Pulmonary sarcoidosis

肺结节病
  • 文章类型: Case Reports
    结节病是一种全身性疾病,其特征在于身体各种器官中肉芽肿性炎症的发展。高钙血症是其表现之一。我们介绍了一个77岁男性的恶性高钙血症病例,在多次评估和组织学确认显示非干酪性肉芽肿后诊断为结节病。在没有病因诊断的情况下,治疗最初是基于水化,利尿剂,和双膦酸盐。糖皮质激素治疗可作为结节病相关高钙血症的标准治疗方法。
    Sarcoidosis is a systemic disorder characterized by the development of granulomatous inflammation in various organs of the body. Hypercalcemia is one of its manifestations. We present a case of malignant hypercalcemia in a 77-year-old man, diagnosed as sarcoidosis following multiple assessments and histological confirmation showing noncaseating granulomas. In the absence of an etiological diagnosis, treatment was initially based on hydration, diuretics, and bisphosphonate. Glucocorticoid therapy can be the standard treatment for hypercalcemia related to sarcoidosis.
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  • 文章类型: Case Reports
    在定期体检中,一名39岁的蒙古地下矿工根据胸部X线检查被诊断出患有矽肺,计算机断层扫描(CT),和工作历史。胸部X线检查显示两侧肺野弥漫性双侧圆形结节,右上带有上叶优势和大的混浊。胸部CT显示右上叶聚集性大改变,左上肺小结节合并。在血液测试中,肺癌标志物神经元特异性烯醇化酶(NSE)的血清水平升高(24.58ng/mL).癌胚抗原(CEA)和细胞角蛋白19片段(CYFRA21-1)水平在参考范围内。在怀疑右上叶肿瘤之后,进行了右上叶切除术.肺标本的组织病理学检查显示许多二氧化硅结节的合并,伴有相关结节病的适应症。组织学特征表明存在两个并发的病理过程:矽肺病和结节病。此病例证明了一名患者诊断出的三种临床状况的组合,包括与结节病相关的复杂矽肺和血清NSE水平升高。此病例报告可作为未来研究的基础,探索NSE作为矽肺标志物的潜力。
    In a periodical medical checkup, a 39-year-old Mongolian underground miner was diagnosed with silicosis based on chest radiography, computed tomography (CT), and work history. Chest radiography showed diffuse bilateral rounded nodules in both lung fields, with upper lobe dominance and large opacities in the right upper zone. Chest CT presented conglomerated massive changes in the right upper lobe and the coalescence of small nodules in the left upper lung. In the blood test, serum levels of the lung cancer marker neuron-specific enolase (NSE) were elevated (24.58 ng/mL). Carcinoembryonic antigen (CEA) and cytokeratin 19 fragment (CYFRA 21-1) levels were within the reference range. Subsequent to the suspicion of a tumour in the right upper lobe, a right upper lobectomy was performed. The histopathological examination of the lung specimen revealed the coalescence of numerous silica nodules, accompanied by indications of associated sarcoidosis. The histological features suggested the presence of two concurrent pathological processes: silicosis and sarcoidosis. This case demonstrated the combination of three clinical conditions diagnosed in one patient, including complicated silicosis associated with sarcoidosis and elevated serum NSE levels. This case report may serve as a foundation for future investigations exploring the potential of NSE as a marker for silicosis.
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  • 文章类型: Case Reports
    本文介绍了一个12岁儿童患有关节炎的罕见结节病病例,双侧腮腺肿大和眼部,但不幸的是,由于缺乏肺部受累而错过了诊断。
    结节病的诊断是排除,有时,这可能是具有挑战性的。本文介绍了一名12岁儿童的罕见结节病病例,伴有双侧腮腺肿大。腮腺肿大之前有肌肉骨骼和眼部受累的迹象,腮腺肿胀持续了3年;但不幸的是,由于罕见的情况,以前的医疗保健专业人员错过了明确的诊断,尤其是缺乏肺部受累。因此,不同医疗保健专业之间的合作对于有效的诊断和管理很重要。尽管它很罕见,当遇到像关节炎这样的多系统实体时,结节病应该总是出现在鉴别诊断列表中,眼部症状和腮腺肿胀。
    UNASSIGNED: This paper presents a rare sarcoidosis case in a child of 12 years of age presented with arthritis, bilateral parotid enlargement and ocular, but unfortunately the diagnosis has been missed due to lack of pulmonary involvement.
    UNASSIGNED: Diagnosis of sarcoidosis is by exclusion, and sometimes, it can be challenging. This paper presents a rare sarcoidosis case in a child of 12 years of age presented with bilateral parotid enlargement. The signs of musculoskeletal and ocular involvement were present before the parotid enlargement, and the parotid swelling persisted for 3 years; but unfortunately the definite diagnosis has been missed by the previous healthcare professionals most probably due to the rarity of the situation, especially lack of pulmonary involvement. Therefore, cooperation between different healthcare specialties is important for an effective diagnosis and management. Despite its rarity, sarcoidosis should always be present in the list of differential diagnosis when encountering multisystem entities like arthritis, ocular symptoms and parotid swelling.
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  • 文章类型: Journal Article
    结节病是一种病因不明的多系统肉芽肿性疾病。它可以存在于许多器官中。肺和胸内淋巴结最常见。肺结节病在亚洲并不常见。然而,由于我国人口众多和支气管镜检查的发展,经皮肺穿刺,和其他医疗技术,肺结节病患者的数量正在上升。肺结节病患者早期无明显症状,后期的临床表现可能因人而异。最终,该疾病进展为危及生命的肺纤维化。因此,肺结节病患者应及时诊断。近年来,肺结节病的影像学特征和血清学生物标志物一直在研究。影像学和血清学生物标志物对肺结节病的诊断价值总结如下。
    Sarcoidosis is a multisystem granulomatous disease of an unknown aetiology. It can exist in many organs. Pulmonary and intrathoracic lymph nodes are most commonly involved. Lung sarcoidosis is uncommon in Asia. However, due to the large population of our country and the development of bronchoscopy, percutaneous lung puncture, and other medical technologies, the number of pulmonary sarcoidosis patients is on the rise. Pulmonary sarcoidosis patients have no obvious symptoms in the early stage, and the clinical manifestations in the later stage may vary from person to person. Eventually, the disease progresses to life-threatening pulmonary fibrosis. Therefore, patients with pulmonary sarcoidosis should receive a timely diagnosis. In recent years, the imaging features and serologic biomarkers of pulmonary sarcoidosis have been continuously studied. The diagnostic value of imaging and serologic biomarkers for pulmonary sarcoidosis is summarized below.
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  • 文章类型: Journal Article
    肺结节病是一种多系统肉芽肿性间质性肺病(ILD),表现和预后可变。肺结节病的早期准确检测可以防止进展为肺纤维化,一种严重的、可能危及生命的疾病。然而,缺乏金标准的诊断测试和特定的影像学检查结果给诊断肺结节病带来了挑战.胸部计算机断层扫描(CT)成像是常用的,但需要专家,胸部训练的放射科医师区分肺结节病和肺恶性肿瘤,感染,和其他ILD。在这项工作中,我们开发了多渠道,CT和影像组学引导的集成网络(RadCT-CNNViT)对肺结节病的视觉可解释性与使用胸部CT图像进行肺癌(LCa)分类。我们利用CT和手工制作的影像组学功能作为输入通道,以及3D卷积神经网络(CNN)和视觉变换器(ViT)集成网络,用于在分类头之前进行特征提取和融合。3DCNN子网络捕获病变的局部空间信息,而ViT子网捕获远程,功能之间的全局依赖关系。通过多通道输入和特征融合,我们的模型实现了最高的性能和准确性,灵敏度,特异性,精度,F1分数,和联合AUC分别为0.93±0.04、0.94±0.04、0.93±0.08、0.95±0.05、0.94±0.04和0.97,在一项针对肺结节病(n=126)和LCa(n=93)病例的5倍交叉验证研究中。详细的消融研究显示了CNN+ViT与单独的CNN或ViT相比的影响,和CT+影像组学输入,与单独的CT或影像组学相比,也在这项工作中提出。总的来说,这项工作中开发的AI模型为从胸部CT分类肺结节病患者以便及时诊断和治疗提供了有希望的潜力.
    Pulmonary sarcoidosis is a multisystem granulomatous interstitial lung disease (ILD) with a variable presentation and prognosis. The early accurate detection of pulmonary sarcoidosis may prevent progression to pulmonary fibrosis, a serious and potentially life-threatening form of the disease. However, the lack of a gold-standard diagnostic test and specific radiographic findings poses challenges in diagnosing pulmonary sarcoidosis. Chest computed tomography (CT) imaging is commonly used but requires expert, chest-trained radiologists to differentiate pulmonary sarcoidosis from lung malignancies, infections, and other ILDs. In this work, we develop a multichannel, CT and radiomics-guided ensemble network (RadCT-CNNViT) with visual explainability for pulmonary sarcoidosis vs. lung cancer (LCa) classification using chest CT images. We leverage CT and hand-crafted radiomics features as input channels, and a 3D convolutional neural network (CNN) and vision transformer (ViT) ensemble network for feature extraction and fusion before a classification head. The 3D CNN sub-network captures the localized spatial information of lesions, while the ViT sub-network captures long-range, global dependencies between features. Through multichannel input and feature fusion, our model achieves the highest performance with accuracy, sensitivity, specificity, precision, F1-score, and combined AUC of 0.93 ± 0.04, 0.94 ± 0.04, 0.93 ± 0.08, 0.95 ± 0.05, 0.94 ± 0.04, and 0.97, respectively, in a five-fold cross-validation study with pulmonary sarcoidosis (n = 126) and LCa (n = 93) cases. A detailed ablation study showing the impact of CNN + ViT compared to CNN or ViT alone, and CT + radiomics input, compared to CT or radiomics alone, is also presented in this work. Overall, the AI model developed in this work offers promising potential for triaging the pulmonary sarcoidosis patients for timely diagnosis and treatment from chest CT.
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  • 文章类型: Case Reports
    结节病是一种全身性炎症,其特征是非干酪性肉芽肿。肺部受累是典型的,虽然肺外表现,尤其是淋巴结病,在很大一部分病例中观察到。病因涉及免疫细胞和介质之间复杂的相互作用,导致肉芽肿形成能够独立产生1,25-二羟基维生素D,导致未调节的高钙血症和高钙尿症。诊断可能具有挑战性,特别是当高钙血症是最初的症状时。活检中存在非干酪样肉芽肿是结节病的特征。我们介绍了一个53岁女性的严重高钙血症病例,在99msestamibi甲状旁腺扫描中,最初提示原发性甲状旁腺功能亢进症是由于未抑制的完整甲状旁腺激素(PTH)水平和单侧甲状腺内示踪剂摄取所致。病人出现高血压,急性肾损伤,和严重的高钙血症.初步评估,包括甲状旁腺扫描,提示原发性甲状旁腺功能亢进。然而,进一步评估,包括胸部计算机断层扫描(CT)和支气管内活检,显示结节病伴非干酪样肉芽肿。泼尼松治疗导致血清钙和肌酐水平正常化。该病例强调了诊断结节病的复杂性,尤其是当出现严重的高钙血症时。尽管没有抑制PTH和暗示性成像,最终诊断依赖于支气管内活检结果.该研究强调了常规诊断标志物的局限性,强调需要全面和个性化的方法。
    Sarcoidosis is a systemic inflammatory condition characterized by noncaseating granulomas. Lung involvement is typical, while extrapulmonary manifestations, notably lymphadenopathy, are observed in a significant proportion of cases. The etiology involves complex interactions among immune cells and mediators, resulting in granuloma formation capable of independently producing 1,25-dihydroxyvitamin D, leading to unregulated hypercalcemia and hypercalciuria. Diagnosis can be challenging, especially when hypercalcemia is the initial symptom. The presence of noncaseating granulomas on biopsy is characteristic of sarcoidosis. We present a case of severe hypercalcemia in a 53-year-old woman, initially suggestive of primary hyperparathyroidism due to non-suppressed intact parathyroid hormone (PTH) levels and unilateral intrathyroidal tracer uptake on a technetium 99m sestamibi parathyroid scan. The patient presented with hypertension, acute kidney injury, and severe hypercalcemia. Initial assessment, including a parathyroid scan, hinted at primary hyperparathyroidism. However, further evaluation, including chest computed tomography (CT) and endobronchial biopsy, revealed sarcoidosis with noncaseating granulomas. Prednisone therapy led to normalization of serum calcium and creatinine levels. The case underscores the complexities in diagnosing sarcoidosis, especially when presenting with severe hypercalcemia. Despite non-suppressed PTH and suggestive imaging, the final diagnosis relied on endobronchial biopsy findings. The study highlights the limitations of conventional diagnostic markers, emphasizing the need for a comprehensive and individualized approach.
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  • 文章类型: Case Reports
    由于其与影响不同器官的各种疾病的潜在关联,诊断不明原因发热(FUO)提出了重大挑战。1961年,Petersdorf和Beeson最初将FUO定义为在至少三周内至少三次温度超过38.3°C的条件。尽管进行了一周的住院调查,明确的诊断仍不清楚.结节病,影响多个系统的肉芽肿疾病,是FUO的原因之一。虽然肺部通常受到影响,任何器官都可以参与,导致不同的表现和临床课程。诊断依赖于临床病理发现和排除肉芽肿疾病的替代原因。结节病的标志是在受影响的器官中肉芽肿的发展。这里,我们介绍了一例61岁的男性患者,他有复发性自发性尿道周围脓肿的病史,接受了多次泌尿外科介入治疗.他在治疗感染性疾病后住院期间发展了FUO。
    Diagnosing fever of unknown origin (FUO) presents a substantial challenge due to its potential association with various diseases affecting different organs. In 1961, Petersdorf and Beeson initially defined FUO as a condition characterized by a temperature exceeding 38.3 °C on at least three occasions over a minimum three-week period. Despite a week of inpatient investigation, a definitive diagnosis remains unclear.  Sarcoidosis, a granulomatous disease impacting multiple systems, is among the causes of FUO. While the lungs are commonly affected, any organ can be involved, leading to diverse manifestations and clinical courses. Diagnosis relies on clinicopathologic findings and the exclusion of alternative causes of granulomatous disease. The hallmark of sarcoidosis is the development of granulomas in affected organs. Here, we present the case of a 61-year-old man with a history of recurrent spontaneous periurethral abscesses who underwent multiple urological interventions. He developed FUO during hospitalization following treatment for the infectious condition.
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  • 文章类型: Journal Article
    背景:结节病是一种多系统肉芽肿性疾病,最常累及肺部。在影像学检查期间,可能会偶然诊断出其他疾病或非特异性症状。偶然诊断的无症状1期疾病的适当随访尚未明确。
    目的:定义偶然诊断的无症状1期结节病的临床病程,并提出对这些患者进行随访的算法。
    方法:对所有EBUS-TBNA(支气管内超声引导下经支气管针吸活检术)证实的1期结节病病例进行回顾性病例分析,偶然出现在布里斯托尔和利物浦间质性肺病服务。临床病史,血清学结果,成像扫描,在基线时检查肺功能参数,12和24个月。进行了成本分析,比较了当前2年随访指导与1年随访期的成本。
    结果:67例患者被确定为最终队列。在两年的随访期间,肺功能检查没有显着变化。在大多数患者中观察到放射学疾病的稳定性(58%,n=29),在1年时,有40%(n=20)的疾病消退。在2年时进行成像的地方,大多数(69.8%,n=37)有疾病消退的放射学证据,30.2%(n=16)显示出稳定性的放射学证据。所有患者在研究期间保持无症状并且不需要治疗性干预。
    结论:我们的研究结果表明,无症状的胸部淋巴结非干酪样肉芽肿患者在2年内没有进展。我们的结果表明,可能没有必要对此类患者进行长期的二级护理随访。我们建议对这些患者进行1年的随访,并在出院前再进行一年的患者开始随访(PIFU)。
    BACKGROUND: Sarcoidosis is a multi-system granulomatous disease most commonly involving the lungs. It may be incidentally diagnosed during imaging studies for other conditions or non-specific symptoms. The appropriate follow-up of incidentally diagnosed asymptomatic stage 1 disease has not been well defined.
    OBJECTIVE: To define the clinical course of incidentally diagnosed asymptomatic stage 1 sarcoidosis and propose an algorithm for the follow-up of these patients.
    METHODS: A retrospective case note analysis was performed of all EBUS-TBNA (endobronchial ultrasound-guided transbronchial needle aspiration)-confirmed cases of stage 1 sarcoidosis presenting incidentally to Bristol and Liverpool Interstitial Lung Disease services. Clinical history, serology results, imaging scans, and lung function parameters were examined at baseline, 12, and 24 months. A cost analysis was performed comparing the cost of the current 2-year follow-up guidance to a 1 year follow-up period.
    RESULTS: Sixty-seven patients were identified as the final cohort. There was no significant change in the pulmonary function tests over the two-year follow-up period. Radiological disease stability was observed in the majority of patients (58%, n = 29), and disease regression was evidenced in 40% (n = 20) at 1 year. Where imaging was performed at 2 years, the majority (69.8%, n = 37) had radiological evidence of disease regression, and 30.2% (n = 16) showed radiological evidence of stability. All patients remained asymptomatic and did not require therapeutic intervention over the study period.
    CONCLUSIONS: Our results show that asymptomatic patients with incidental findings of thoracic lymph nodal non-caseating granulomas do not progress over a 2-year period. Our results suggest that the prolonged secondary-care follow-up of such patients may not be necessary. We propose that these patients are followed up for 1 year with a further year of patient-initiated follow-up (PIFU) prior to discharge.
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  • 文章类型: Case Reports
    结节病是一种起源不明的复杂炎症性疾病,以非坏死性上皮样细胞肉芽肿(NNEG)影响多器官系统为特征。尽管可以存在许多不同的临床和病理表型,涉及不同的器官,肺是最常见的部位。在这个案例报告中,我们(a)提出并讨论了具有肺癌临床和影像学特征的孤立性肺肿块患者的广泛鉴别诊断,最终被证实为II期肺结节病的罕见表现,(b)分析和比较文献中的类似案例。
    Sarcoidosis is a complex inflammatory disease of uncertain origin, characterized by non-necrotizing epithelioid cell granulomas (NNEGs) affecting multiple organ systems. Although many different clinical and pathological phenotypes can be present, with different organs involved, the lung is the most common site described. In this case report, we (a) present and discuss the broad differential diagnosis of a patient presenting with a solitary lung mass with clinical and imaging features of lung cancer that ultimately was confirmed with a rare manifestation of stage II pulmonary sarcoidosis, and (b) analyze and compare similar cases from the literature.
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  • 文章类型: Journal Article
    在结节病患者中,肺动脉高压的发展与显著的发病率和死亡率相关。据报道,结节病相关肺动脉高压(SAPH)的全球患病率在结节病患者的2.9%至20%之间。多种因素可能有助于SAPH的发展,包括晚期肺实质疾病,严重的收缩和/或舒张左心室功能障碍,静脉闭塞性或血栓栓塞性疾病,以及外在因素,如淋巴结肿大的肺血管压迫,贫血,和肝脏疾病。SAPH的早期诊断很重要,但很少实现,主要是由于筛查策略不够准确。完全依赖于非侵入性测试和临床评估。SAPH的明确诊断需要右心导管插入术(RHC),根据当前指南,经胸超声心动图作为RHC的推荐看门人。根据最近的登记结果,6分钟步行测试(6MWT)在SAPH患者中具有最大的预后价值,而使用降低的DLCO(预测<35%)确定的晚期肺病与毛细血管前SAPH的无移植存活率降低有关。临床管理涉及潜在机制的识别和治疗。肺血管扩张剂在几种情况下是有用的,特别是当肺血管表型占优势时。晚期SAPH可能需要考虑肺移植,这仍然是一个高风险的选择。需要多中心的随机对照试验来进一步开发现有的治疗方法并改善SAPH患者的预后。
    In patients with sarcoidosis, the development of pulmonary hypertension is associated with significant morbidity and mortality. The global prevalence of sarcoidosis-associated pulmonary hypertension (SAPH) reportedly ranges between 2.9% and 20% of sarcoidosis patients. Multiple factors may contribute to the development of SAPH, including advanced parenchymal lung disease, severe systolic and/or diastolic left ventricular dysfunction, veno-occlusive or thromboembolic disease, as well as extrinsic factors such as pulmonary vascular compression from enlarged lymph nodes, anemia, and liver disease. Early diagnosis of SAPH is important but rarely achieved primarily due to insufficiently accurate screening strategies, which rely entirely on non-invasive tests and clinical assessment. The definitive diagnosis of SAPH requires right heart catheterization (RHC), with transthoracic echocardiography as the recommended gatekeeper to RHC according to current guidelines. A 6-min walk test (6MWT) had the greatest prognostic value in SAPH patients based on recent registry outcomes, while advanced lung disease determined using a reduced DLCO (<35% predicted) was associated with reduced transplant-free survival in pre-capillary SAPH. Clinical management involves the identification and treatment of the underlying mechanism. Pulmonary vasodilators are useful in several scenarios, especially when a pulmonary vascular phenotype predominates. End-stage SAPH may warrant consideration for lung transplantation, which remains a high-risk option. Multi-centered randomized controlled trials are required to develop existing therapies further and improve the prognosis of SAPH patients.
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