bone sarcoidosis

骨结节病
  • 文章类型: Case Reports
    结节病是一种全身性疾病,其特征是非干酪性肉芽肿形成,可以影响身体的任何器官;然而,骨骼受累相对少见。该病例报告介绍了一例罕见的结节病病例,主要影响一名39岁有神经结节病病史的男性的骨骼系统。病人出现恶心症状,呕吐,疲劳,减肥,下背部和骨盆疼痛,最初怀疑是恶性肿瘤。计算机断层扫描显示溶解性骨病变和淋巴结肿大。然而,左腹股沟淋巴结活检证实坏死性肉芽肿性淋巴结炎,这与坏死性结节病肉芽肿病一致-结节病的一种罕见变体。患者接受全身性皮质类固醇治疗,导致临床改善。结节病的预后一般良好,自发缓解发生在多达三分之二的患者;然而,一些患者可能发展为慢性和/或进行性疾病。特别是,有神经结节病病史的患者患慢性或复发性疾病的风险可能增加.该病例强调了在表现为非特异性症状和淋巴结病的患者的鉴别诊断中考虑结节病的重要性。即使没有肺部受累。
    Sarcoidosis is a systemic disorder characterized by noncaseating granuloma formation, which can affect any organ in the body; however, skeletal involvement is relatively uncommon. This case report presents a rare case of sarcoidosis primarily affecting the skeletal system in a 39-year-old man with a history of neurosarcoidosis. The patient presented with symptoms of nausea, vomiting, fatigue, weight loss, and lower back and pelvic pain, which were initially suspicious for malignancy. Computed tomography scans revealed lytic bone lesions and lymphadenopathy. However, a biopsy of a left inguinal lymph node confirmed necrotizing granulomatous lymphadenitis, which was consistent with necrotizing sarcoid granulomatosis - a rare variant of sarcoidosis. The patient was treated with systemic corticosteroids, which led to clinical improvement. The prognosis of sarcoidosis is generally good, with spontaneous remission occurring in up to two-thirds of patients; however, some patients may develop chronic and/or progressive disease. In particular, patients with a history of neurosarcoidosis may be at an increased risk for chronic or recurrent disease. This case highlights the importance of considering sarcoidosis in the differential diagnosis of patients presenting with nonspecific symptoms and lymphadenopathy, even in the absence of pulmonary involvement.
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  • 文章类型: Journal Article
    结节病是一种不明原因的多系统肉芽肿性疾病。最常见的定位是胸部淋巴结和/或肺实质疾病,然而,任何其他器官都可能参与其中。肌肉骨骼结节病,以前被认为是这种疾病的罕见表现,目前认识到频率越来越高,由于现代成像模式的发展。骨结节病的经典X射线征象是手指骨中的花边图像。大多数其他位置存在非典型放射学图像。因此,它们可能模仿转移性肿瘤疾病,特别是当它们是结节病的第一个迹象之前没有认识到。在这种场合,没有一种成像方法能给出正确的诊断,组织病理学验证,对结节病确诊患者的病变或临床数据进行监测。本文总结了有关骨结节病的认识和治疗的知识现状。此外,介绍了一例骨和骨髓结节病患者的说明性病例。
    Sarcoidosis is a multisystem granulomatous disease of unknown origin. The most frequent localizations are thoracic lymph nodes and/or parenchymal lung disease, nevertheless any other organ may be involved. Musculoskeletal sarcoidosis, previously considered a rare manifestation of the disease, is presently recognized with increasing frequency, due to the development of modern imaging modalities. The classical X-ray sign of bone sarcoidosis is the image of lace in the phalanges of the hands. Most other locations present with atypical radiological images. Therefore, they may mimic metastatic neoplastic disease, especially when they are the first sign of sarcoidosis not previously recognized. On such occasions, none of the imaging methods will give the correct diagnosis, histopathological verification, monitoring of lesions or clinical data in a patient with confirmed sarcoidosis are indicated. The article summarizes the current status of knowledge concerning the recognition and therapy of bone sarcoidosis. In addition, an illustrative case of patient with bone and bone marrow sarcoidosis is presented.
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  • 文章类型: Journal Article
    结节病,病因不明的全身性炎症性疾病,可以影响身体的任何部位。在多器官结节病患者中,氟脱氧葡萄糖位置发射断层扫描/计算机断层扫描(FDGPET/CT)意外发现了骨病变。应考虑使用FDGPET/CT检测结节病的临床无症状病变。
    Sarcoidosis, a systemic inflammatory disease of unknown etiology, can affect any site in the body. A bone lesion was unexpectedly detected by fluorodeoxyglucose position emission tomography/computed tomography (FDG PET/CT) in a patient with multiorgan sarcoidosis. FDG PET/CT should be considered for the detection of clinically silent lesions of sarcoidosis.
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  • 文章类型: Case Reports
    在结节病和颈部癌症(HNC)中,骨位置都不常见。因此,骨病变的诊断在患有两种疾病的患者中区分其性质是具有挑战性的。我们报告了一例因P16阳性HPV-HNC转诊的69岁女性。磁共振成像(MRI)显示T2低信号在髂棘和脊柱。18FDG-PET在这些位置显示放射性示踪剂摄取,提示骨转移。然而,骨活检显示上皮样肉芽肿无恶性细胞与结节病位置相容。局部晚期HPV-HNC和系统性结节病的诊断(骨,中枢神经系统)被保留。患者接受0.5mg/kg/天的皮质类固醇方案和甲氨蝶呤治疗结节病,放疗和铂类药物治疗癌症。由于肉芽肿性骨髓浸润可能在18FDG-PET上有摄取,骨结节病可以模拟转移性疾病。此外,MRI通常无法区分结节病病变与骨骼中的转移病变。由于没有可靠的成像测试可以破译这两种疾病,我们病例的描述加强了对患有两种疾病的患者进行骨活检的必要性,以了解骨病变的性质。
    Bone location is uncommon in both sarcoidosis and in neck cancer (HNC). Diagnosis of a bone lesion is therefore challenging to distinguish its nature in a patient suffering from both diseases. We report the case of a 69-years-old woman referred for P16 positive HPV-HNC. Magnetic Resonance Imaging (MRI) showed T2 hypo-signal on iliac crest and spine. 18FDG-PET demonstrated radiotracer uptake on these locations suggesting bone metastasis. However, bone biopsy showed epithelioid granuloma without malignant cells compatible with sarcoidosis location. The diagnosis of both localized advanced HPV-HNC and systemic sarcoidosis (bone, central nervous system) were retained. The patient received corticosteroid regimen at 0.5mg/kg/day and Methotrexate for sarcoidosis and radiation and chemotherapy with platins for carcinoma. As granulomatous bone marrow infiltration may have an uptake on 18FDG-PET, bone sarcoidosis can mimic metastatic disease. In addition, MRI often fails to distinguish sarcoidosis lesions from metastatic lesion in bones. As no reliable imaging test can decipher both diseases, the description of our case reinforces the necessity to perform bone biopsy in a patient suffering from both conditions to expertise the nature of bone lesions.
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  • 文章类型: Case Reports
    Sarcoidosis is a systemic granulomatous inflammatory disease that can involve almost any organ system in the human body. It most frequently presents with pulmonary infiltrates, hilar lymphadenopathy, and skin lesions. Clinical and subclinical involvement of other organ systems is not uncommon. However, the simultaneous development of clinically apparent multisystem sarcoidosis is very rare.
    This 44-year-old Caucasian man presented to an outpatient clinic with a 2-month history of fatigue, night sweats, weight loss, loss of appetite, and mild abdominal discomfort. Initial laboratory finding showed elevated liver enzymes. Imaging studies revealed cirrhotic liver with steatosis, few enhancing hepatic masses, and multiple enlarged periaortic and portocaval lymph nodes. Liver biopsy revealed scattered necrotizing granulomatous hepatitis. Positron emission tomography scan showed extensive hepatic uptake, diffuse lymphadenopathy, as well as numerous fluorodeoxyglucose-avid osseous lesions. After extensive workup to rule out malignancy and infectious etiologies, a diagnosis of diffuse multi-organ sarcoidosis was made. He was ultimately treated with methotrexate and steroids, resulting in marked improvement in symptoms and liver function, with stable disease on repeat imaging.
    Diffuse multi-organ sarcoidosis is often associated with widespread lymphadenopathy and osseous lesions, which appear indistinguishable from malignancy on imaging. The angiotensin converting enzyme levels and inflammatory markers may be normal. Clinicians should be aware of the possibility of diffuse systemic sarcoidosis in any patient with a remote sarcoidosis history and the simultaneous development of multi-organ-related symptoms.
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  • 文章类型: Case Reports
    目的:脊柱结节病,提到脊柱受累于结节病,是相对罕见的,可能模仿影响脊柱的其他神经系统疾病。作者介绍了18例三级医院的脊柱结节病患者的临床放射学回顾,重点是初始成像和放射学对治疗的反应。
    方法:我们回顾性回顾了我们15年的科室影像档案,发现49例神经结节病,其中18例患者进行了脊柱磁共振成像。
    结果:大约72%(13/18)的神经结节病患者表现出某种形式的脊髓受累。临床,在就诊和随访时对这13例患者的流行病学和影像学资料进行了回顾。磁共振成像的发现包括软脑膜增强(61%),厚膜(23%),髓内增强病变(38%)和骨受累(15%)。宫颈段最常见,其次是胸段。受累通常是长段(4.2脊柱段),背侧索倾向。平均随访23.2个月。66%的患者出现完全或接近完全的放射学反应,而25%的患者出现部分反应。四名患者有孤立的中枢神经系统受累,其中一名患有孤立的脊髓受累。在弥散加权成像中,在基线和随后的随访扫描中,髓内病变的表观扩散系数与正常脊髓相比增加.
    结论:脊髓结节病以前被认为并不常见,但随着磁共振成像的广泛使用,其认识越来越多。背侧表面受累是特征性的,虽然不一定是pathognomonic。此外,定量扩散研究可以作为疾病活动和实质损伤的生物标志物。
    OBJECTIVE: Spinal sarcoidosis, referring to involvement of the spine in sarcoidosis, is relatively rare and may mimic other neurological disease affecting the spine. The authors present a clinic radiological review of 18 spinal sarcoidosis patients who presented to a tertiary hospital, with emphasis on initial imaging and radiological response to treatment.
    METHODS: We retrospectively reviewed our departmental imaging archives over a 15-year period and found 49 cases of neurosarcoidosis out of which 18 patients had spinal magnetic resonance imaging.
    RESULTS: Approximately 72% (13/18) of the neurosarcoidosis patients showed some form of spinal involvement. The clinical, epidemiological and imaging data were reviewed for these 13 patients at presentation and follow-up. The findings on magnetic resonance imaging included leptomeningeal enhancement (61%), pachymeningeal (23%), intramedullary enhancing lesions (38%) and bony involvement (15%). The cervical segment was most frequently involved followed by the thoracic segment. Involvement was often long segment (4.2 spinal segments) with proclivity for the dorsal cord. Mean follow-up was 23.2 months. A complete or near-complete radiological response occurred in 66% while partial response was seen in 25% patients. Four patients had isolated central nervous system involvement including one with isolated spinal cord involvement. On diffusion-weighted imaging, the apparent diffusion coefficient of intramedullary lesions was increased compared to normal-appearing cord on baseline and subsequent follow-up scans.
    CONCLUSIONS: Spinal sarcoidosis was previously considered uncommon but is being increasingly recognized with widespread use of magnetic resonance imaging. Proclivity for dorsal surface involvement is characteristic, although not necessarily pathognomonic. Also, quantitative diffusion studies may serve as a biomarker for the disease activity and parenchymal injury.
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  • 文章类型: Journal Article
    为了评估临床特征,诊断,以及在美国的骨结节病的治疗。
    根据种族确定骨结节病患者并与结节病患者相匹配,性别,和年龄。通过病历审查获得了详细的特征。
    本研究共纳入64例骨结节病患者。女性:男性比例为1.46:1,白色:黑色比例为3:1。64例患者中有38例(59.4%)出现骨症状。与匹配的病例相比,骨结节病患者多器官受累,肝脏发病率较高,脾,脾胸外淋巴结受累优于对照组(P<0.05)。脊柱是44例(68.8%)患者中最常见的骨,其次是骨盆(35.9%),和手(15.6%)。MRI和PET/CT扫描是常用的成像技术,在36名患者和32名患者中进行,分别,骨摄取阳性率为97.2%和93.8%。实验室检查提示骨结节病组贫血发生率高于对照组(P=0.044)。英夫利昔单抗更常用于骨结节病患者(P=0.009)。
    骨结节病与多器官病变有关,和高频率的肝脏,脾,脾或胸外淋巴结受累.对于侵袭性和难治性骨结节病患者,应考虑使用英夫利昔单抗。
    To assess the clinical features, diagnosis, and treatment of bone sarcoidosis in the United States.
    Patients with bone sarcoidosis were identified and matched to sarcoidosis patients based on race, gender, and age. Detailed characteristics were obtained by medical record review.
    A total of 64 patients with bone sarcoidosis were enrolled in this study. The female:male ratio was 1.46:1 and the white:black ratio was 3:1. Thirty-eight (59.4%) of 64 patients had bone symptoms. Compared to matched cases, bone sarcoidosis patients have more multi-organ involvement and higher incidence with liver, spleen, and extrathoracic lymph node involvement than controls (P < 0.05). Spine was the most commonly affected bone in 44 (68.8%) of patients, followed by pelvis (35.9%), and hands (15.6%). MRI and PET/CT scan was the common imaging technology, which performed in 36 patients and 32 patients, respectively, and with 97.2% and 93.8% positive bone uptake. Laboratory test indicated anemia was more common in bone sarcoidosis group than controls (P = 0.044). Infliximab was more commonly used in bone sarcoidosis patients than controls (P = 0.009).
    Bone sarcoidosis was associated with multi-organs affection, and high frequency of liver, spleen, or extrathoracic lymph node involvement. Infliximab should be considered in those patients with aggressive and refractory bone sarcoidosis.
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