Prevalence Of Giant Cell Arteritis Research Articles

Epidemiology of giant cell arteritis in Waikato, Aotearoa New Zealand.

Giant cell arteritis (GCA) is the most common primary vasculitis in adults over 50 years of age. Our primary objective was to assess the incidence and prevalence of GCA in Waikato in a bid to deepen our understanding of the epidemiology of GCA in Aotearoa New Zealand. From January 2014 to December 2022, cases of GCA were identified prospectively and retrospectively through temporal artery ultrasound request lists and temporal artery biopsy histology reports. Using electronic health records, data were collected retrospectively on patient demographics and clinical features. These were used to calculate the incidence, prevalence and standardised mortality ratio (SMR) of GCA in Waikato. There were 214 patients diagnosed with GCA over the 9-year period. The majority of patients were European (93.9%, 201/214) with Māori patients being significantly younger than European patients. The mean annual incidence of clinical GCA was 14.7 per 100,000 people over 50 years (95% confidence interval [CI] 12.7-16.6). The SMR was 1.18 (95% CI 0.83-1.52). This is the largest study to date on the epidemiology of GCA in Aotearoa New Zealand. The incidence of GCA is comparable to other studies performed in Aotearoa New Zealand and appears to be stable over time. GCA is uncommon in Māori, Pacific Islander and Asian ethnic groups.

Presence of subclinical giant cell arteritis in patients with morning stiffness of duration less than 45 minutes at the time of diagnosis of polymyalgia rheumatica.

In some patients with polymyalgia rheumatica (PMR), giant cell arteritis (GCA) is subclinical as underlying inflammation of large vessels (LV) is present without evidence of related clinical manifestations. Different factors have been proposed as predictive of subclinical GCA in PMR patients. To date, the literature reports scant data about the association between subclinical GCA and long-lasting morning stiffness (MS) in patients at the time of diagnosis of PMR. Given this background, the aim of this study was to assess the association between subclinical GCA and MS < 45 min in patients with newly diagnosed PMR. We performed an observational, retrospective, single-centre cohort study of patients consecutively referred to our public out-of-hospital rheumatologic clinic between January 2015 and December 2020, who could be classified as having PMR according to the 2012 EULAR/ACR criteria. Subclinical GCA was investigated through ultrasound examination of a core set of arteries (temporal, axillary, common carotid, and subclavian arteries), in accordance with the EULAR recommendations for the use of imaging in LV vasculitis. Patients who did not have GCA symptoms but showed halo sign in at least one of these arteries were described as having subclinical GCA. We included a total of 143 patients (35 men and 108 women). Their median age was of 71.5 years. Thirty-five had MS duration < 45 min at the time of PMR diagnosis. Subclinical GCA was found in 23 PMR patients (16.1%); 18 had a cranial and 5 an extracranial GCA. A univariate analysis highlighted that MS < 45 min was associated with a lower prevalence of GCA (OR = 0.11, 95% CI: 0.04-0.29; p < 0.0001). This association was retained in a multivariable analysis that accounted for 6 different potential covariates (OR = 0.06, 95% CI: 0.01-0.26; p < 0.0001. In our study MS < 45 min at the time of PMR diagnosis was associated with a significantly lower risk of subclinical GCA, when patients were screened by ultrasound, of approximately 90%. Identification of a more accurate MS cut-off value could improve the accuracy for subclinical GCA in patients with newly diagnosed PMR.

Prevalence and characteristics of giant cell arteritis in patients with newly diagnosed polymyalgia rheumatica - a prospective cohort study.

It is known that giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) often occur together. So far, the prevalence of GCA in newly diagnosed PMR patients has not been evaluated in a prospective ultrasound study. The aim of this study was to assess the prevalence of GCA using vascular ultrasound in patients with newly diagnosed PMR. A consecutive cohort of newly diagnosed PMR patients was prospectively evaluated for the presence of GCA with the use of systematic musculoskeletal and vascular ultrasound examination. Overall, 60 patients with newly diagnosed PMR were prospectively enrolled. Symptoms and laboratory findings were collected. All patients underwent ultrasound of shoulder and hip joints, and vascular ultrasound evaluating the facial, temporal, carotid, vertebral and axillary arteries. Patients were diagnosed with GCA if they had ultrasound imaging findings of GCA. Patients with PMR (PMR-group) and patients with PMR and GCA (PMR-GCA-group) were compared, and a C-reactive protein (CRP) cut-off value was evaluated. GCA was diagnosed in 28 of 60 PMR patients (46%). The PMR-group consisted of 20 (62.5%) females with a mean age of 69 (±9.9) years, while the PMR-GCA-group consisted of 11 (39.3%) females with a mean age of 74 (±8.4) years. In 13 of 28 patients (46%) in the PMR-GCA-group, GCA was subclinical and only diagnosed by ultrasound. The PMR-GCA-group showed higher values of joint effusion and significantly higher CRP values. A CRP cut-off value of 26.5 mg/litre (reference range 0-5 mg/litre) yielded a sensitivity of 66% with a specificity of 73% for GCA. GCA was found in 46% of newly diagnosed PMR patients; 22% of the patients with PMR had asymptomatic GCA. Joint effusions were higher in the PMR-GCA-group, with significant results for the hip joint. A CRP cut-off value of ⩾26.5 mg/litre in PMR can help in the identification of subclinical GCA.

Incidence and Prevalence of Polymyalgia Rheumatica and Giant Cell Arteritis in a Healthcare Management Organization in Buenos Aires, Argentina.

To estimate incidence and prevalence of polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) in a university hospital-based health management organization (Hospital Italiano Medical Care Program) in Argentina. Overall and sex-specific incidence rates (IRs) and prevalence were calculated (age ≥ 50 yrs). Incidence study followed members with continuous affiliation ≥ 1 year from January 2000 to December 2015. Diagnosis as per the 2012 European Alliance of Associations for Rheumatology/American College of Rheumatology (ACR) criteria for PMR or the ACR 1990 criteria for GCA. Prevalence was calculated on January 1, 2015. There were 176,558 persons who contributed a total of 1,046,620 person-years (PY). Of these, 825 developed PMR, with an IR (per 100,000 PY) of 78.8 (95% CI 73.4-84.2) overall, 90.1 (95% CI 82.9-97.2) for women, and 58.9 (95% CI 51.1-66.6) for men. Ninety persons developed GCA; the IR was 8.6 (95% CI 6.8-10.4) overall, 11.1 (95% CI 8.5-10.6) for women, and 4.2 (2.2-6.3) for men. There were 205 prevalent PMR cases and 23 prevalent GCA cases identified from a population of 80,335. Prevalence of PMR was 255 per 100,000 (95% CI 220-290) overall, 280 (95% CI 234-325) for women, and 209 (95% CI 150-262) for men; and the prevalence of GCA was 28.6 per 100,000 (95% CI 16.9-40.3) overall, 36.4 (95% CI 20.1-52.8) for women, and 14.2 (95% CI 0.3-28.1) for men. This is the first study of incidence and prevalence of PMR and GCA in Argentina. There were similarities and differences with cohorts from other parts of the world, but population-based epidemiologic studies in Latin America are needed.

Subclinical giant cell arteritis in new onset polymyalgia rheumatica A systematic review and meta-analysis of individual patient data

ObjectivesTo determine the prevalence and predictors of subclinical giant cell arteritis (GCA) in patients with newly diagnosed polymyalgia rheumatica (PMR). MethodsPubMed, Embase, and Web of Science Core Collection were systematically searched (date of last search July 14, 2021) for any published information on any consecutively recruited cohort reporting the prevalence of GCA in steroid-naïve patients with PMR without cranial or ischemic symptoms. We combined prevalences across populations in a random-effect meta-analysis. Potential predictors of subclinical GCA were identified by mixed-effect logistic regression using individual patient data (IPD) from cohorts screened with PET/(CT). ResultsWe included 13 cohorts with 566 patients from studies published between 1965 to 2020. Subclinical GCA was diagnosed by temporal artery biopsy in three studies, ultrasound in three studies, and PET/(CT) in seven studies. The pooled prevalence of subclinical GCA across all studies was 23% (95% CI 14%-36%, I2=84%) for any screening method and 29% in the studies using PET/(CT) (95% CI 13%-53%, I2=85%) (n=266 patients). For seven cohorts we obtained IPD for 243 patients screened with PET/(CT). Inflammatory back pain (OR 2.73, 1.32-5.64), absence of lower limb pain (OR 2.35, 1.05-5.26), female sex (OR 2.31, 1.17-4.58), temperature >37° (OR 1.83, 0.90-3.71), weight loss (OR 1.83, 0.96-3.51), thrombocyte count (OR 1.51, 1.05-2.18), and haemoglobin level (OR 0.80, 0.64-1.00) were most strongly associated with subclinical GCA in the univariable analysis but not C-reactive protein (OR 1.00, 1.00-1.01) or erythrocyte sedimentation rate (OR 1.01, 1.00-1.02). A prediction model calculated from these variables had an area under the curve of 0.66 (95% CI 0.55-0.75). ConclusionMore than a quarter of patients with PMR may have subclinical GCA. The prediction model from the most extensive IPD set has only modest diagnostic accuracy. Hence, a paradigm shift in the assessment of PMR patients in favour of implementing imaging studies should be discussed.

Is There an Association between Herpetic Infections and Giant Cell Arteritis? A Population-Based Study.

Recent data suggests that herpes zoster (HZ) and herpes simplex virus (HSV) may be one of the underlying immunological triggers for giant cell arteritis (GCA). However, there is limited population-based data to support this. Our goal was to determine if herpetic infections increase the likelihood of GCA in the British Columbia (BC) population. The background prevalence of GCA was compared to the prevalence of GCA in subjects with HZ and HSV using diagnostic billing code data from an online BC database (BC Data ScoutTM). BC residents ≥30 years old at the time of diagnosis from January 2000 to January 2019 were included. The relevant International Classification of Disease codes was used to identify patients with GCA, HZ, and HSV. Comparisons were made using two-sample Z tests. There were 4315 GCA diagnoses, from a total population of 3,026,005 subjects. The prevalence of GCA was 143 per 100,000 people. In terms of herpetic infections, 850 GCA cases were identified in 249,900 subjects with HZ versus 310 diagnoses of GCA in 163,170 subjects with HSV. The prevalence of GCA in subjects with HZ (0.340%) was significantly higher than the prevalence of GCA (0.143%) in the general population (p < 0.00001). The prevalence of GCA in HSV subjects (0.190%) was also significantly higher (p < 0.00001) than the population prevalence but lower than (p < 0.00001) the GCA with HZ prevalence. The likelihood of GCA appears to increase with herpetic infections, more significantly with HZ.

Comment on: Incidence and prevalence of giant cell arteritis in Ontario, Canada.

Comment on: Incidence and prevalence of giant cell arteritis in Ontario, Canada.

Comment on: Incidence and prevalence of giant cell arteritis in Ontario, Canada: reply.

Comment on: Incidence and prevalence of giant cell arteritis in Ontario, Canada: reply.

Corrigendum to: Incidence and prevalence of giant cell arteritis in Ontario, Canada.

Corrigendum to: Incidence and prevalence of giant cell arteritis in Ontario, Canada.

Incidence and prevalence of giant cell arteritis in Ontario, Canada.

To estimate trends in the incidence and prevalence of GCA over time in Canada. We performed a population-based study of Ontario health administrative data using validated case definitions for GCA. Among Ontario residents ≥50 years of age we estimated the annual incidence and prevalence rates between 2000 and 2018. We performed sensitivity analyses using alternative validated case definitions to provide comparative estimates. Between 2000 and 2018 there was a relatively stable incidence over time with 25 new cases per 100000 people >50 years of age. Age-standardized incidence rates were significantly higher among females than males [31 cases (95% CI: 29, 34) vs 15 cases (95% CI: 13, 18) per 100000 in 2000]. Trends in age-standardized incidence rates were stable among females but increased among males over time. Incidence rates were highest among those ≥70 years of age. Standardized prevalence rates increased from 125 (95% CI 121, 129) to 235 (95% CI 231, 239) cases per 100000 from 2000 to 2018. The age-standardized rates among males rose from 76 (95% CI 72, 81) cases in 2000 to 156 (95% CI 151, 161) cases per 100000 population in 2018. Between 2000 and 2018, the age-standardized rates among females similarly increased over time, from 167 (95% CI 161, 173) to 304 (95% CI 297, 310) cases per 100000 population. The incidence and prevalence of GCA in Ontario is similar to that reported in the USA and northern Europe and considerably higher than that reported for southern Europe and non-European populations.

Data linkage analysis of giant cell arteritis in Italy: Healthcare burden and cost of illness in the Italian region of Friuli Venezia Giulia (2001-2017).

Giant cell arteritis (GCA) is the most common vasculitis in adults. However, comprehensive analyses of the healthcare burden are still scarce. The aim of the study is to report the healthcare burden and cost of illness of GCA in the Friuli Venezia Giulia (FVG) region of Italy, based on a data linkage analysis. To this end, a retrospective study was conducted through the integration of many administrative health databases of the FVG region as the source of information. Cases were identified from two verified, partially overlapping sources (the rare disease registry and medical exemption database). From 2001 to 2017, 208 patients with GCA were registered. The prevalence of GCA in the population aged ⩾ 45 years as of December 31, 2017 was 27.2/100,000 inhabitants (95% CI 23.5-31.4). The mean time of observation was 4.5 ± 3.6 years. A total of 3182 visits (338 per 100 patient-years) was recorded. The most frequent specialty visits were rheumatology (n = 610, 19.2%), followed by internal medicine (n = 564, 17.7%). A total of 287 hospitalizations (30 per 100 patient-years) were reported. A total of 13,043 prescriptions (1386 per 100 patient-years) were registered. More than half of the patients were prescribed an immunosuppressive agent. The overall estimated direct healthcare cost was €2,234,070, corresponding to €2374 per patient-year. Overall, GCA is a rare disease which implies a high healthcare cost.

166. PREVALENCE OF BIOPSY-PROVEN GIANT CELL ARTERITIS IN SOUTHERN SWEDEN

Background: Few previous studies have examined the prevalence of giant cell arteritis (GCA). The main objective of this study is to investigate the prevalence of temporal artery positive GCA (TAB+GCA) in southern Sweden.Methods: The study area is the region of Skane in southern Sweden with a population of 1,3 million inhabitants (36% aged ⩾50 years). To be included in the study, patient should fulfil the following criteria: 1) diagnosed with TAB+GCA after 1997, 2) living in Skane at date of diagnosis and date of point prevalence (p.p.) on December 31st, 2014, and 3) on oral glucocorticosteroids (GCs) treatment at the date of p.p. (that redeemed at least one GC prescription during 12 months preceding the date of p.p.) The national Swedish prescribed drug register (SPDR) has been used to identify patients still on GC treatment. The SPDR include data on all the purchased prescriptions since July 2005 and data available through October 2015. Using the unique Swedish personal identification number (PIN), the GCA cohort has been linked to the SPDR. Accordingly, for the prevalence estimate, the numerator was all patients with TAB+GCA who fulfil the study criteria and the denominator was all people aged ⩾50 years living in Skane at date of p.p. The ATC-codes of Betamethasone, Dexamethasone, Prednisolone, Prednisone and Methylprednisolone were identified.Results: At the date of p.p. estimate a total of 631 patients were alive and living in the study area. Of them 359 patients were on GCs at date of p.p. according to the study definition. Accordingly, the p.p. of TAB+ GCA per 100 000 inhabitants aged ⩾50 years was 75.3 (95% CI 67.5-83.1); 40.5 (32.2-48.8) for men and 107 (94.2-119.9) for women. The median time of being on GCs (from time of diagnosis to date of p.p.) 2.23 years (IQR 0.96-4.40). The percentage of patients with positive TAB who were alive at date of p.p. without GC treatment was 43.1%.Conclusion: This is the first estimate of prevalence of TAB+GCA from Sweden. Our prevalence estimate takes into account that GCA may be cured with patients no longer regarded as prevalent cases.Disclosures: Lund University (Less)

Contemporary prevalence estimates for giant cell arteritis and polymyalgia rheumatica, 2015.

There are no estimates of the prevalence of giant cell arteritis (GCA) or polymyalgia rheumatica (PMR) in a US population in the current millennium. The purpose of this study was to estimate the 2015 prevalence of GCA and PMR in a US population-based setting. Cohorts of incident GCA in 1950-2009 and incident PMR in 1970-2014 were previously identified among residents of Olmsted County, Minnesota. Prevalence estimates were obtained from the age-, sex-, and calendar year-specific incidence rates and adjusted to the US white 2010 population aged ≥50 years. Confidence intervals (CI) were computed using bootstrap sampling. There were 248 incident cases of GCA in 1950-2009 and 790 cases of PMR in 1970-2014. The overall age- and sex-adjusted prevalence rate of GCA on January 1, 2015 was 204 (95% CI: 161-254) per 100,000 population aged ≥50 years. GCA prevalence rate in women was 304 (95% CI: 229-375) and in men was 91 (95% CI: 46-156) per 100,000 population. The overall age and sex adjusted prevalence rate of PMR on January 1, 2015 was 701 (95% CI: 651-750) per 100,000 population. PMR prevalence rate in women was 870 (95% CI: 787-957) and in men was 508 (95% CI: 426-589) per 100,000 population. Prevalence rates of GCA and PMR in 2015 are similar to previously published prevalence estimates in the late 1990s. The health burden of these diseases among older persons continues to be substantial and reflects the need for continued efforts to better manage it.

Incidence, Prevalence, and Survival of Biopsy-Proven Giant Cell Arteritis in Northern Italy During a 26-Year Period.

To investigate the epidemiology and mortality in patients with biopsy-proven giant cell arteritis (GCA) in northern Italy. All patients with incident temporal-artery biopsy-positive GCA, diagnosed between 1986 and 2012 and living in the Reggio Emilia area, were identified by using a pathology register and by reviewing all histopathologic specimens. For each patient, we identified 1 comparison subject from the same geographic area, matched for age and sex. Mortality rates and specific causes of death were reported. There were 285 incident cases of biopsy-proven GCA (210 women) during the 26-year study period. The overall age- and sex-adjusted incidence per 100,000 persons ages ≥50 years was 5.8 (95% confidence interval [95% CI] 5.1, 6.5). Incidence was significantly higher in women (7.8 [95% CI 6.7, 8.9]) than in men (3.3 [95% CI 2.6, 4.1]) (P < 0.0001). Annual age- and sex-adjusted incidence rates significantly increased by 15.9% per 3 years from 1986 to 2000, then significantly fell by -4.8% per 3 years from 2001-2012. The prevalence of GCA on December 31, 2012 was 87.9 (95% CI 75.8, 101.4). No significant differences in the mortality rates were observed between GCA patients (4.9 per 100 person-years [95% CI 4.1, 5.8]) and non-GCA subjects (5.6 [95% CI 4.7, 6.6]). No significant differences in causes of death were observed comparing GCA patients to non-GCA subjects. This large population-based study of biopsy-proven GCA confirmed the lower incidence of GCA in Mediterranean countries and did not observe any increased mortality risk.

The prevalence of giant cell arteritis and polymyalgia rheumatica in a UK primary care population.

BackgroundTo update community-based prevalence values for Polymyalgia Rheumatic (PMR) and Giant Cell Arteritis (GCA) using case record review supplemented by population survey and subsequent clinical review.MethodsClinical data were obtained from case records of a large primary care practice in Norfolk, UK and reviewed for diagnoses of GCA and PMR. In addition postal survey was carried out to capture potentially undiagnosed cases within the practice population. Those screening positive for potential diagnoses of GCA and PMR were invited for clinical review. A cumulative prevalence estimate was subsequently calculated on those diagnosed within the GP practice and subsequently on those fulfilling the various published classification criteria sets. The date of the database lock and mail merge was March 2013.ResultsThrough detailed systematic review of 5,159 GP case records, 21 patients had a recorded diagnosis of GCA and 117 had PMR.No new cases were identified among 2,227 completed questionnaires returned from the population survey of a sample of 4,728. The resulting cumulative prevalence estimate in those aged ≥ 55 years meeting the ACR classification criteria set for GCA was 0.25 % (95 % CI 0.11 to 0.39 %) and for five published criteria sets for PMR ranged from 0.91 to 1.53 % (95 % CI ranges 0.65 %, 1.87 %). The prevalence of both conditions was higher in women than in men and in older age groups.ConclusionThis study provides the first UK prevalence estimate of GCA and PMR in over 30 years and is the first to apply classification criteria sets.

043. The Prevalence of Giant Cell Arteritis and Polymyalgia Rheumatica in a UK Primary Care Population

To update community-based prevalence values for Polymyalgia Rheumatic (PMR) and Giant Cell Arteritis (GCA) using case record review supplemented by population survey and subsequent clinical review. Clinical data were obtained from case records of a large primary care practice in Norfolk, UK and reviewed for diagnoses of GCA and PMR. In addition postal survey was carried out to capture potentially undiagnosed cases within the practice population. Those screening positive for potential diagnoses of GCA and PMR were invited for clinical review. A cumulative prevalence estimate was subsequently calculated on those diagnosed within the GP practice and subsequently on those fulfilling the various published classification criteria sets. The date of the database lock and mail merge was March 2013. Through detailed systematic review of 5,159 GP case records, 21 patients had a recorded diagnosis of GCA and 117 had PMR.No new cases were identified among 2,227 completed questionnaires returned from the population survey of a sample of 4,728. The resulting cumulative prevalence estimate in those aged ≥ 55 years meeting the ACR classification criteria set for GCA was 0.25 % (95 % CI 0.11 to 0.39 %) and for five published criteria sets for PMR ranged from 0.91 to 1.53 % (95 % CI ranges 0.65 %, 1.87 %). The prevalence of both conditions was higher in women than in men and in older age groups. This study provides the first UK prevalence estimate of GCA and PMR in over 30 years and is the first to apply classification criteria sets.

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.

Giant Cell Arteritis: Clinical Features of Patients Visiting a Headache Clinic in Japan

The first symptom of giant cell arteritis (GCA) is usually a headache. Japan has a low prevalence of GCA, and clinical features of this disorder have not been fully investigated. We conducted a retrospective evaluation of clinical features in patients with giant cell arteritis who visited a headache clinic in Japan. Clinical and demographic data were obtained from clinical examinations, face-to-face interviews, and hospital records. Subjects comprised 19 patients (9 men, 10 women). Mean age at disease onset was 78.1 ± 4.8 years (range, 71-86 years). Seventeen of 19 patients (89.5%) had consulted other medical institutions before consulting our hospital, but only 2 of those patients had been diagnosed with GCA at these medical institutions. Manifestations at disease onset included headache (89.5%), ear pain (5.3%), and jaw pain (5.3%). Ocular manifestations were reported in 2 patients (10.5%). No loss of vision occurred. One patient showed trigeminal nerve palsy involving the third division of the nerve. Jaw claudication was observed in 3 patients (15.8%). Concomitant polymyalgia rheumatica was seen in 3 patients (15.8%). No patient showed upper respiratory tract symptoms, arm claudication, or aortic aneurysms. Although most patients had consulted other medical institutions before consulting our hospital, they were not diagnosed with GCA at these institutions. Infrequent clinical findings of GCA and lack of symptoms other than headache may contribute to the high rate of unrecognized and misdiagnosed cases of GCA.

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.

The Geo-epidemiology of Temporal (Giant Cell) Arteritis

Giant cell arteritis (GCA) is the most common vasculopathy in patients over the age of 50. The majority of data on the geo-epidemiology of GCA is derived from Scandinavia, although there is very good documentation and epidemiological descriptions from studies throughout Europe and North America. There remains, however, a paucity of data on the incidence and prevalence of GCA in North American minority populations, as well as from Africa or Asia. The data that does exist suggests that the incidence of GCA is lower in Hispanic, Asian, and African American populations. It is interesting to note that as the population throughout the world continues to age, we anticipate an increased prevalence of disease based upon increases in annual incidence and improved survival. Considerable research is still needed to identify genetic, environmental, and gender-specific factors that influence not only the etiology, but also the natural history of disease.