Investigations in systemic vasculitis – The role of imaging

Imaging plays an increasing role for confirming a suspected diagnosis of giant cell arteritis (GCA) or Takayasu arteritis (TAK). Ultrasound, magnetic resonance imaging (MRI), and computed tomography demonstrate a homogeneous, most commonly concentric, arterial wall thickening. 18F-Fluorodeoxyglucose (FDG) positron emission tomography (PET) displays increased FDG uptake of inflamed artery walls delineating increased metabolism. Ultrasound and MRI are recommended to be the initial imaging modalities in cranial GCA and TAK, respectively. Extracranial disease can be confirmed by all four modalities, particularly by PET in case of inflammation of unknown origin. If the diagnosis remains uncertain, additional investigations including biopsy and/or additional imaging are recommended. Imaging should be performed by a trained specialist using appropriate operational procedures and settings with appropriate equipment. Further research is necessary on the role of imaging for disease monitoring.This review will discuss advantages and disadvantages of imaging modalities in the diagnosis of vasculitis.     

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.     

Early large vessel systemic vasculitis in adults

Giant cell arteritis (GCA) is the most common vasculitis in individuals older than 50 years in Western countries. In addition to the typical pattern of cranial ischemic manifestations, large vessel vasculitis (LVV) involvement has emerged as a common feature of GCA. Patients with predominant LVV manifestations differ from those with the cranial pattern. They are usually affected at a younger age and often have nonspecific manifestations such as constitutional syndrome, fever of unknown origin, or refractory/atypical polymyalgia rheumatica (PMR). In these patients, cranial manifestations are often absent. Furthermore, patients with isolated PMR should be followed up because of the potential risk of severe vascular complications in the setting of an underlying GCA. Whereas temporal artery biopsy and/or color duplex ultrasound of the temporal arteries is useful for the diagnosis of cranial GCA, Doppler sonography of the subclavian and axillary arteries, fluorine-18-fluorodeoxyglucose–positron emission tomography/computed tomography, magnetic resonance, and computed tomography-angiography are very useful to identify the presence of LVV, and they may play a potential role in the follow-up of these patients.     

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。     

Usefulness of 18F‐fluorodeoxyglucose positron emission tomography for diagnosis of asymptomatic giant cell arteritis in a patient with Alzheimer's disease

It is often difficult to diagnose disease in elderly patients, in particular those with dementia, who do not present with typical symptoms. This report describes our experience of an elderly patient (an 83‐year‐old woman) who presented with a chief complaint of memory loss, showed a marked inflammatory response, and was diagnosed with large‐vessel giant cell arteritis (GCA) on the basis of ¹⁸F‐fluorodeoxyglucose positron emission tomography (FDG‐PET) findings. She had no symptoms typical of GCA including jaw claudication, visual field defect and heavy headed feeling. Corticosteroid therapy resulted in a trend toward improvement in the inflammatory response and then she first recognized that she might have experienced slight dull headache before treatment of GCA. This was probably because this patient had large‐vessel GCA, which produces a few symptoms in the head and neck, and because she had Alzheimer's disease and could not accurately describe her symptoms. Our experience suggests the usefulness of FDG‐PET for the diagnosis of GCA, particularly in elderly patients without typical symptoms. Geriatr Gerontol Int 2011; 11: 114–118.     

Vascularite des artères utérines révélant une artérite à cellules géantes : cas clinique et revue de la littérature

IntroductionGenital vasculitis are uncommon. They may be localized or be a manifestation of a systemic vasculitis. We report a patient with a giant cell arteritis (GCA) involving uterine arteries and a literature review on genital vasculitis.Case reportA 65-year-old woman was referred to a gynecologist for a cervical intraepithelial neoplasia (CIN) associated with an ovarian mass. An unexpected diagnosis of GCA involving small to medium sized uterine arteries was made through the anatomopathological analysis while the patient was asymptomatic. Two weeks later, she presented typical cranial symptoms of giant cell arteritis (GCA). PET-scanner confirmed the diagnosis of GCA with an involvement of the ascending aorta, and the axillary and the subclavian arteries.ConclusionGynecologic vasculitis are rare and usually an asymptomatic manifestations of GCA.     

Takayasu arteritis and giant cell arteritis: are they a spectrum of the same disease?

Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are forms of large‐vessel vasculitides that affect the aorta and its branches. There is ongoing debate about whether they are within a spectrum of the same disease or different diseases. Shared commonalities include clinical features, evidence of systemic inflammation, granulomatous inflammation on biopsy, role of T‐helper (Th)‐1 and Th17 in the pathogenesis, and, abnormalities of the aorta and its branches on imaging. However, there are also several differences in the geographic distribution, genetics, inflammatory cells and responses to treatment. This review highlights the similarities and differences in the epidemiology, pathogenesis, clinical manifestations, imaging findings and treatment responses in these conditions. Current data supports that they are two distinct conditions despite the numerous similarities.     

Imaging in large-vessel vasculitis

Imaging has recently been acknowledged as at least equivalent to histology for confirming large-vessel vasculitis in the recommendations of scientific societies. Many studies have been recently published on the use of ultrasound, magnetic resonance imaging (MRI), computed tomography (CT) and fluorodeoxyglucose (FDG) positron emission tomography (PET) in giant cell arteritis and Takayasu arteritis. Ultrasound, MRI and CT show concentric, arterial wall thickening in vasculitis. PET demonstrates enhanced FDG uptake of inflamed artery walls due to increased glucose metabolism. Ultrasound is particularly useful for smaller arteries like the temporal arteries due to its high resolution. MRI and PET/CT provide an excellent overview particularly on extracranial arteries. However, ultrasound can also detect extracranial vasculitis while PET/CT may delineate vasculitis in temporal arteries. The diagnosis needs to be confirmed without delay as the sensitivity of imaging decreases with treatment. Ongoing studies are evaluating the role of imaging for follow-up.     

PET-CT findings in large vessel vasculitis presenting as FUO, a case report

There are increasing data demonstrating the role of flourodeoxyglucose positron emission tomography with computerized tomography fusion (¹⁸FDG PET-CT) in the diagnosis of large vessel vasculitides, including Takayasu arteritis and giant cell arteritis (Hara et al. 1999; Blockmans et al. 1999; Turlakow et al. 2001]. We report a case of large vessel giant cell arteritis involving the major branches of the aorta as detected on ¹⁸FDG PET-CT. A 56-year-old woman returning to the USA after visiting her native Iraq presented to our rheumatology department with fever of unknown origin (FUO) of 2-month duration, night sweats, and arthralgias. The patient did not have claudication; systolic blood pressure measurements demonstrated a 20-mmHg difference between her arms. Infectious disease, malignancy, and collagen vascular disease workup was unrevealing. Temporal artery and bone marrow biopsies were negative. To exclude FUO of malignancy, ¹⁸FDG PET-CT imaging was performed. The images demonstrated significant ¹⁸FDG uptake (indicating increased metabolic activity) in a circumferential fashion along the aorta and its major braches, including the carotid, subclavian, and common iliac arteries. Contrast-enhanced CT imaging demonstrated wall thickening involving these vessels along with left subclavian vein thrombosis and findings consistent with superficial thrombophlebitis involving the right forearm, wrist, and hand. The combination of laboratory and imaging findings, including the characteristic inflammatory changes involving the large vessel walls as seen on CT, as well as the vessel wall hypermetabolism on FDG PET indicating active inflammation, resulted in the diagnosis of large vessel giant cell arteritis. The patient was treated with high-dose corticosteroids followed by a course of Immuran. Her symptoms resolved and a follow-up FDG PET-CT showed complete resolution of the large vessel hypermetabolism. ¹⁸F-FDG PET-CT can be a useful and noninvasive tool in diagnostic evaluation of FUO by excluding a malignant etiology and providing unexpected information that aids in correct diagnosis.     

La périartérite noueuse,un diagnostic alternatif à l’artérite gigantocellulaire devant une artérite temporale

IntroductionWeight loss, myalgias, neurologic manifestations and arterial hypertension are common features of polyarteritis nodosa (PAN) at diagnosis. Temporal arteritis is a rarer manifestation of PAN, more suggestive of giant cell arteritis (GCA).CaseWe report the case of a 77-year-old woman who presented with fatigue, weight loss, fever, neck pain, jaw claudication and cough, diagnosed with giant cell arteritis. Diagnosis was reconsidered in favour of a medium and small-sized vessels necrotizing vasculitis corresponding to PAN because of steroid dependence, mononeuritis and suggestive histological features.ConclusionAlthough temporal arteritis is suggestive of GCA, other causes of temporal arteritis can be identified with temporal artery biopsy.     

肺血管炎的临床与影像学特点解析

肺部血管炎包括原发性与继发性两大类.继发性血管炎包括感染性疾病、结缔组织病、恶性肿瘤和过敏性疾病所致肺血管炎.原发性血管炎的分类通常根据受累血管的大小分为大血管炎、中血管炎和小血管炎.肺部血管受累常见于原发性大血管炎[大动脉炎(Takayasu arteritis),巨细胞动脉炎(giant cell arteritis,GCA),白塞病(Behcetdisease)]和原发性抗中性粒细胞胞浆抗体(anti-neutrophil cytoplasmic antibody,ANCA)相关性小血管炎[肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA),显微镜下多血管炎(microscopic polyangiitis),嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)].原发性肺血管炎的影像学表现极具多样性,包括血管壁增厚、结节影、空洞、磨玻璃影和实变影等.原发性肺部小血管炎常导致弥漫性肺泡出血(diffuse alveolar hemorrhage,DAH).相比于胸片,胸部CT更能够显示肺血管炎的病变特征和侵及范围.肺部血管炎的诊断极具挑战性,需要通过患者的临床特征、影像学特点、实验室检查结果和组织病理学特征作出综合判断.     

Different Scoring Methods of FDG PET/CT in Giant Cell Arteritis: Need for Standardization

Giant cell arteritis (GCA) is the most frequent form of vasculitis in persons older than 50 years. Cranial and systemic large vessels can be involved. [¹⁸F] fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) is increasingly used to diagnose inflammation of the large arteries in GCA. Unfortunately, no consensus exists on the preferred scoring method. In the present study, we aim to define the optimal FDG PET/CT scoring method for GCA diagnosis using temporal artery biopsy and clinical diagnosis as the reference method.FDG PET/CT scans of GCA patients (12 glucocorticoid-naive, 6 on glucocorticoid treatment) and 3 control groups (inflammatory, atherosclerotic, and normal controls) were evaluated. We compared 2 qualitative visual methods (i.e. (1a) first impression and (1b) vascular uptake versus liver uptake) and 4 semiquantitative methods ((2a) SUVmax aorta, (2b) SUVmax aorta-to-liver ratio, (2c) SUVmax aorta-to-superior-caval-vein ratio, and (2d) SUVmax aorta-to-inferior-caval-vein ratio). FDG uptake pattern (diffuse or focal) and presence of arterial calcifications were also scored.Diagnostic accuracy of the visual method vascular versus liver uptake (1b) was highest when the cut-off point “vascular uptake higher than liver uptake” (sensitivity 83%, specificity 91%) was used. Sensitivity increased to 92% when patients on glucocorticoids were excluded from the analysis. Regarding the semiquantitative methods, the aorta-to-liver ratio (2b) with a cutoff of 1.03 had the highest diagnostic accuracy, with a sensitivity and specificity of 69% and 92%, respectively. Sensitivity increased to 90% when patients on glucocorticoids were excluded. The number of vascular segments with diffuse FDG uptake pattern was significantly higher in GCA patients without glucocorticoid use compared with all control patient groups. CRP was not significantly different between positive and negative FDG PET scans in the GCA group.Visual vascular uptake higher than liver uptake resulted in the highest diagnostic accuracy for the detection of GCA, especially in combination with a diffuse FDG uptake pattern. Of the semiquantitative methods, the aorta-to-liver SUVmax ratio (cutoff point = 1.03) had the highest diagnostic accuracy. The diagnostic accuracy increased when patients using glucocorticoids were excluded from the analyses.     

Giant cell arteritis

Giant cell arteritis (GCA) is the most common vasculitis of the elderly. The diagnosis can be challenging at times because of the limitation of the American Rheumatology Association (ARA) classification criteria and the significant proportion of biopsy-negative patients with GCA. We discuss the role of advanced imaging techniques, including positron emission tomography (PET) scanning, in establishing diagnosis and improved histopathology techniques to improve the sensitivity of temporal artery biopsy.There have been significant advances in the understanding of the pathogenesis of GCA, particularly the role of cytokine pathways such as the interleukins, IL-6-IL-17 axis, and the IL-12-interferon-γ axis and their implication for new therapies. We highlight that glucocorticoids remain the primary treatment for GCA, but recognize the risk of steroid-induced side effects. A number of pharmacotherapies to enable glucocorticoid dose reduction and prevent relapse have been studied.Early diagnosis and fast-track pathways have improved outcomes by encouraging adherence to evidence-based practice.     

Giant Cell Arteritis Presenting with Ptosis and Diplopia

Giant cell arteritis (GCA) is vasculitis of large-sized vessels that can lead to vision loss. We herein report a rare case of GCA accompanied by ptosis and diplopia as early symptoms, which were caused by third nerve palsy. A 78-year-old man presented with fever, right temporal headache, right eyelid ptosis, and diplopia. GCA was confirmed by a temporal artery biopsy. The symptoms disappeared after a slight delay following the administration of prednisolone. Unlike vision loss, ptosis and diplopia are considered to be reversible and responsive to treatment. GCA should not be ruled out if patients exhibit these ophthalmic symptoms.     

Place de la tomographie par émission de positrons (TEP) au [18F]FDG dans l’exploration et la surveillance des vascularites

[18F]fluorodeoxyglucose positron emission tomography (PET) is a noninvasive metabolic imaging modality that is well-suited to the assessment of activity and extent of large vessel vasculitis. PET imaging has demonstrated its usefulness in diagnosing giant cell arteritis (notably in its silent form), Takayasu's arteritis, and unclassified aortitis. PET imaging could be more effective than magnetic resonance imaging in detecting the earliest stages of vascular wall inflammation. The visual grading of vascular [18F]FDG uptake makes it possible to discriminate arteritis from active atherosclerosis, providing therefore high specificity. High sensitivity can also be achieved provided scanning is performed during active inflammatory phase, preferably before starting corticosteroid treatment. Prospective studies are needed to determine the exact value of PET imaging in assessing other vasculitis subsets, infectious aortitis, and large vessel vasculitis outcome and response to immunosuppressive treatment.     

Temporal arteritis associated with systemic necrotizing vasculitis

OBJECTIVE: To evaluate the clinical and laboratory characteristics of patients with systemic vasculitis associated with temporal artery involvement. METHODS: From a cohort of 120 patients fulfilling American College of Rheumatology criteria for temporal arteritis, we retrospectively identified 7 patients with systemic necrotizing vasculitis associated with histological temporal arteritis. RESULTS: Among the 7 patients, 2 had classic polyarteritis nodosa, one had unclassified systemic vasculitis, one had Wegener's granulomatosis (WG), and 3 had microscopic polyangiitis. The mean age of the patients was 70.2 years, and cranial symptoms revealed the disease in all but one patient. Temporal arteritis was generally associated with extracephalic manifestations suggestive of systemic vasculitis. Antineutrophilic cytoplasmic antibodies were positive in 3 of the 4 patients with small vessel vasculitis. Pathologically, the main temporal artery was involved in all but one patient, with inflammatory infiltrate of vasa vasorum and adventitia associated in 5 with small tributary involvement. Fibrinoid necrosis was rare, observed in 2 specimens; 2 patients with unclassified systemic vasculitis and WG had a classic giant cell arteritis (GCA) histologic pattern. Only one patient had exclusive involvement of small vessels, surrounding the spared main temporal artery. Muscle biopsies showed histopathological evidence of vasculitis in 2 patients, skin biopsy in one, and vein biopsy in the other. CONCLUSION: Temporal artery involvement in systemic necrotizing vasculitis was generally associated with extracranial clinical features suggestive of systemic vasculitis. Temporal artery biopsy is a simple tool for diagnosis of vasculitis, but the histopathological findings do not always discriminate between necrotizing vasculitis and classic GCA.     

Supraorbital nodule biopsy leading to the diagnosis of giant cell arteritis

Giant cell arteritis is a systemic vasculitis which usually involves the cranial arteries. We report the case of a 57‐year‐old patient presenting with headache and painful subcutaneous supraorbital nodule. A subsequent biopsy of a supraorbital nodule led to the diagnosis of giant cell arteritis.     

Vaskulitiden der gro?en Gef??e

Large vessel vasculitis is characterized by local inflammation of the vessel wall of the aorta and its first and second degree branches. Giant cells are found regularly. The process leads to stenoses and occlusions as well as to aneurysmatic dilatation. Recent progress in vascular imaging (colour Doppler ultrasound, MRI and PET) allows to detect Takayasu arteritis at an earlier stage and to reveal the involvement of larger arteries in temporal arteritis (giant cell arteritis, GCA). With increasing frequency, a subtype of predominantly female and less aged GCA patients without cranial symptoms is noticed in whom obstructions are prevailing in the proximal arm vessels or in the aorta. The latter may be affected exclusively. Initial therapy is based on corticosteroids. In GCA, methotrexate hitherto is the only other immunosuppressive drug that has proved to be helpful. Disease activity has to be controlled both by laboratory parameters and vascular imaging.     

Giant cell arteritis: Diagnosis and therapeutic management

Giant cell arteritis (GCA) is the most common systemic vasculitis in the elderly in Western countries. Anemia and very high erythrocyte sedimentation rates are associated with lower incidence of severe ischemic events. Temporal artery biopsy is the gold standard in the diagnosis of GCA. A threshold size of 1 cm of post-formalin fixed segment of the temporal artery was associated with increased diagnostic yield of GCA. Other tools such as ultrasound, MRI, and fluorodeoxyglucose positron emission tomography are useful in the diagnosis of GCA. Corticosteroids are the cornerstone treatment of GCA. Studies using other drugs have yielded contradictory results.     

3-T MRI detects inflammatory stenosis of the vertebral artery in giant cell arteritis

Giant cell arteritis (GCA) is a granulomatous vasculitis. Early diagnosis is important for the initiation of corticosteroid treatment because the arteritis can result in blindness. In most of the cases, the superficial cranial arteries are affected. However, extracranial involvement of various arteries is known. Here, we report a case of histologically proven GCA with an inflammatory stenosis of the right vertebral artery. For complete evaluation of the extension of the disease, an optimized protocol of high-resolution magnetic resonance imaging at 3 T in combination with contrast-enhanced magnetic resonance angiography was performed. This non-invasive method facilitates the differentiation of inflamed and healthy segments of small cranial arteries, may help to find appropriate sites for biopsy, and allows the assessment of affected extracranial vessels. In this patient case, even the cause of vertebral stenosis—inflammatory versus arteriosclerotic—could be elucidated.