Onset Of Giant Cell Arteritis Research Articles

Apolipoproteins and the risk of giant cell arteritis—a nested case–control study

BackgroundThe etiology of giant cell arteritis (GCA) and its predictors are incompletely understood. Previous studies have indicated reduced risk of future development of GCA in individuals with obesity and/or diabetes mellitus. There is limited information on blood lipids before the onset of GCA. The objective of the study was to investigate the relation between apolipoprotein levels and future diagnosis of GCA in a nested case–control analysis.MethodsIndividuals who developed GCA after inclusion in a population-based health survey (the Malmö Diet Cancer Study; N = 30,447) were identified by linking the health survey database to the local patient administrative register and the national patient register. A structured review of medical records was performed. Four controls for every validated case, matched for sex, year of birth, and year of screening, were selected from the database. Anthropometric measures, self-reported physical activity, based on a comprehensive, validated questionnaire, and non-fasting blood samples had been obtained at health survey screening. Concentrations of apolipoprotein A-I (ApoA-I) and apolipoprotein B (ApoB) in stored serum were measured using an immunonephelometric assay. Potential predictors of GCA were examined in conditional logistic regression models.ResultsThere were 100 cases with a confirmed clinical diagnosis of GCA (81% female; mean age at diagnosis 73.6 years). The median time from screening to diagnosis was 12 years (range 0.3–19.1). The cases had significantly higher ApoA-I at baseline screening compared to controls (mean 168.7 vs 160.9 mg/dL, odds ratio [OR] 1.57 per standard deviation (SD); 95% confidence interval [CI] 1.18–2.10) (SD 25.5 mg/dL). ApoB levels were similar between cases and controls (mean 109.3 vs 110.4 mg/dL, OR 0.99 per SD; 95% CI 0.74–1.32) (SD 27.1 mg/dL). The ApoB/ApoA1 ratio tended to be lower in cases than in controls, but the difference did not reach significance. The association between ApoA-I and GCA development remained significant in analysis adjusted for body mass index and physical activity (OR 1.48 per SD; 95% CI 1.09–1.99).ConclusionSubsequent development of GCA was associated with significantly higher levels of ApoA-I. These findings suggest that a metabolic profile associated with lower risk of cardiovascular disease may predispose to GCA.

Association between infection and the onset of giant cell arteritis and polymyalgia rheumatica: a systematic review and meta-analysis

ObjectiveWe aimed to analyse the association between infections and the subsequent risk of giant cell arteritis (GCA) and/or polymyalgia rheumatica (PMR) by a systematic review and a meta-analysis of observational...

Atypical posterior reversible encephalopathy syndrome (PRES) as onset of giant cell arteritis (GCA).

Atypical posterior reversible encephalopathy syndrome (PRES) as onset of giant cell arteritis (GCA).

Lower Frequency of Comorbidities Prior to Onset of Giant Cell Arteritis: A Population-Based Study.

To assess the frequency of comorbidities and metabolic risk factors at and prior to giant cell arteritis (GCA) diagnosis. This is a retrospective case control study of patients with incident GCA between January 1, 2000, and December 31, 2019, in Olmsted County, Minnesota. Two age- and sex-matched controls were identified, and each assigned an index date corresponding to an incidence date of GCA. Medical records were manually abstracted for comorbidities and laboratory data at incidence date, 5 years, and 10 years prior to incidence date. Twenty-five chronic conditions using International Classification of Diseases, 9th revision, diagnosis codes were also studied at incidence date and 5 years prior to incidence date. One hundred and twenty-nine patients with GCA (74% female) and 253 controls were identified. At incidence date, the prevalence of diabetes mellitus (DM) was lower among patients with GCA (5% vs 17%; P = 0.001). At 5 years prior to incidence date, patients were less likely to have DM (2% vs 13%; P < 0.001) and hypertension (27% vs 45%; P = 0.002) and had a lower mean number (SD) of comorbidities (0.7 [1.0] vs 1.3 [1.4]; P < 0.001) compared to controls. Moreover, patients had significantly lower median fasting blood glucose (FBG; 96 mg/dL vs 104 mg/dL; P < 0.001) and BMI (25.8 vs 27.7; P = 0.02) compared to controls. Multivariable logistic regression analysis revealed negative associations for FBG with GCA at 5 and 10 years prior to diagnosis/index date. DM prevalence and median FBG and BMI were lower in patients with GCA up to 5 years prior to diagnosis, suggesting that metabolic factors influence the risk of GCA.

Malignancies in Giant Cell Arteritis: A Population-based Cohort Study

To investigate the risk of cancer in patients with biopsy-proven giant cell arteritis (GCA) from a defined population in southern Sweden. The study cohort consisted of 830 patients (mean age at GCA diagnosis was 75.3 yrs, 74% women) diagnosed with biopsy-proven GCA between 1997 and 2010. Temporal artery biopsy results were retrieved from a regional database and reviewed to ascertain GCA diagnosis. The cohort was linked to the Swedish Cancer Registry. The patients were followed from GCA diagnosis until death or December 31, 2013. Incident malignancies registered after GCA diagnosis were studied. Based on data on the first malignancy in each organ system, age- and sex-standardized incidence ratios (SIR) with 95% CI were calculated compared to the background population. One hundred seven patients (13%) were diagnosed with a total of 118 new malignancies after the onset of GCA. The overall risk for cancer after the GCA diagnosis was not increased (SIR 0.98, 95% CI 0.81-1.17). However, there was an increased risk for myeloid leukemia (2.31, 95% CI 1.06-4.39) and a reduced risk for breast cancer (0.33, 95% CI 0.12-0.72) and upper gastrointestinal tract cancer (0.16, 95% 0.004-0.91). Rates of other site-specific cancers were not different from expected. In this Swedish population-based cohort of GCA, the overall risk for cancer was not increased compared to the background population. However, there was an increased risk for leukemia and a decreased risk for breast and upper gastrointestinal tract cancer.

Giant cell arteritis, polymyalgia rheumatica, and late-onset rheumatoid arthritis: Can they be components of a single disease process in elderly patients?

To report two patients with giant cell arteritis (GCA) who developed rheumatoid arthritis (RA) and to review the literature in terms of coexistence of RA, GCA, and polymyalgia rheumatica (PMR). We conducted a comprehensive review of the English literature from 1980 to 2015 to analyze data on the coexistence of GCA and RA. The PubMed, Web of Science, Proquest, and Ovid databases were searched for articles using the term RA combined with temporal arteritis, GCA, and PMR. We identified 17 other cases of coexistent GCA and RA reported in the English literature, together with our 2 cases (19 cases). They included 14 females and 5 males, with a mean age of 74.3 years (range: 57-84) at the time of GCA. The mean age at the time of RA diagnosis was 69.6 years (range 24-83). The average time elapsed between the onset of GCA and the development of RA was 6.7 years (range: 3 month-34 years). RA and GCA were reported as the first disease in 10 cases and 4 cases, respectively. The development of these 2 diseases in a narrow period of time appeared in 4 cases (3 months-19 months). PMR was the first disease in 1 case. RA, GCA, and PMR may appear simultaneously or consecutively; therefore, we suggest that physicians should be alert about such a fact so that a proper diagnosis and treatment could be tailored accordingly.

Visual Complications in Patients with Biopsy-proven Giant Cell Arteritis: A Population-based Study.

To study the clinical and laboratory characteristics of patients with biopsy-proven giant cell arteritis (GCA) with visual complications, and to evaluate the incidence rate of visual complications in GCA compared to the background population. Data from 840 patients with GCA in the county of Skåne, Sweden, diagnosed between 1997 and 2010, were used for this analysis. Cases with visual complications were identified from a diagnosis registry and confirmed by a review of medical records. The rate of visual complications in patients with GCA was compared with an age- and sex-matched reference population. There were 85 patients (10%) who developed ≥ 1 visual complication after the onset of GCA. Of the patients, 18 (21%) developed unilateral or bilateral complete visual loss. The mean age at diagnosis was 78 years (± 7.3); 69% were women. Compared with patients without visual complications, those with visual complication had lower C-reactive protein levels at diagnosis and were less likely to have headache, fever, and palpable abnormal temporal artery. The use of β-adrenergic inhibitors was associated with visual complications. The incidence of visual complications among patients with GCA was 20.9/1000 person-years of followup compared to 6.9/1000 person-years in the reference population, resulting in a rate ratio of 3.0 (95% CI 2.3-3.8). Ten percent of patients with GCA developed visual complications, a rate substantially higher than that of the general population. Patients with GCA who had visual complications had lower inflammatory responses and were more likely to have been treated with β-adrenergic inhibitors compared with patients without visual complications.

SAT0282 Clinical and Laboratory Characteristics Associated with Visual Complications in Patients with Biopsy-Proven GIANT Cell Arteritis

BackgroundEarly diagnosis of giant cell arteritis (GCA) and prompt initiation of high-dose glucocorticoid treatment is important to avoid permanent organ damage. One of the most feared outcomes in GCA are...

Giant Cell Arteritis with or without Aortitis at Diagnosis. A Retrospective Study of 22 Patients with Longterm Followup

Studies have shown that aortitis may be present in half the patients with recent-onset giant cell arteritis (GCA). We assessed whether aortitis at diagnosis affects longterm outcome in patients with GCA. We retrospectively analyzed the longterm outcome of a prospective cohort of 22 patients with biopsy-proven GCA who all had aortic computed tomography (CT) evaluation at the time of diagnosis of GCA between May 1998 and November 1999. Longterm outcome, especially vascular events such as aortic aneurysm, mortality, relapses of GCA, and requirement for steroids, was assessed in 2011 by chart review and patient/physician interviews. At disease onset, 10/22 patients had aortitis on CT scan. Patients with and without aortitis had similar baseline characteristics, including cardiovascular risk profile. At the time of the study, 12/22 (57%) patients had died. Vascular causes of death were more frequent in patients with aortitis (5/7 vs 0/5; p = 0.02). A higher number of vascular events was noted in patients with aortitis (mean events per patient 1.33 vs 0.25; p = 0.009). Stroke was more frequent in patients with aortitis. These patients seemed to exhibit a more chronic or relapsing disease course, and they were less likely to completely discontinue steroid therapy (p = 0.009, log-rank test). Our study suggests for the first time that inflammatory aortic involvement present at onset of GCA could predict a more chronic/relapsing course of GCA, with higher steroid requirements and an increased risk for vascular events in the long term.

New indications for biological therapies

Biological agents have originally been developed to treat refractory arthritis, but evidence has been accruing, supporting their use in vasculitis as well. In the large-vessel vasculitides giant cell arteritis and Takayasu arteritis, TNF-α inhibitors have shown some efficacy in patients with relapsing disease. In contrast, in patients with recent onset of giant cell arteritis, TNF-α inhibitors failed to provide a significant benefit over and above that conferred by glucocorticoids alone. More recent, preliminary data suggest a role for the interleukin-6 receptor antagonist tocilizumab in both resistant and treatment-naïve giant cell arteritis and Takayasu arteritis. Biological agents have also been proposed to treat difficult anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Uncontrolled observations suggest that the TNF-α inhibitor infliximab might be beneficial in resistant cases. On the contrary, a randomized controlled trial did not show superiority of the recombinant human soluble TNF-α p75 receptor fusion protein etanercept over placebo in maintaining remission in granulomatosis with polyangiitis. Two randomized controlled trials have demonstrated that the anti-CD20 monoclonal antibody rituximab was as effective as the standard-of-care agent cyclophosphamide in inducing remission. In addition, rituximab appeared to be superior to cyclophosphamide in inducing remission in the subset of patients with relapsing disease. These findings prove that biological therapy has a role in vasculitis. Research is investigating novel therapies as well as focusing on how to best use the available drugs.

Long-Term Follow-Up of Upper and Lower Extremity Vasculitis Related to Giant Cell Arteritis

We conducted this retrospective study to determine the prevalence of giant cell arteritis (GCA) in patients exhibiting nonatherosclerotic upper and/or lower extremity arterial involvement and to evaluate the clinical features and long-term outcome of those patients.From January 1997 to March 2008, 36 consecutive patients in the Department of Internal Medicine at the University of Rouen medical center received a diagnosis of symptomatic upper/lower extremity vasculitis related to GCA. In the 36 patients, upper/lower extremity vasculitis preceded the initial GCA diagnosis in 7 patients (19.4%), it was identified in association with GCA in 13 patients (36.1%), and it developed after the onset of GCA in the remaining 16 patients (44.4%). GCA clinical manifestations were severe resulting in ischemic complications of the extremities in 10 patients (27.8%). GCA-related large-vessel involvement was located in the upper extremity alone in 21 patients (58.3%), the lower extremity alone in 7 patients (19.4%), and both the upper and lower extremities in 8 patients (22.2%).Arterial involvement in GCA patients with upper extremity vasculitis was distributed in the subclavian (55.6%), axillary (47.2%), and brachial (22.2%) arteries. In patients with lower extremity vasculitis, involvement included the internal iliac artery (11.1%), common femoral artery (13.9%), superficial femoral artery (33.3%), deep femoral artery (5.6%), and popliteal and anterior tibial arteries (5.6%). Aortic localizations were common in GCA patients with upper/lower extremity vasculitis (68.9% of cases).All patients were given steroid therapy at a median daily dose of 1 mg/kg initially. Reconstructive study was performed in 10 patients (27.8%): venous bypass graft (n = 6), angioplasty (n = 1), thromboendarteriectomy (n = 2), or thrombectomy (n = 1); 2 other patients with extremity ischemia underwent amputation. The median observation time was 32 months; the outcome of upper/lower extremity vasculitis was disappearance of clinical symptoms (44.4%), improvement of clinical manifestations (44.4%), and deterioration of clinical manifestations (11.1%). At last follow-up, the median daily dose of prednisone was 6 mg. Steroid therapy could be discontinued in 12 patients (33.3%).We found that upper/lower extremity vasculitis is not uncommon in patients with GCA, and may be present in the early acute phase of GCA. Nevertheless, because upper/lower extremity vasculitis occurs during the course of GCA, yearly clinical vascular examinations may be adequate to screen for upper/lower extremity vasculitis at an early stage in GCA patients. Early diagnosis of GCA-related upper/lower extremity vasculitis is crucial, and can result in decreased severe ischemic complications. Because aortic localizations were common, GCA patients with upper/lower extremity vasculitis should undergo routine investigations for underlying life-threatening aortic complications (aortic ectasia/aneurysm). We also suggest that patients exhibiting aortic complications should undergo routine clinical vascular examination to detect upper/lower extremity vasculitis.

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.

Procalcitonin at the onset of giant cell arteritis and polymyalgia rheumatica: the GRACG prospective study

An epidemic pattern has been reported for GCA and PMR. Immunological studies have shown that an unknown antigen activates the dendritic cells of the adventitia and the type 4 toll-like receptors. Procalcitonin (PCT) is an early marker of bacterial infection. The goal of the study was to assess the level of PCT in GCA and PMR at the onset of the disease. Patients diagnosed during the 2002-06 period were randomly selected. All the 46 patients fulfilled the ACR or the Hunder criteria, and all blood samples were taken before steroid therapy. PCT was normal in all patients. PCT was slightly increased in men (0.087 +/- 0.023 microg/l) compared with women (0.066 +/- 0.027 microg/l) (P = 0.009), and in PMR (0.092 +/- 0.027 microg/l) compared with GCA (0.068 +/- 0.026 microg/l) (P = 0.018). There was no significant correlation with inflammation markers. These results are not in favour of a bacterial trigger for GCA or PMR. Increased PCT levels in patients with inflammatory syndrome, GCA-PMR symptoms and negative temporal artery biopsy may rule out the diagnosis of GCA and PMR.

Qu'a-t-on appris sur la maladie de Horton depuis dix ans ?

Introduction. – We propose a review on the current state of knowledge in giant cell arteritis (GCA), with particular emphasis on advances produced during the past 10 years. Current knowledge and key points. – The physiopathology of GCA is better understood. GCA is considered as a T-cell mediated, antigen driven disease. The expression of interferon- γ greatly influences the clinical expression of the vasculitis. The disease occurs preferentially in women. HLADR4 group and an atheromatous background have been shown to be risk factors for the disease. Infections (parainfluenzae virus, parvovirus B19) may act as triggers at the onset of GCA. In Europe, GCA incidence seems to be increasing in most countries in which it has been studied, especially in women. The diagnostic usefulness of the halo sign detected by high resolution Doppler ultrasonography seems limited. An excess of cardiovascular mortality is described in some countries. There is no consensus regarding the risk factors for thrombotic events. Randomized studies have not proved that methotrexate is a significantly efficient corticosteroid sparing agent. The value of high doses of methylprednisolone for severe thrombotic events has not been established. Benefits and risks of antiaggregation and anticoagulation are unknown. Perspectives. – Numerous important questions regarding the management of GCA remain unresolved. Well designed prospective studies are rare, and necessary in the future.

CD4+ and CD8+ T cell expansions using selected TCR V and J gene segments at the onset of giant cell arteritis.

To investigate T cell receptor (TCR) V alpha/V beta (and in selected cases, J beta) usage in CD4+ and CD8+ peripheral blood lymphocytes of patients with giant cell arteritis (GCA), before and after treatment, as well as to analyze the HLA types of these patients. Flow cytometry, with 10 anti-TCR V-specific monoclonal antibodies (MAb), was used. To analyze J beta usage by cell populations expressing certain V beta, we used the polymerase chain reaction (PCR) technique, with V beta- and C beta-specific primers, Southern blotting of PCR products, and subsequent hybridization with radiolabeled J beta-specific probes. HLA typing was performed using the microlymphocytotoxicity technique. Seven of the 9 GCA patients had increased anti-TCR V MAb reactivities (interpreted as T cell expansions), which in many cases, correlated with clinical signs of disease. A strict preference for particular J beta segments was found in 3 of 3 expanded CD4+ T cell populations. T lymphocytes expressing specific antigen receptors are implicated in the pathogenesis of GCA.

Giant cell arteritis in Jerusalem: a 12-year epidemiological study.

The prevoting conception of the incidence of giant cell arteritis (GCA) among population underscores the very low incidence of this disease among Jews. We studied the epidemiology, ethnic background, and seasonality of GCA among the Jewish population of Jerusalem. Eighty-four patients with biopsy-proven GCA were identified in a period of 12 yr, from 1980-1991. The age, sex and period-adjusted incidence of GCA was 10.2 per 100,000 population aged > or = 50 yr, approximately 20-fold higher than that reported for the Jewish population in Israel for the years 1960-1978. The female:male ratio was 1.6. A declining trend in the annual incidence was observed for men but not for women. The incidence rate was similar among non-western and western Jews. The onset of GCA was more common during the early summer months (May-June).

Seronegative polyarthritis in giant cell arteritis.

Nineteen of 520 patients with biopsy-proven giant cell arteritis were found to have persistent seronegative, symmetric polyarthritis with a mean joint count of 20 (swollen plus tender). In 9 patients in the onset of polyarthritis occurred prior to the diagnosis of giant cell arteritis, 3 had simultaneous onset, and 7 developed polyarthritis within 3 years after the onset of giant cell arteritis. Ten of the 19 patients demonstrated radiographic features of joint space narrowing and/or erosions. In 1 patient in a synovial biopsy was performed, revealing marked multinucleated giant cell infiltration. A persistent seronegative polyarthritis, although uncommon in giant cell arteritis, may be its presenting symptom. Other symptoms of giant cell arteritis should be sought in patients who exhibit this feature, especially in those whose arthritis begins at age 50 or older.