Time Of Giant Cell Arteritis Diagnosis Research Articles

Elevated cerebrospinal fluid levels of total protein in patients with secondary central nervous system vasculitis and giant cell arteritis

Objectives: Secondary central nervous system vasculitis (SCNSV) is an extremely rare, refractory, and fatal disease in patients with giant cell arteritis (GCA). We compared the characteristics of GCA patients with and without SCNSV.Methods: This retrospective, single-center, observational cohort study included 35 patients with GCA admitted to Juntendo University Hospital from April 2009 to March 2019. The primary outcome was all-cause mortality.Results: We diagnosed four patients with GCA and SCNSV (SCNSV group) and 31 patients with GCA but no SCNSV (non-SCNSV group). The mortality rate of the SCNSV and non-SCNSV groups was 100% and 10%, respectively (p = .001). The SCNSV group had lower serum levels of C-reactive protein at the time of GCA diagnosis and higher cerebrospinal fluid (CSF) levels of total protein (102 mg/dL vs. 38 mg/dL, p = .008) and albumin (66 mg/dL vs. 21 mg/dL, p = .008) at the time of SCNSV diagnosis.Conclusion: At the time of SCNSV diagnosis, GCA patients had elevated CSF total protein and albumin levels. CSF examination in GCA patients suspected of having SCNSV may be useful for early diagnosis of SCNSV.

Risk of ischaemic events at giant cell arteritis diagnosis according to PET/CT findings.

To analyse the risk of ischaemic events in patients with newly diagnosed giant cell arteritis (GCA) according to PET/CT findings. PET/CT was performed during the first 10days of steroid therapy. Clinical manifestations at diagnosis, and physical examination and PET/CT findings were recorded and compared according to the presence or absence of ischaemic symptoms at disease onset. Analysed territories included the ascending aorta, aortic arch, descending aorta, abdominal aorta, carotid arteries, brachiocephalic trunk, vertebral arteries, subclavian arteries and axillary arteries. The study group comprised 30 patients with a median age of 80.8years. Of these patients, 21 (70%) reported ischaemic symptoms at diagnosis, and 13 (43.3%) had permanent visual loss. Of the 30 patients, 77.8% showed large vessel vasculitis (including aortic and vertebral artery involvement) on PET/CT, and 60% had isolated involvement of the vertebral territory. Vertebral arteries were more frequently involved in patients with ischaemic symptoms (OR 5.0, 95% CI 0.99-24.86, p = 0.051). The presence of vertebral artery involvement in the absence of aortic involvement was associated with the presence of ischaemic manifestations (Fisher's exact test, p = 0.001). The presence of aortitis was found to protect against the development of permanent visual loss (OR 19.0, 95% CI 2.79-127.97, p = 0.001). Our findings suggest an association between the vascular pattern on PET/CT at the time of GCA diagnosis and the risk of ischaemic events.

INTERSTITIAL LUNG DISEASE IN AN ELDERLY MAN WITH GIANT CELL ARTERITIS

PURPOSE: To describe the association of interstitial lung disease (ILD) with giant cell arteritis (GCA). METHODS: Case report RESULTS: An 89-year-old never-smoker male with a history of polymyalgia rheumatica (PMR) on chronic low dose prednisone was later diagnosed with giant cell arteritis (GCA) confirmed by temporal artery biopsy. At the time of GCA diagnosis, the patient reported dyspnea and chest radiographs showed bilateral interstitial infiltrate with peripheral and basilar predominance. Chest computed-tomography (CT) performed several months later showed findings of a fibrosing interstitial pneumonia with mild traction bronchiectasis involving predominantly the bibasilar and peripheral lung zones, consistent with a probable usual interstitial pneumonia (UIP) pattern (Figure A). These findings were new compared to prior CT imaging four years before. Pulmonary function test (PFT) revealed a moderate restrictive pattern with forced vital capacity (FVC) 73% and diffusing capacity of the lungs for carbon monoxide (DLCO) 53%. The patient experienced improvement with increased prednisone dose. At follow up 6 months after hospitalization, repeat PFT revealed FVC 91% and DLCO 65%. Repeat chest CT showed that parenchymal infiltrates improved with decreased reticulation and resolution of ground-glass opacities (Figure B). CONCLUSIONS: The temporal relationship in this case suggests the association of GCA with ILD. Although the CT findings suggested a UIP pattern, the patient experienced improvement with prednisone treatment. CLINICAL IMPLICATIONS: In recent years there have been multiple reports of ILD or pulmonary fibrosis associated with small vessel vasculitis, especially ANCA-related vasculitis. This case suggests large vessel vasculitis may cause ILD and mimic idiopathic pulmonary fibrosis in the elderly.

Giant Cell Arteritis-related Stroke: A Retrospective Multicenter Case-control Study.

Our aim was to describe patients with giant cell arteritis (GCA)-related stroke and to compare them with a control group of GCA patients without stroke. We created a retrospective multicenter cohort of patients with (1) GCA diagnosed according to the American College of Rheumatology criteria between 1995 and 2015, and (2) stroke occurring at the time of GCA diagnosis or occurring within 4 weeks of starting GCA therapy. The control group consisted of GCA patients without stroke. Forty patients [21 women (53%), median age 78 (60-91) yrs] with GCA-related stroke were included and were compared with 200 control patients. Stroke occurred at GCA diagnosis in 29 patients (73%), whereas it occurred after diagnosis in 11 patients. Vertebrobasilar territory was involved in 29 patients (73%). Seven patients died within a few hours or days following stroke. Compared with the control group, stroke patients had more ophthalmic ischemic symptoms [25 (63%) vs 50 (25%), p < 0.001]. Conversely, they demonstrated lower biological inflammatory variables [C-reactive protein: 61 (28-185) mg/l vs 99 (6-400) mg/l, p = 0.04] and less anemia [22/37 (59%) vs 137/167 (79%), p = 0.03] than patients without stroke. Multivariate logistic regression revealed that the best predictors for the occurrence of stroke were the presence of ophthalmic ischemic symptoms at diagnosis (OR 5, 95% CI 2.14-12.33, p = 0.0002) and the absence of anemia (OR 0.39, 95% CI 0.16-0.99, p = 0.04). Stroke, especially in the vertebrobasilar territory, is more likely to occur in patients with GCA who experience recent ophthalmic ischemic symptoms and who exhibit low inflammatory variables.

Prevalence of ischemic complications in patients with giant cell arteritis presenting with apparently isolated polymyalgia rheumatica

ObjectiveTo investigate the frequency and type of giant cell arteritis (GCA)-related ischemic complications in a series of patients with GCA who, for a substantial period of time (i.e., at least 3mo), lacked vascular symptoms and presented with apparently isolated polymyalgia rheumatica (PMR). MethodsRetrospective follow-up study of an unselected population of 167 patients with GCA diagnosed from 1985 to 2014. ResultsIn all, 18 patients (11%) developed GCA on a background of a prior history of PMR. They were diagnosed as having isolated PMR because they did not have clinical evidence of GCA at diagnosis and exhibited a prompt and complete response to low-dose steroid therapy. However, during the course of treatment, 17 patients later experienced an arteritic relapse with the development of typical craniofacial symptoms, and one patient developed signs of upper extremity vascular insufficiency, resulting in the diagnosis of large-vessel GCA. The median time to GCA diagnosis from the initiation of low-dose steroid therapy was 9 ± 14.4mo (range: 3−39).At the time of GCA diagnosis, severe ischemic complications were observed in 50% (9/18) of the patients. Of these patients 22% (4/18) were considered to have “true” occlusive disease (i.e., permanent visual loss, stroke, and/or limb claudication).Late inflammation of the aorta and its branches occurred in 4 (22%) of the patients during long-term follow-up. ConclusionPatients with GCA presenting with apparently isolated PMR have a significant risk of developing transient or permanent disease-related ischemic complications; these complications occurred in 50% of the cases.

The Use of Anti-Platelet and/or Anticoagulant Agents in the Prevention of Large Vessel Vasculitis-Associated Ischemic Complications: A Meta-Analysis

Objective: To determine the effectiveness of antiplatelet and/or anticoagulant therapy (AP/AC) at reducing ischemic events in patients with Large Vessel Vasculitis (LVV). Methods: We performed a random effects meta-analysis of studies examining antiplatelet and/or anticoagulant therapy (AP/AC) and ischemic events in Takayasu’s Arteritis (TAK) or Giant Cell Arteritis (GCA). Severe ischemic events were defined as stroke, ischemic ocular manifestations and claudication symptoms. Any ischemic event included jaw claudication in addition to the above manifestations. Results: Seven studies met inclusion criteria: 1 TAK and 6 GCA. The majority of patients (>80%) were treated with ASA and treatment was initiated prior to diagnosis of LVV. Risk of severe and any ischemic event in patients with LVV treated with AP/AC versus no treatment was not significantly different (OR 0.570, 95% CI 0.243, 1.340 and OR 0.594, 95% CI 0.248, 1.421, respectively). For studies with follow-up data (26-76 months), AP/AC was protective for severe ischemic events (OR 0.18, 95% CI 0.04, 0.83). Findings were similar when excluding studies that did not account for potential confounders, such as cardiovascular risk factors. Conclusion: At follow-up, antiplatelet therapy significantly decreases ischemic events in patients with LVV. However, in most cases of GCA, the treatment was initiated prior to the diagnosis of vasculitis. The benefit of initiating anti-platelet therapy at the time of GCA diagnosis remains unclear.

Strokes at Time of Disease Diagnosis in a Series of 287 Patients With Biopsy-Proven Giant Cell Arteritis

Patients with giant cell arteritis (GCA) generally present with cranial ischemic manifestations that are directly related to vascular involvement. They may also experience strokes in the territory of the carotid or the vertebrobasilar artery. We conducted the current study to assess the frequency and predictors of strokes in general, and of vertebrobasilar stroke in particular, at the time of diagnosis in a series of 287 consecutive patients with biopsy-proven GCA diagnosed over a 27-year period at the single hospital for a well-defined population of northwestern Spain.During the study period, 8 (2.8%) patients had strokes (1 in the carotid and 7 in the vertebrobasilar territory) between the onset of symptoms of the disease and 4 weeks after the onset of corticosteroid therapy. Six of the 7 patients with vertebrobasilar stroke were men. In most cases the vertebrobasilar stroke occurred after the onset of corticosteroid therapy. Smoking history was more common among patients with vertebrobasilar stroke (p = 0.01). Patients with vertebrobasilar stroke more commonly had permanent visual loss due to arteritic involvement of ophthalmic branches derived from the internal carotid (3/7; 42.9%) than the rest of GCA patients (33/280; 11.8%) (p = 0.05). Patients with strokes had higher hemoglobin values (13.2 +/- 1.5 g/dL) than patients without (11.7 +/- 1.6 g/dL) (p = 0.009). Moreover, only 1 (14.3%) of the 7 patients with vertebrobasilar stroke had anemia compared to 157 (56.1%) of the remaining 280 patients (p = 0.05). The best predictors of stroke were permanent visual loss (odds ratio [OR], 5.42) and arterial hypertension (OR, 5.06). In contrast, women (OR, 0.10) and patients with anemia at the time of disease diagnosis (OR, 0.11) had a significantly reduced risk of suffering strokes. Smoking history was the best positive predictor of vertebrobasilar stroke (OR, 5.22). In contrast, a reduced risk of suffering vertebrobasilar strokes was found in individuals who had anemia at the time of GCA diagnosis (OR, 0.13).Results of the current study show an increased risk of strokes, in the vertebrobasilar territory in particular, at the time of GCA diagnosis. Patients with biopsy-proven GCA and traditional cardiovascular risk factors or permanent visual loss have an increased risk of suffering strokes. Results also suggest a potential protective role of anemia against the development of these cerebrovascular complications.

Long-Term Follow-Up of Aortic Involvement in Giant Cell Arteritis

To date, only a few series have analyzed the long-term outcome of giant cell arteritis (GCA) patients with aortic involvement, which prompted us to conduct the current retrospective study. Our aims were to 1) determine the prevalence of GCA in patients exhibiting nonatherosclerotic aortic involvement (that is, aortitis, aortic ectasia, and/or aneurysm); and 2) evaluate clinical features and long-term outcome of GCA patients exhibiting aortitis, aortic ectasia, and/or aortic aneurysm.From January 1997 to March 2008, 66 consecutive patients in the Department of Internal Medicine at the University of Rouen medical center received a diagnosis of nonatheromatous aortic complications (aortitis, aortic ectasia, and/or aneurysm). In these 66 patients, aortic involvement was related to GCA (n = 48), Takayasu arteritis (n = 6), relapsing polychondritis (n = 1), and infection (n = 11).Of the 48 patients with GCA, aortic involvement preceded the initial GCA diagnosis in 1 patient. Aortic involvement was identified in association with GCA in 40 patients (83.3%), and developed after the onset of GCA in the 7 remaining patients (14.6%). Aortic involvement was more often asymptomatic (77.1%). The aortic helical computed tomography (CT)-scan procedure principally showed isolated aortitis (circumferential thickening of the aortic wall >3 mm) in 41 patients (85.4%). In the remaining 7 patients with GCA (14.6%), aortic helical CT scan demonstrated aortic thoracic ectasia and aortitis (n = 3), aortic thoracic aneurysm and both thoracic and abdominal aortitis (n = 3), and both aortic abdominal aneurysm and aortitis (n = 1). All patients were given steroid therapy at a median daily dose of 1 mg/kg initially.At 6-month follow-up, 34 of 48 patients systematically underwent both thoracic and abdominal CT scan. Aortic helical CT scan demonstrated complete disappearance of aortitis in 8.8% of patients, improvement of aortitis in 47.1%, unchanged pattern of aortitis and/or aortic thoracic ectasia/aneurysm in 41.2%, and deterioration of aortic thoracic aneurysm in 1 patient (2.9%). At 18-month follow-up, 11 patients systematically underwent both thoracic and abdominal CT scan. Aortic helical CT scan showed complete disappearance of aortitis (n = 1), improvement of aortitis (n = 1), unchanged pattern of aortic thoracic ectasia/aneurysm (n = 2), and deterioration of aortic thoracic aneurysm (n = 1). At patients' last follow-up, the median daily dose of prednisone was 7 mg. Steroid therapy could be discontinued in 17 patients (35.4%).The current retrospective study suggests that aortic impairment may be more prevalent than previously reported. Our findings suggest that specific inflammatory thickening of the aortic wall is common at the time of GCA diagnosis, and that aortitis may be the first manifestation of GCA-associated aortic complications. Whether isolated aortitis leads to vascular wall injury responsible for late-onset aneurysmal disease remains to be determined. At this time, we recommend long-term monitoring for aortic aneurysms, especially in high-risk subjects, although the optimal frequency and imaging modality have not yet been determined. A yearly screening strategy for thoracic/abdominal aortic aneurysms has been proposed for patients with GCA, including physical examination, 2-view chest radiograph, and abdominal ultrasound.

Cancer in Biopsy-Proven Giant Cell Arteritis. A Population-Based Study

To investigate the potential association between giant cell arteritis (GCA) and cancer in a series of consecutive patients diagnosed with biopsy-proven GCA over a 25-year period at the single reference hospital for a well-defined population. The case records of all patients diagnosed with biopsy-proven GCA at the Department of Medicine of the Hospital Xeral-Calde (Lugo, Northwest Spain) between January 1, 1981 and December 31, 2005 were reviewed. Information on cancer and cause of death over the extended follow-up was assessed. In all cases the presence of cancer was histologically confirmed. Cancer was found in 39 (15.3%) of the 255 GCA patients. Although 7 (18%) of the 39 patients had cancer either at the time or within the first 12 months after GCA diagnosis, the standardized mortality ratio (SMR) due to cancer in patients with biopsy-proven GCA showed no increase (overall SMR 1.06 [0.65-1.60]; men, 0.81; women, 1.50). The time interval between GCA diagnosis and cancer diagnosis was 5.2+/-3.8 years (median 4.2 years; interquartile range: 3-7 years). When multivariate analysis adjusted by age and sex was performed, only the presence of dysphagia (adjusted hazards ratio (HR)=3.90; P=0.04), abnormal temporal artery on physical examination (adjusted HR=4.61; P=0.04), and anemia at the time of GCA diagnosis (adjusted HR=3.39; P=0.01) were associated with an increased risk of cancer over the extended follow-up. The results from this series do not support an overall increase of mortality due to cancer in GCA.

Influence of Traditional Risk Factors of Atherosclerosis in the Development of Severe Ischemic Complications in Giant Cell Arteritis

Because the prognosis of giant cell arteritis (GCA) is related to the development of ischemic complications, we sought to assess the possible influence of traditional risk factors of atherosclerosis in the development of severe ischemic complications of GCA. We conducted a retrospective study of patients with biopsy-proven GCA diagnosed from 1981 to 2001 at the single hospital for a well-defined population of almost 250,000 people. Patients were considered to have severe ischemic manifestations if they suffered visual manifestations, cerebrovascular accidents, jaw claudication, or signs of occlusive changes in large arteries of the extremities. Patients were assessed for the presence of hypercholesterolemia, hypertension, diabetes mellitus, and heavy smoking at the time of GCA diagnosis. The presence of traditional risk factors of atherosclerosis at the time of GCA diagnosis in this series of 210 patients increased significantly the risk of developing at least 1 of the severe ischemic complications (odds ratio [OR], 1.79; 95% confidence intervals [CI], 1.03-3.11; p = 0.04). Patients with traditional atherosclerosis risk factors had fever less commonly than the rest of GCA patients (5.2% vs. 16.0%; p = 0.01). GCA patients with hypertension exhibited a significantly increased risk of developing severe ischemic complications (OR, 1.80; 95% CI, 1.00-3.25; p = 0.05). The current study suggests that the presence of atherosclerosis risk factors at the time of diagnosis of GCA may influence the development of severe ischemic manifestations of the disease.