Classification Of Giant Cell Arteritis Research Articles

The role of 18F-FDG PET/CT in large-vessel vasculitis: appropriateness of current classification criteria?

Patients with clinical suspicion of large-vessel vasculitis (LVV) may present with nonspecific signs and symptoms and increased inflammatory parameters and may remain without diagnosis after routine diagnostic procedures. Both the nonspecificity of the radiopharmaceutical 18F-FDG and the synergy of integrating functional and anatomical images with PET/CT offer substantial benefit in the diagnostic work-up of patients with clinical suspicion for LVV. A negative temporal artery biopsy, an ultrasonography without an arterial halo, or a MRI without aortic wall thickening or oedema do not exclude the presence of LVV and should therefore not exclude the use of 18F-FDG PET/CT when LVV is clinically suspected. This overview further discusses the notion that there is substantial underdiagnosis of LVV. Late diagnosis of LVV may lead to surgery or angioplasty in occlusive forms and is often accompanied by serious aortic complications and a fatal outcome. In contrast to the American College of Rheumatology 1990 criteria for vasculitis, based on late LVV effects like arterial stenosis and/or occlusion, 18F-FDG PET/CT sheds new light on the classification of giant cell arteritis (GCA) and Takayasu arteritis (TA). The combination of these observations makes the role of 18F-FDG PET/CT in the assessment of patients suspected for having LVV promising.

AB0766 The role of color-doppler-sonography in the diagnosis of giant cell arteritis characterized by adventitial inflammation of the temporal arteries

BackgroundThe classic histological appearance of inflamed temporal arteries (TA) in giant cell arteritis (GCA) is transmural cell infiltration. However, periadventitial small vessel vasculitis (SVV) surrounding uninflamed TA and/or isolated vasculitis...

OP0207 The “Fast Track” Giant Cell Arteritis Outpatient Clinic: A Useful Tool to Reduce the Morbidity of Disease?

BackgroundPermanent visual loss is one of the most serious complications in giant cell arteritis (GCA). Visual manifestations have been reported in 26% and permanent visual loss in up to 15%...

FRI0333 Leflunomide as a corticosteroid-sparing agent in giant cell arteritis and polymyalgia rheumatica: a consecutive case series

BackgroundGiant cell arteritis (GCA) and polymyalgia rheumatica (PMR) affecting individuals older than 50 years and corticosteroids are the mainstay of treatment. Azathioprine and methotrexate have shown little or moderate efficacy...

SAT0174 Clinical and Histological Features in Patients with Permanent Visual Los Due to Biopsy-Proven Giant Cell Arteritis

BackgroundAmaurosis is the most frequent ischemic complication and the main cause of permanent disability among patients with giant cell arteritis (GCA). Some premonitory symptoms of visual loss such as amaurosis...

FRI0219 Involvement of supra-aortic vessels in giant cell arteritis diagnosed by color duplex ultrasonography

BackgroundThe highest incidence of giant cell arteritis (GCA) worldwide has been reported from Southern Norway [1]. Aortic branch involvement [i.e. large vessel vasculitis (LVV)] has been reported to be present...

A 66-year old woman with large vessel vasculitis after a dengue virus infection

A 66-year old Caucasian woman with a history of hypertension, primary hypothyroidism and a history suggestive of polymyalgia rheumatica presented with progressive dyspnoea, a few weeks after a dengue fever infection she had caught in Indonesia. Diagnosis of a primo dengue infection was serologically confirmed in Indonesia with a positive dengue IgM and negative IgG. Cardiologic analysis revealed several small aneurysms in the coronary arteries; a critical stenosis of the left ante rior descending artery was treated with bypass surgery. The patient had no family history of cardiovascular dis ease. One month later, she developed dizziness and vascular claudication of both upper and lower extremities. There was no fever; joint inflammation or skin lesions were absent. Physical examination revealed almost impalpable pulsations of the radial and ulnary arteries. Her blood pressure, though difficult to measure, was 120/65 mmHg. Plasma C-reactive protein (79 mg/L) and erythrocyte sedimentation rate (ESR; 50 mm/hr) were increased; leukocyte counts were normal (8.9 x109/L), as was autoimmune serology (including antinuclear and antineutrophil cytoplasmic antibodies). HLA-DR4 and HLA-DRB1*04 alleles were not present. Arterial duplex examination and computer tomography-angiography showed bilateral stenoses of over 50% of subclavian arteries and of over 75% of the axillary arteries (5-6 cm stenosis). Positron emission tomography further revealed increased activity in the entire aorta as well as the carotid, iliac, femoral and axillary arteries, suggestive of vasculitis of the large arteries (Figure 1). The patient’s demographics and lab results made Takayasu arteritis unlikely. Although biopsies of the temporal artery showed no arteritis and the traditional American College of Rheumatology criteria for the classification of giant cell arteritis were not met (only 2 out of 5 criteria: age at onset and ESR), vasculitis of all the large arteries on PET-CT combined with the patient’s age, history of polymyalgia rheumatica and increased ESR are highly suggestive of this diagnosis. 1 Successful treatment was initiated with long-term high doses of prednisone, relieving the patient’s complaints and normalizing C-reactive protein and erythrocyte sedimentation rate, which is sustained to date.

Small‐vessel vasculitis surrounding an uninflamed temporal artery and isolated vasa vasorum vasculitis of the temporal artery: Two subsets of giant cell arteritis

To evaluate the frequency and clinical characteristics of periadventitial small-vessel vasculitis (SVV) and isolated vasa vasorum vasculitis (VVV). We identified 455 temporal artery biopsies performed in residents of Reggio Emilia, Italy between 1986 and 2003. Slides of temporal artery biopsy specimens were reviewed by a pathologist who was blinded with regard to clinical data. SVV was defined as inflammation of the small vessels external to the temporal artery adventitia, and VVV was defined as isolated inflammation of temporal artery vasa vasorum. Medical records of patients with SVV and/or VVV were reviewed, and demographic, clinical, laboratory, and followup data were collected. For comparison purposes, we collected the same data from an equal number of randomly selected patients with evidence of classic giant cell arteritis (GCA). Sixteen patients had SVV, 18 had isolated VVV, and 5 had both SVV and VVV. Compared with patients with classic GCA, the frequencies of headache, scalp tenderness, abnormalities of temporal arteries, jaw claudication, anorexia, and weight loss, the levels of acute-phase reactant at diagnosis, and the initial and cumulative doses prednisone were significantly lower and the frequency of peripheral synovitis was higher in the patients with SVV, and the frequency of cranial ischemic events was similar in the 2 groups. In contrast, the clinical characteristics and erythrocyte sedimentation rate at diagnosis of patients with isolated VVV were similar to those of patients with classic GCA. Our findings indicate that isolated VVV and SVV should be considered part of the histopathologic spectrum of GCA.

Magnetic Resonance Angiography in Extracranial Giant Cell Arteritis

Noninvasive diagnosis of giant cell arteritis (GCA) remains challenging, particularly with regard to evaluation of extracranial arterial disease. The objective of the study was to retrospectively review extracranial involvement in patients with GCA and/or polymyalgia rheumatica (PMR), evaluated with magnetic resonance imaging (MRI), especially 3-dimensional contrast-enhanced magnetic resonance angiography images of the aortic arch and its branches. Clinical information, biopsy status, and MRI examinations of 28 patients with GCA/PMR were reviewed. Patient images were mixed randomly with 20 normal control images and were independently reviewed by 2 radiologists. Interobserver agreement for detection of arterial stenosis was determined by the k coefficient. Both readers described vascular alterations in keeping with extracranial GCA in 19 of 28 patients (67%) with good interobserver agreement (k = 0.73) and with even higher agreement on diagnosing nonocclusive versus occlusive disease (k = 1.00). The most common lesions were bilateral axillary stenosis or obstructions, observed by both readers in 8 patients (28%). Among the 19 patients with magnetic resonance angiography lesions in the subclavian/axillary arteries, 12 (75%) had biopsy-proven GCA, but only 5 (41%) of these patients had clinical features of large artery disease. In our series review, MRI could provide accurate information on involvement of the aortic arch and its branches in extracranial GCA, depicting different degrees of stenosis. Our analysis also illustrates that occult large artery vasculitis should be considered in patients without biopsy-proven GCA, patients with classic GCA but without clinical signs of large artery disease, and in patients initially diagnosed as having PMR.

Role of imaging studies in the diagnosis and evaluation of giant cell arteritis in Japanese: report of eight cases

The objective of this study is to clarify the characteristics and imaging results of Japanese patients with giant cell arteritis (GCA). Eight patients with biopsy-proven GCA were enrolled. Their clinical data and imaging results were retrospectively examined from their medical records. All the patients met the criteria for the classification of GCA by the American College of Rheumatology. Although the clinical manifestations are similar to those previously reported, none of the eight patients presented ocular symptoms, and half of them presented jaw claudication. Ultrasonography (US) of temporal artery showed the halo sign in all the patients. Fluorodeoxyglucose positron emission tomography (FDG-PET) was performed in four patients and indicated the presence of aortitis of the patients. US is a quick and noninvasive test to detect inflammation of temporal artery, and FDG-PET is very helpful for early diagnosis of aortitis in GCA. Awareness of the disease and appropriate imaging tests will result in diagnosis of GCA.

Web of Confusion

c t p m ( PRESENTATION The occurrence of a late-stage complication in a patient with early disease, reminds us that any illness can take an unpredictable course. A 63-year-old man presented with complaints of fatigue and rash. He had been in his usual state of health until approximately 2 months prior, when he developed decreased visual acuity in his right eye. He was seen by a local ophthalmologist, who diagnosed nonarteritic ischemic optic neuritis and prescribed a daily aspirin. Soon thereafter, the patient noticed that he was having trouble moving about his house and working, and he felt as if he needed to sit and rest often. Additionally, he admitted to night sweats and a 15-lb weight loss. Upon questioning, he also reported that he had developed a nonpruritic rash on his legs and torso. He denied headache, joint pain, myalgia, morning stiffness, jaw claudication, weakness, cough, or shortness of breath. He took no medications other than a daily adult aspirin. On examination, the patient exhibited livedo reticularis involving his lower limbs, buttocks, and lower torso circumferentially (Figures 1 and 2). He was normotensive, afebrile, and had no other rash, nodules or ulcers. His joints were normal, as were his heart, lungs, and abdomen. Peripheral pulses were present, and his temporal arteries appeared normal and were nontender.

Role of imaging studies in the diagnosis and evaluation of giant cell arteritis in Japanese: report of eight cases

The objective of this study is to clarify the characteristics and imaging results of Japanese patients with giant cell arteritis (GCA). Eight patients with biopsy-proven GCA were enrolled. Their clinical data and imaging results were retrospectively examined from their medical records. All the patients met the criteria for the classification of GCA by the American College of Rheumatology. Although the clinical manifestations are similar to those previously reported, none of the eight patients presented ocular symptoms, and half of them presented jaw claudication. Ultrasonography (US) of temporal artery showed the halo sign in all the patients. Fluorodeoxyglucose positron emission tomography (FDG-PET) was performed in four patients and indicated the presence of aortitis of the patients. US is a quick and noninvasive test to detect inflammation of temporal artery, and FDG-PET is very helpful for early diagnosis of aortitis in GCA. Awareness of the disease and appropriate imaging tests will result in diagnosis of GCA.

The diagnostic value of ultrasonography-derived edema of the temporal artery wall in giant cell arteritis: a second meta-analysis

BackgroundUltrasonography of temporal arteries is not commonly used in the approach of patients with suspected giant cell arteritis (GCA) in clinical practice. A meta-analysis of primary studies available through April 2004 concluded that ultrasonography could indeed be helpful in diagnosing GCA. We specifically re-examined the diagnostic value of the ultrasonography-derived halo sign, a dark hypoechoic circumferential thickening around the artery lumen, indicating vasculitic wall edema, in GCA.MethodsOriginal, prospective studies in patients with suspected GCA that examined ultrasonography findings of temporal arteries using the ACR 1990 classification criteria for GCA as reference standard, published through 2009, were identified. Only eight studies involving 575 patients, 204 of whom received the final diagnosis of GCA, fulfilled technical quality criteria for ultrasound. Weighted sensitivity and specificity estimates of the halo sign were assessed, their possible heterogeneity was investigated and pooled diagnostic odds ratio was determined.ResultsUnilateral halo sign achieved an overall sensitivity of 68% (95% CI, 0.61-0.74) and specificity of 91% (95% CI, 0.88-0.94) for GCA. The values of inconsistency coefficient (I2) of both sensitivity and specificity of the halo sign, showed significant heterogeneity concerning the results between studies. Pooled diagnostic odds ratio, expressing how much greater the odds of having GCA are for patients with halo sign than for those without, was 34 (95% CI, 8.21-138.23). Diagnostic odds ratio was further increased to 65 (95% CI, 17.86-236.82) when bilateral halo signs were present (sensitivity/specificity of 43% and 100%, respectively). In both cases, it was found that DOR was constant across studies.ConclusionTemporal artery edema demonstrated as halo sign should be always looked for in ultrasonography when GCA is suspected. Providing that currently accepted technical quality criteria are fulfilled, halo sign's sensitivity and specificity are comparable to those of autoantibodies used as diagnostic tests in rheumatology. Validation of revised GCA classification criteria which will include the halo sign may be warranted.

A 46‐year‐old woman with chin pain and a fainting spell

History of the present illness The patient had a history of primary central nervous system (CNS) lymphoma that had recurred in the past but had been in remission for 2 years. She was admitted to the hospital in August 2008, following a syncopal episode. For several weeks prior to her syncope, she had experienced generalized malaise and the sensation of total body weakness. On the day of admission, she was nauseated and had one episode of non-bloody emesis. As the day progressed, she felt lightheaded and eventually lost consciousness while walking to the bathroom. In the emergency department, she was suspected of dehydration. However, an electrocardiogram revealed complete heart block (CHB). A temporary transjugular pacing wire was placed, intravenous fluids were started, and she was admitted to the Cardiac Unit. The patient stated that she had been completely well until 2 months before admission, when she began to experience easy fatigability. One month before admission, she developed a series of episodes of myalgias that involved her shoulders and neck. These symptoms responded to acetaminophen. She had also experienced periodic night sweats during this time. Two weeks before admission, the patient presented to her oncologist with the symptom of chin pain that radiated bilaterally toward the angle of her mandible. The pain was described as “sharp and shooting.” It sometimes appeared to be worsened by jaw movement. The patient indicated that chewing exacerbated the pain, starting with the first bite. Her oncologist had obtained a brain magnetic resonance imaging (MRI) study to exclude a lymphoma recurrence. The MRI study was interpreted as showing no new lesions. The pain did not respond to nonsteroidal antiinflammatory drugs and had been present for 2 weeks at the time of her syncope.

A 24-YEAR-OLD MALE WITH HEADACHES

A 24-year-old man presented with a ten-day history of severe headache leading to collapse. CT studies showed filling defects involving the anterior, middle and posterior cerebral arteries and evidence of ischemia and infarction. Post-mortem examination revealed multiple cerebral infarcts secondary to an arteritic process composed of multi-nucleated giant cells, lymphocytes and histiocytes in both middle and anterior cerebral arteries and one posterior cerebral artery. Both carotid siphons and one renal artery segment were also involved. Extensive workup and stains for systemic and infectious causes were negative, leading to a diagnosis of atypical giant cell arteritis (GCA). Disseminated GCA involving extracranial arteries and the anterior, middle and posterior cerebral arteries leading to cerebral infarction has not been previously reported. We report this atypical case of disseminated GCA in a young patient with clinical features distinct from classic GCA (temporal arteritis) and discuss the differential diagnosis.

Noninfectious Ascending Aortitis: A Case Series of 64 Patients

To identify the clinical presentation and histopathologic characteristics of noninfectious ascending aortitis. A retrospective medical record and histopathology review was performed of patients with histologic evidence of active noninfectious aortitis who underwent ascending aortic aneurysm resection at Mayo Clinic between January 1, 2000, and February 28, 2006. Clinicopathologic features were recorded, including demographics, clinical presentation, laboratory, imaging findings, histopathology, complications, treatment, and outcome. Sixty-four patients (50% women) were identified; the majority were Caucasian (83%) and elderly (mean age 69.1 yrs). Upon initial presentation, 45% had aneurysm-related symptoms, 33% were asymptomatic, 12.5% had constitutional symptoms, 4.7% had symptoms referable to cranial arteries, and 9.4% had polymyalgia rheumatica (PMR) symptoms. The majority (81%) were of "isolated" variant, with no rheumatologic history. Mean preoperative erythrocyte sedimentation rate was 16.2 +/- 23.3 mm/h (n = 20). Additional vascular imaging abnormalities were present in 72% of patients, including stenoses and/or ectasia of major aortic branches and descending thoracic or abdominal aneurysms. Giant cells were seen in 71.9%. Median followup time was 15.4 months, during which 6 (9.4%) patients died. Only 22 (34%) patients received corticosteroids, with uncertain effect on development of recurrent aneurysms, rupture, or dissections. Noninfectious ascending aortitis frequently occurs even in the absence of history, symptoms, or signs of giant cell arteritis (GCA) or PMR. When discovered, such patients should be followed closely, as a majority have additional vascular abnormalities. More studies are needed to determine optimal strategies for surveillance, detection, and treatment of ascending aortitis, which may represent a clinical entity distinct from classical GCA.

Diplopie brutale après l’âge de 50 ans, toujours rechercher une maladie de Horton

Although giant cell arteritis (GCA) is a rare cause of ophthalmoplegia, swift diagnosis and treatment are necessary to avoid other complications. We report here a case of GCA in a 59-year-old woman with a history of hypertension and thyroid lobectomy. Diagnosis resulted from binocular diplopia, although classical GCA symptoms had been present a few days before. Oral corticotherapy led to a rapid disappearance of headaches and normal ocular motility in 1 month. We discuss the ophthalmological signs of the disease and the place of temporal artery biopsy and treatment.

Epidemiological and clinical features of giant cell arteritis in Tunisia

Background Giant cell arteritis (GCA) is a systemic vasculitis of the elderly that could result in vision loss or even be life threatening. Unlike western countries, this disease is considered exceptional in Tunisia. Objective The aims of this study were to determine epidemiological and clinical features of GCA in Tunisian population and to identify management difficulties. Patients and methods A multicentric study of 96 patients in whom GCA was diagnosed between 1986 and 2003. All patients fulfilled the ACR criteria for classification of GCA. Results The majority of cases (77%) were diagnosed since 1994. The male/female ratio was 0.88 and the mean age at the time of diagnosis was 70.8 ± 7.7 years. Clinical features were characterized by gradual onset in 64.4% of cases. The most frequent clinical manifestations were headache (91.7%), abnormalities in temporal arteries (85.4%), severe ischemic manifestations (80.2%), constitutional symptoms (75%), and polymyalgia rheumatica (56.3%). Biological inflammatory syndrome was noted in all patients. Temporal artery biopsy established histological diagnosis in 73% of cases. All patients were treated by corticosteroids. Remission was obtained in 45.6%. Relapses occurred in 40.4% of cases and 30 patients were still receiving corticosteroids at the time of study. Four patients died and irreversible ischemic complications were noted in 15.6% of cases. Steroid adverse effects occurred in 56 patients. Conclusion GCA is not exceptional to Tunisia. It occurs amongst elderly patients with no female predominance noticed. Clinical features are similar to those reported in other series.

Relationship between histological subtypes and clinical characteristics at presentation and outcome in biopsy-proven temporal arteritis

Temporal arteritis (TA) may offer major complications, whilst high dosage of prednisone may result in serious side effects. We tried to identify a subgroup of TA, which can be treated with a lower dosage of prednisone. Retrospectively, clinical and laboratory data were studied at presentation, as well as the outcome in 44 consecutive patients with biopsy-proven temporal arteritis. These data were related to three particular histological subgroups, (a) classical giant cell arteritis, (b) atypical arteritis, and (c) 'healed arteritis', defined according to Allsop and Gallagher (The American Journal of Surgical Pathology 5:317-332, 1981). At presentation in subgroup c, erythrocyte sedimentation rate was lower and the level of haemoglobin was higher than in the other two subgroups. During follow-up in the healed arteritis group, reactivation, recurrence, or early death were not observed, whilst prednisone dosage after 2 and 3 years was lower compared to subgroup b. Major complications (permanent blindness and cerebrovascular accident) were only observed in subgroups a and b. We believe that the healed arteritis subgroup represents a relatively benign subgroup with a mild clinical presentation and a good prognosis. Therefore, a much lower initial prednisone dosage (15 mg/day) is suggested for patients in subgroup c than in the other two subgroups (40-60 mg/day).

OP12. DISEASE ACTIVITY ASSESSMENT IN POLYMYALGIA RHEUMATICA (PMR) AND GIANT CELL ARTERITIS (GCA)

Polymyalgia rheumatica (PMR) constitutes a common rheumatic disorder in the elderly population, affecting from 0.1 to 0.5% of people older than 50 years. The diagnosis is based upon a clinical syndrome, consisting of pain and stiffness in the shoulder and pelvic girdle, muscle tenderness of the upper and lower limbs, non-specific somatic complaints, frequently occurring fever, weight loss and fatigue. Moreover the acute phase response measured by Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) is highly elevated, despite the fact that PMR may also exist without elevated acute phase reactants [1–3]. Although PMR and Giant Cell Arteritis (GCA) are commonly regarded as two clinical variations of the same disease, their clinical picture is quite different. Whilst in PMR the musculoskeletal symptoms predominate, arterial inflammation and its consequences constitute the major features of GCA [1]. Giant-cell arteritis is less frequent than polymyalgia rheumatica. The American College of Rheumatology-criteria include an erythrocyte sedimentation rate of at least 50 mm per hour as one of the five criteria found to be useful in the classification of giant-cell arteritis [4]. It is well known and overall consensus that corticosteroid therapy in PMR and GCA usually leads to a rapid and dramatic improvement. Usually higher starting doses are administered in GCA patients. [1]. The EULAR response criteria for PMR and the PMR-disease activity score (PMR-AS) [5, 6] were developed on the basis of corticosteroid treatment. Along with the reduction of corticosteroids almost all parameters of disease activity, showed significant improvement indicating decrease of inflammatory activity, reduction of pain, and amelioration of functional status of the patients. The EULAR response criteria for PMR comprise a core set of markers of disease activity for monitoring therapeutic responses in PMR, namely ESR or CRP, representing the acute phase response, and the VAS of patient's pain, Physician's global assessment (PhGA), morning stiffness (MST) and the ability to elevate the upper limbs, representing the clinical situation [5]. In view of the crucial role pain plays during the course of the disease, it was decided to insist on the presence of a change of pain intensity, whereas out of the other four parameters only three have to change to indicate improvement or deterioration [5]. In order to express PMR-activity as an absolute number a PMR disease activity score, the PMR-AS, was developed on the basis of the EULAR response criteria. The PMR-AS is to be calculated as: CRP (mg/dl) + VAS p (0 – 10) + VAS ph (0 – 10) + [MST (min)*0,1] + EUL (3 – 0) [6]. Cronbach's alpha for the composite score amounted to 0.914 and 0.881 in two patient cohorts, indicating high internal consistency. Factor analysis revealed that each single item contributes significantly to the total score. PMR-AS values below 7 can be regarded as indicating low disease activity, between 7 and 17 medium disease activity, and greater than 17 high PMR-activity. In a third control cohort the PMR-AS was proven to be highly correlated to patient's global assessment, patient satisfaction and ESR (p<0.001) [6]. In GCA the onset of symptoms may occur subacutely, but also abruptly. An elevated ESR as well as elevated CRP-levels and elevated Interleukin-6-levels (IL-6) are considered as diagnostic features and as markers for disease activity [1]. Imaging techniques, such as magnetic resonance imaging and PET-scans play an important role as diagnostic, but also as monitoring tools during the course of GCA and its treatment respectively [7, 8]. Up to now the gold standard of monitoring GCA- patients comprises ESR or CRP, which is thought to provide higher specificity, in addition the control of symptoms as well as patient's global assessment. Anticardiolipin antibody levels may be helpful to predict flares and relapses in GCA patients; additionally IL-6 levels were recently described not only as markers for disease activity assessment, but also as prognostic markers. An elevated Il-6 production was seen to be related to a lower incidence of disease related ischemic events in GCA-patients [9, 10]. It may be anticipated that a better knowledge of disease activity processes, an exact monitoring of disease activity and treatment responses, as well as an increased risk-estimation of treatment schedules, e.g. corticosteroids, in PMR and GCA will result in improved clinical decision–making and ultimately in improved patient care.