正电子发射计算机断层显像计算机断层扫描在系统性血管炎诊断及病情评估中的应用

系统性血管炎临床表现复杂,对于缺乏特征性表现者的诊断和病情严重程度评估,临床医生面临巨大的挑战。现有影像学检查手段对系统性血管炎的早期、活动性病变敏感性较低。正电子发射计算机断层显像与计算机断层扫描技术(positron emission tomographycomputed tomography,PETCT)对系统性血管炎中巨细胞动脉炎风湿性多肌痛、大动脉炎等大血管炎的诊断具有比较高的敏感新和特异性,特别是在疾病的早期和活动期,而对于中小血管受累的系统性血管炎并无太大帮助。通过PETCT可以全面了解系统性血管炎的解剖和功能定位,弥补其他影像学检查的不足。应注意在PETCT上系统性血管炎与其他血管疾病的鉴别。PETCT检查在系统性血管炎疗效评估中的作用尚有待进一步研究。     

The Role of 18F Fluorodeoxyglucose Positron Emission Tomography Scanning in the Diagnosis and Management of Systemic Vasculitis

Primary systemic vasculitis is a group of heterogeneous disorders, characterized by inflammation of blood vessels causing end organ damage from ischemia, aneurysm formation or dissection. Delay in the early diagnosis owing to non‐specific symptoms, lack of definitive serological tests, limited availability of biopsy and standard imaging tests pose significant challenge in the management of these diseases. 18F‐fluorodeoxyglucose (FDG) positron emission tomography (PET) scanning is being increasingly used in the management of systemic vasculitis, especially the large vessel vasculitides: giant cell arteritis (GCA) and Takayasu arteritis (TAK). FDG‐PET involves detection of positron rays emitted by FDG, a fluorinated glucose analogue which is avidly taken up by metabolically active inflammatory cells in the walls of involved blood vessels. 18F‐FDG‐PET scan, especially when combined with computed tomography or magnetic resonance imaging (MRI) can give information about active inflammation as well as structural damage associated with vasculitis. In patients with GCA, FDG‐PET has acceptable sensitivity and specificity for the early diagnosis in non‐cranial GCA, cranial GCA with negative biopsy, assessment of immediate response to treatment, predicting prognosis, but has limited value in serial follow‐up and prediction of relapses. In TAK, FDG‐PET can be useful for early diagnosis and probably for serial assessment of disease activity. FDG‐PET has a limited role in medium and small vessel vasculitis.     

大血管血管炎发病机制中的遗传因素

大血管血管炎包括大动脉炎和巨细胞动脉炎,病因涉及遗传、感染、环境、免疫反应等因素,发病机制仍待明确.在遗传背景研究方面发现,大动脉炎与HLA-Ⅰ类基因区(HLA-B?52)关系最为密切,参与炎症反应的相关基因(IL12B、IL6、RPS9/LILRB3)也发挥着重要作用.在巨细胞动脉炎中,HLA-Ⅱ类(HLA-DRB1...     

血管炎的心脏病变

血管炎是一类血管壁及周围炎性细胞浸润,并伴有血管损伤的疾病。常累及全身多个系统,如消化道、神经系统,乃至心血管系统。实际上,心脏的受累所致的心血管系统的病变如心力衰竭、冠心病常是此类疾病的重要死亡、致残原因。现就大动脉炎、巨细胞动脉炎及结节性多动脉炎等三种血管炎伴发的心血管病变做一综述。     

Vasculitis research: Current trends and future perspectives

We discuss recent and prospective research in small and large vessel vasculitis. Large cohorts of Takayasu arteritis (TA) have been recently published from across the world, clarifying our understanding of this uncommon disease. Novel open‐ended approaches like large‐scale genotyping, proteomics and metabolomics have helped gain novel insights into TA, giant cell arteritis (GCA) and anti‐neutrophil cytoplasmic antibody‐associated vasculitis (AAV). Recent advances in the imaging of TA and GCA offer promise for earlier diagnosis and better monitoring of response to therapy. Although two randomized controlled trials of abatacept and tocilizumab failed to meet their primary end‐points, successful large‐scale studies of abatacept and tocilizumab in GCA hold promise for better disease control. While cyclophosphamide has revolutionized the management of AAV, increasing use of rituximab as an alternative induction regimen, as well as use of novel approaches involving reduced or no corticosteroid use for AAV and alternative agents such as avacopan (a complement 5a receptor antagonist) hold promise for lesser toxic induction regimens in the future. Increasingly, the risk of cardiovascular events and comorbidities such as osteoporosis are being recognized as factors affecting long‐term prospects of patients with vasculitis. There is a shift in emphasis to utilize patient‐reported outcomes to more accurately gauge the impact of vasculitides and their treatment.     

Organizing Pneumonia as the First Presentation in a Patient with Takayasu Arteritis: A Report of Rare Complication

A 48-year-old woman without any medical history visited an outpatient clinic with a chief complaint of cough persisting for more than 1 year and was diagnosed with organizing pneumonia. Computed tomography showed wall thickening with luminal stenosis of the main branch vessels of the aorta, and a detailed examination including fluorodeoxyglucose-positron emission tomography revealed Takayasu arteritis. There have been some reports of combined organizing pneumonia in similar vasculitis cases, but Takayasu arteritis and organizing pneumonia have not been reported to be associated. This case can be referred to when considering the association of lung lesions with Takayasu arteritis.     

Delayed Diagnosis of Biopsy-Negative Giant Cell Arteritis Presenting as Fever of Unknown Origin

Fever of unknown origin (FUO) presents a diagnostic challenge. Giant cell arteritis (GCA) may present with FUO and this entity should be included in the differential of elderly patients who present with constitutional symptoms. While a temporal artery biopsy is considered the gold standard for the diagnosis of GCA, a subset of patients with large vessel involvement by GCA may have a negative temporal artery biopsy and no cranial symptoms. We present a 79 year-old woman with FUO and negative temporal artery biopsies in whom diagnosis of GCA was delayed. Further imaging with CT-angiogram and positron emission tomography/computed tomography (PET/CT) scan showed diffuse extensive active vasculitis. The above case underscores the value of imaging studies in the evaluation of patients with FUO from occult large vessel vasculitis.     

Pneumatosis Intestinalis Developed in a Patient with Giant Cell Arteritis While in a Clinically Sustained Remission Phase

We herein report a patient with giant cell arteritis (GCA) who developed pneumatosis intestinalis (PI) while she was in a clinically sustained remission phase. A 79-year-old woman with GCA involving the thoracic aorta and its first branches to the posterior tibial arteries had been treated with high-dose prednisolone. Nine weeks after initiating treatment and while in clinically sustained remission with a normal CRP level, PI and pneumoperitoneum were incidentally found during scheduled positron emission tomography-computed tomography, which also revealed slight residual inflammation of GCA. This is a very rare case of PI complicated by GCA, and we discuss the possible relationships.