathological, and immunological phenotypes may help to take the correct therapeutic decisions.

综述的目的:系统性硬化症(SSc)和肌炎是两种不同的疾病,可能作为一种重叠综合征并存。抗PM/Scl或抗Ku等免疫学生物标志物强化了这一综合征。本综述将重点讨论该综合征不同特征表现的治疗方法:在 SSc 患者肌肉受累的不同表型中,纤维化模式和散发性包涵体肌炎必须及早识别,以避免徒劳的免疫抑制治疗。考虑到大剂量糖皮质激素可能会诱发SSc患者的硬皮病肾危象,因此其他类型的肌炎,如皮肌炎、非特异性肌炎和免疫介导的坏死性肌病需要接受常规免疫抑制治疗。医生必须意识到遗传性肌病与自身免疫现象之间存在 "双重麻烦"。几种自身抗体,主要是抗PM/Scl和抗Ku抗体,可帮助确定具有特征性临床表现的特定表型,从而需要更有针对性的治疗。血管病变是联系 SSc 和肌炎的潜在机制之一。小结:目前对 SSc 相关肌病的治疗必须针对所涉及的特定器官。确定特定的临床、病理和免疫表型有助于做出正确的治疗决定。
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引用次数: 0
Axillary lymphadenopathy in a high-risk breast screening patient following the COVID-19 vaccine: a diagnostic conundrum. 一名接种 COVID-19 疫苗后接受乳腺筛查的高危患者出现腋窝淋巴结病:诊断难题。
IF 0.6 Q4 Medicine Pub Date : 2022-03-09 eCollection Date: 2022-03-01 DOI: 10.1259/bjrcr.20210063
Besma Musaddaq, Adam Brown, Sam Dluzewski, Teresa Marafioti, Anmol Malhotra

A number of COVID-19 vaccines have been approved worldwide to help tackle the pandemic. As with many vaccines, this causes a reactive axillary lymphadenopathy which can mimic potentially metastatic disease in a breast screening patient. It is therefore important to be aware of this side-effect of the vaccination when evaluating the axilla in a breast screening patient. We present a case of biopsy-proven unilateral reactive axillary lymphadenopathy in a high risk BRCA carrier following administration of the Astra Zeneca vaccine.

全球已批准了多种 COVID-19 疫苗,以帮助应对这一流行病。与许多疫苗一样,这种疫苗会引起反应性腋窝淋巴结病,这可能是乳腺筛查患者潜在转移性疾病的假象。因此,在对乳腺筛查患者的腋窝进行评估时,必须注意疫苗接种的这种副作用。我们介绍了一例经活检证实的单侧反应性腋窝淋巴结病例,患者是一名高风险 BRCA 携带者,在接种阿斯利康疫苗后出现该症状。
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引用次数: 0
Corticosteroids for COVID-19-Associated ARDS 皮质类固醇治疗COVID-19相关ARDS
Q4 Medicine Pub Date : 2020-11-01 DOI: 10.1097/cpm.0000000000000381
M. Marmor, A. Jonas
Systemic corticosteroids have emerged as a possible therapy to mitigate lung injury in severe COVID-19 infection Here, we provide historical context for corticosteroid administration in acute respiratory failure due to viral infection and review existing data for the use of systemic corticosteroids for SARS-CoV-2 infection The results of these limited data consistently suggest a mortality benefit for patients with COVID-19-associated acute respiratory distress syndrome with no existing evidence to suggest harm © 2020 Lippincott Williams and Wilkins All rights reserved
系统性皮质类固醇已成为缓解严重新冠肺炎感染肺损伤的可能疗法,我们提供了因病毒感染导致急性呼吸衰竭时使用皮质类固醇的历史背景,并回顾了系统性皮质类固醇治疗SARS-CoV-2感染的现有数据。这些有限数据的结果一致表明,COVID-19相关急性呼吸窘迫综合征患者的死亡率有益处,但没有现有证据表明其危害©2020 Lippincott Williams and Wilkins版权所有
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引用次数: 4
The Complex Relationship Between Poor Sleep Quality and Chronic Obstructive Pulmonary Disease 睡眠质量差与慢性阻塞性肺疾病的复杂关系
Q4 Medicine Pub Date : 2020-11-01 DOI: 10.1097/CPM.0000000000000384
Nicholas Hogan, A. Cypro, A. Malhotra
Sleep-related symptoms are prevalent among patients with chronic obstructive pulmonary disease (COPD). The disease process often manifests with nocturnal respiratory symptoms. Long-acting antimuscarinic medications improve nocturnal COPD symptoms, though their effect on sleep quality requires further investigation. Those with COPD often suffer from comorbidities that negatively impact sleep, including obstructive sleep apnea (OSA) and mood disorders such as anxiety and depression. Sleep quality is also predictive of COPD exacerbations. Patients with concurrent COPD and OSA suffer from overlap syndrome (OVS), characterized by a synergistic effect on poor health outcomes. The intersection of COPD and OSA offers the clinical pulmonary audience a useful lens for ongoing basic, clinical, and translational research. Patients with OVS experience higher mortality compared with either COPD or OSA alone. This observation is attributable to the compound effect each condition has on adverse cardiovascular events. A complex interplay exists between COPD, sleep symptoms, and OSA. COPD appears to influence important nonanatomical contributors to OSA. The presence of underlying COPD makes the definitive diagnosis of OSA a challenge. Chronic noninvasive ventilation (NIV) is the backbone of therapy for OVS, OSA, and hypercarbic COPD. NIV is additionally a well-established treatment for acute COPD exacerbations and emerging research demonstrates that NIV decreases mortality and hospitalizations in patients with hypercarbic COPD. Clinicians often need to individualize therapeutic interventions for patients with COPD, OSA, and OVS, balancing the benefits and adverse effects of such interventions. NIV can have unwanted impact on the quality of life for some patients with COPD. Certain medications used for COPD, such as corticosteroids, have adverse effects on sleep quality. Future therapeutic approaches are needed to improve the sleep symptoms and health outcomes of patients suffering from COPD and OVS.
睡眠相关症状在慢性阻塞性肺疾病(COPD)患者中很普遍。病程常表现为夜间呼吸道症状。长效抗毒蕈碱药物可改善夜间COPD症状,但其对睡眠质量的影响有待进一步研究。慢性阻塞性肺病患者通常患有对睡眠产生负面影响的合并症,包括阻塞性睡眠呼吸暂停(OSA)和焦虑和抑郁等情绪障碍。睡眠质量也是COPD恶化的预测指标。同时患有COPD和OSA的患者患有重叠综合征(OVS),其特征是对不良健康结果的协同效应。COPD和OSA的交叉为临床肺病患者提供了一个有用的视角,用于正在进行的基础、临床和转化研究。与单纯COPD或OSA相比,OVS患者的死亡率更高。这一观察结果可归因于每种情况对不良心血管事件的复合影响。COPD、睡眠症状和OSA之间存在复杂的相互作用。COPD似乎影响OSA的重要非解剖学因素。潜在COPD的存在使得OSA的明确诊断具有挑战性。慢性无创通气(NIV)是OVS, OSA和高碳COPD治疗的支柱。此外,NIV是一种公认的治疗急性COPD加重的方法,新兴研究表明,NIV可以降低高碳COPD患者的死亡率和住院率。临床医生通常需要对COPD、OSA和OVS患者进行个体化治疗干预,平衡这些干预措施的益处和不良影响。NIV会对一些COPD患者的生活质量产生意想不到的影响。某些治疗慢性阻塞性肺病的药物,如皮质类固醇,对睡眠质量有不良影响。未来的治疗方法需要改善COPD和OVS患者的睡眠症状和健康结果。
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引用次数: 0
The Diagnosis of Hypersensitivity Pneumonitis and the Role of Lung Biopsy 超敏性肺炎的诊断及肺活检的作用
Q4 Medicine Pub Date : 2020-11-01 DOI: 10.1097/CPM.0000000000000385
E. Seixas, P. Serra, R. Aguiar, Margarida Ferreira, P. Ferreira
Hypersensitivity pneumonitis (HP) refers to a collective noun of diffuse lung diseases encompassing some degree of bronchiolar and interstitial granulomatous inflammation that results from persistent inhalation exposure and consequent immune sensitization to a large potential diversity of (predominantly) organic antigens in predisposed individuals. In suspected cases of HP, forceps transbronchial lung biopsy (TBLB) has been traditionally performed on a case-by-case basis along with bronchoalveolar lavage. This option has been subject to some debate and its use is more restrained in the presence of a chronic fibrotic form of HP—where surgical lung biopsy is classically recommended in the face of the need for a more reliable differentiation from fibrotic idiopathic interstitial pneumonias. We intended to assess the diagnostic contribution of conventional TBLB in the combined multidisciplinary diagnosis of an HP patient cohort. A retrospective evaluation of all the diagnostic elements and level of confidence from all HP cases followed in an interstitial lung disease ILD outpatient clinic of a district hospital center (Centro Hospitalar do Baixo Vouga), from June 2015 to August 2019, and simultaneously evaluated in a multidisciplinary team discussion of the same hospital, comprising an interstitial lung disease dedicated lung physician, a chest radiologist, 2 rheumatologists, and a pathologist. We identified 78 patients (mean age: 70.5 y, interquartile range: 58.5 to 78.0) with a slight female predominance. Most of the patients (61.5%) had chronic/fibrotic HP. The most frequently identified inducing antigens were avian antigens in 59.0% of cases, followed by molds in 20.5%. Of the 72 patients who underwent bronchofibroscopy, 36.1% (n=26) conventional TBLB performed, predominantly in the segments of the right lower lobe with an average number of 3.9 biopsies (SD±1.4) accomplished per patient. In 50.0% of the cases submitted to TBLB, the biopsies showed representative material with histologic features (definite or supportive) which had some degree of contribution for the diagnostic discussion. Among the patients where TBLBs were not performed or whose results were found to be devoid of significant findings, 73.1% were still diagnosed as HP without the need for surgical video-assisted thoracoscopic lung biopsy/transbronchial lung cryobiopsy (VATS/TBLCB) on the grounds of other diagnostic elements; 15.4% of patients were diagnosed with HP after a VATS/TBLCB procedure. Lastly, around 11.5% of patients were considered to have an unacceptable risk for VATS/TBLCB but, on the basis of clinical, radiologic, and immunologic elements received a multidisciplinary provisional diagnosis still with a reasonable level of confidence. Regarding complications with TBLB, there were 2 cases of moderate bleeding (7.6%) and 1 pneumothorax (3.8%) that did not require drainage. Notwithstanding its limitations, TBLB can still have a role in the diagnostic workup of HP, namely
超敏性肺炎(HP)是指弥漫性肺部疾病的统称,包括一定程度的细支气管和间质性肉芽肿性炎症,这些炎症是由持续吸入暴露和易感个体对大量潜在多样性(主要是)有机抗原的免疫增敏引起的。在疑似HP病例中,传统上是在个案基础上进行钳式经支气管肺活检(TBLB)和支气管肺泡灌洗。这一选择一直存在一些争议,在存在慢性纤维化形式的HP的情况下,它的使用更受限制——在需要更可靠地与纤维化特发性间质性肺炎进行鉴别时,通常建议进行外科肺活检。我们打算评估传统TBLB在HP患者队列的多学科联合诊断中的诊断作用。2015年6月至2019年8月,在地区医院中心(Centro Hospitalar do Baixo Vouga)的间质性肺病ILD门诊对所有HP病例的所有诊断要素和置信水平进行了回顾性评估,并在同一医院的多学科团队讨论中同时进行了评估,包括一名间质性肺病专职肺部医生、一名胸部放射科医生、两名风湿病学家和一名病理学家。我们确定了78名患者(平均年龄:70.5岁) y、 四分位间距:58.5至78.0),女性略占优势。大多数患者(61.5%)患有慢性/纤维化HP。最常见的诱导抗原是59.0%的病例中的禽抗原,其次是20.5%的霉菌。在接受支气管纤维镜检查的72名患者中,36.1%(n=26)的患者进行了常规TBLB,主要在右下叶节段,平均每位患者完成3.9次活组织检查(SD±1.4)。在提交给TBLB的50.0%的病例中,活检显示具有组织学特征(明确或支持性)的代表性材料,这对诊断讨论有一定程度的贡献。在未进行TBLB或其结果没有显著发现的患者中,73.1%的患者仍被诊断为HP,而无需基于其他诊断因素进行手术电视胸腔镜肺活检/经支气管肺冷冻活检(VATS/TBLCB);15.4%的患者在VATS/TBLCB手术后被诊断为HP。最后,约11.5%的患者被认为有不可接受的VATS/TBLCB风险,但根据临床、放射学和免疫学因素,接受了多学科的临时诊断,仍具有合理的置信水平。关于TBLB的并发症,有2例中度出血(7.6%)和1例不需要引流的肺气肿(3.8%)。尽管有局限性,TBLB仍然可以在HP的诊断检查中发挥作用,即在急性/炎症性HP中,为高达50%的病例的多学科讨论增加明确或支持性的组织学信息。TBLB可以以最小的风险增加为代价提高诊断效率,因为它是一种普遍可用的技术,可以与支气管肺泡灌洗一起进行。这具有重要意义,特别是在没有TBLCB的中心,因为大约一半最终被诊断为HP的患者可以避免手术活检。
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引用次数: 0
Vaping-associated Lung Injury Successfully Treated With Pulse Dose Corticosteroids 脉冲剂量皮质类固醇成功治疗电子烟相关肺损伤
Q4 Medicine Pub Date : 2020-11-01 DOI: 10.1097/cpm.0000000000000386
A. Loutfy, S. Rashid, H. Budke, D. Praprotnik, Ajit Chary, Yuhann Lopez, C. Rimmer, K. Geckle, E. Thomas, R. Fadul
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引用次数: 0
Is a Trial of Observation Safer Than Intervention With Spontaneous Pneumothorax? 自发性气胸的观察试验比干预试验更安全吗?
Q4 Medicine Pub Date : 2020-11-01 DOI: 10.1097/cpm.0000000000000376
M. Brandorff, D. Zappetti
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引用次数: 0
Excessive Dynamic Airway Collapse: A COPD/Asthma Mimic or a Treatment-emergent Consequence of Inhaled Corticosteroid Therapy: Case Series and Brief Literature Review 过度动态气道塌陷:COPD/哮喘模拟或吸入皮质类固醇治疗的治疗紧急后果:病例系列和简要文献综述
Q4 Medicine Pub Date : 2020-11-01 DOI: 10.1097/CPM.0000000000000382
S. Heraganahally, Anandpreet S. Ghataura, X. Y. Er, S. Heraganahally, Edwina Biancardi
Excessive dynamic airway collapse (EDAC) is a clinical entity characterized by narrowing of larger airways during tidal breathing. Symptoms of EDAC can be similar to chronic obstructive airway disease (COPD)/asthma and EDAC can coexist with airway disease. More recently widespread use of inhaled corticosteroids (ICS) among patients with COPD/asthma has been implicated for the emergence of EDAC. In this report, we describe 6 adult patients presenting with chronic cough with a background diagnosis of either COPD or asthma on ICS, who were noted to have EDAC. We also made an attempt to briefly review the earlier published reports on EDAC. Our review suggested that EDAC is prevalent among patients with previous diagnosis of COPD/asthma and with ICS use. Female sex, older age, higher body mass index, and presence of gastroesophageal reflux disease (GORD), and chronic upper respiratory tract infections (URTI) may be a risk factor for EDAC. Chronic barking cough and shortness of breath are the common clinical presentation and acute presentation could be triggered by lower respiratory tract infection and episodic presentations can be related to chronic recurrent aspiration secondary to GORD or chronic URTI. Dynamic computed tomography of the chest and bronchoscopy are useful in the diagnosis. Pulmonary function tests could be variable, demonstrating normal, obstructive, or restrictive pattern. Management of EDAC with weight loss strategies, addressing GORD and URTI issues and antibiotics during acute lower respiratory tract infection may be helpful. Noninvasive positive pressure ventilation may be beneficial in some patients. ICS should be used wisely to prevent the emergence of EDAC among patients with chronic airway disease.
过度动态气道塌陷(EDAC)是一种以潮汐呼吸时较大气道狭窄为特征的临床症状。EDAC的症状可能与慢性阻塞性气道疾病(COPD)/哮喘相似,EDAC可与气道疾病共存。最近在COPD/哮喘患者中广泛使用吸入皮质类固醇(ICS)与EDAC的出现有关。在本报告中,我们描述了6例成人慢性咳嗽患者,背景诊断为慢性阻塞性肺病或哮喘,并注意到有EDAC。我们也试着简要回顾了早期发表的关于EDAC的报告。我们的综述表明,EDAC在既往诊断为COPD/哮喘和使用ICS的患者中普遍存在。女性、年龄较大、体重指数较高、存在胃食管反流病(GORD)和慢性上呼吸道感染(URTI)可能是EDAC的危险因素。慢性吠叫性咳嗽和呼吸短促是常见的临床表现,急性表现可由下呼吸道感染引发,发作性表现可与继发于GORD或慢性尿路感染的慢性反复吸入性有关。胸部动态计算机断层扫描和支气管镜检查有助于诊断。肺功能检查可能是可变的,显示正常、阻塞性或限制性。EDAC的管理与减肥策略、解决GORD和URTI问题以及急性下呼吸道感染期间的抗生素可能有所帮助。无创正压通气可能对某些患者有益。应明智地使用ICS,以防止慢性气道疾病患者出现EDAC。
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引用次数: 6
Immune-related Pulmonary Toxicity From Cancer Immunotherapy: A Systematic Approach 来自癌症免疫治疗的免疫相关肺毒性:一个系统的方法
Q4 Medicine Pub Date : 2020-11-01 DOI: 10.1097/CPM.0000000000000383
N. Thiruchelvam, V. Rajasurya, Sivagowri Tharmendira, Heather Torbic, M. Waldron, J. Stoller, D. Culver
Immune checkpoint inhibitors (ICIs) are one of the major advances in cancer treatment. ICIs have shown significant benefit in treating several types of cancer. Currently there are 6 ICIs available in the United States and multiple ICIs in the pipeline. Immune checkpoint signaling leads to immune tolerance of cancer cells through downregulation of T-cell activation. The reversal in tumor-tolerance and self-tolerance effected by ICIs likely drives both T-cell–mediated toxicity and immune-related adverse effects (irAEs); however, the exact mechanism remains not completely understood. Pulmonary irAEs are among the most feared high-grade irAEs leading to discontinuation of ICIs and, not uncommonly, treatment-related death. Because of the high degree of morbidity and mortality associated with pulmonary irAEs and the exponential growth of ICI use, clinicians must increasingly be facile in diagnosing and managing these irAEs.
免疫检查点抑制剂(ICIs)是癌症治疗的主要进展之一。ICIs在治疗几种类型的癌症方面显示出显著的益处。目前在美国有6种ici可用,还有多个ici正在筹备中。免疫检查点信号通过下调t细胞激活导致癌细胞的免疫耐受。ICIs对肿瘤耐受和自身耐受的逆转可能驱动t细胞介导的毒性和免疫相关的不良反应(irAEs);然而,确切的机制尚不完全清楚。肺部irae是最令人担忧的高级别irae之一,可导致中断ICIs治疗,并导致与治疗相关的死亡。由于肺部irae的高发病率和死亡率以及ICI使用的指数增长,临床医生必须越来越容易地诊断和管理这些irae。
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引用次数: 0
Home, Hospice, or Hospital: Where Are Our Patients With Chronic Lung Disease Dying? 家、临终关怀院或医院:我们的慢性肺病患者在哪里死亡?
Q4 Medicine Pub Date : 2020-11-01 DOI: 10.1097/cpm.0000000000000377
Thomas A. Di Vitantonio, E. Lafond, D. Zappetti
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引用次数: 0
期刊
Clinical Pulmonary Medicine
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