Myeloproliferative neoplasms and clonal haematopoiesis in patients with giant cell arteritis: a case-control and exploratory study.

OBJECTIVES: GCA is a large vessel vasculitis for which triggering factors remain unknown. Clonal haematopoiesis (CH) was associated with atherosclerosis through the induction of inflammation in myeloid cells, and data suggest that CH expansion and inflammation may support each other to induce a pro-inflammatory loop. Our objective was to describe the impact of JAK2p.V617F-mutated myeloproliferative neoplasms (MPNs) on GCA and to screen MPN-free patients for CH mutations. METHODS: We performed a retrospective case-control study comparing the characteristics of 21 GCA patients with MPN and 42 age- and gender-matched GCA patients without MPN. Also, 18 GCA patients were screened for CH through next-generation sequencing (NGS). RESULTS: The most frequent associated MPN was essential thrombocythaemia (ET; n = 11). Compared with controls, GCA patients with MPN had less-frequent cephalic symptoms (71.4 vs 97.6%; P = 0.004) and higher platelet counts at baseline [485 × 109/l (interquartile range 346-586) vs 346 (296-418); P = 0.02]. There was no difference between groups for other clinical features. Overall survival was significantly shorter in patients with MPN compared with controls [hazard ratio 8.2 (95% CI 1.2, 56.6); P = 0.03]. Finally, screening for CH using NGS in 15 GCA patients without MPN revealed CH in 33%. CONCLUSION: GCA patients with MPN display higher platelet counts and shorter overall survival than controls. This association is not fortuitous, given the possible pathophysiological relationship between the two diseases. CH was found in one-third of GCA patients, which may be higher than the expected prevalence for a similar age, and should be confirmed in a larger cohort.

All-cause and cause-specific mortality in patients with giant cell arteritis: a nationwide, population-based cohort study.

OBJECTIVES: To investigate whether GCA is associated with increased all-cause and cause-specific mortality. METHODS: A nationwide, population-based cohort study in Denmark using medical and administrative registries. GCA cases were defined as patients aged ≥50 years from 1996-2018 with a first-time discharge diagnosis of GCA and ≥3 prescriptions for prednisolone within 6 months following diagnosis. Each GCA patient was matched based on age, sex and calendar time to 10 persons without a history of GCA. Index date was the date for the third prednisolone prescription. We used a pseudo-observation approach to calculate all-cause and cause-specific mortality, adjusted risk differences (RDs) and relative risks (RRs). RESULTS: We included 9908 GCA patients and 98 204 persons from the general population. The median time for GCA patients to redeem the third prednisolone prescription was 74 days [interquartile range (IQR: 49-106)]. Among GCA patients, the overall mortality was 6.4% (95% CI: 5.9, 6.9) 1 year after index date and 45% (95% CI: 44, 47) after 10 years. Compared with the reference cohort, adjusted RDs and RRs of deaths in the GCA cohort were 2.2% (95% CI: 1.7, 2.7) and 1.49 (95% CI: 1.36, 1.64) after 1 year, and 2.1% (95% CI: 1.0, 3.3) and 1.03 (95% CI: 1.00, 1.05) 10 years after index date. GCA patients had a higher risk of death due to infectious, endocrine, cardiovascular and gastrointestinal diseases. CONCLUSIONS: GCA is associated with increased all-cause mortality, particularly within the first year following the diagnosis. Cause-specific mortality indicates that mortality in GCA may in part be due to glucocorticoid-related complications.

Real-life analysis of the causes of death in patients consecutively followed for giant cell arteritis in a French centre of expertise.

OBJECTIVES: To describe, in a real-life setting, the direct causes of death in a cohort of consecutive patients with GCA. METHODS: We retrospectively analysed the deaths that occurred in a cohort of 470 consecutive GCA patients from a centre of expertise between January 2000 and December 2019. Among the 120 patients who died, we retrieved data from the medical files of 101 patients. RESULTS: Cardiovascular events were the dominant cause of death (n = 41, 41%) followed by infections (n = 22, 22%), geriatric situations (i.e. falls or senile deterioration; n = 17, 17%) and cancers (n = 15, 15%). Patients in each of these four groups were compared with the other deceased patients pooled together. Patients who died from cardiovascular events were more frequently male (46 vs 27%; P = 0.04) with a past history of coronary artery disease (29 vs 8%; P = 0.006). Patients who died from infections mostly had ongoing glucocorticoid treatment (82 vs 53%; P = 0.02) with higher cumulative doses (13 994 vs 9150 mg; P = 0.03). Patients who died from geriatric causes more frequently had osteoporosis (56 vs 17%; P = 0.0009) and had mostly discontinued glucocorticoid treatment (76 vs 33%; P = 0.001). The predictive factors of death in multivariate analysis were a history of coronary disease [hazard ratio (HR) 2.39; 95% CI 1.27, 4.21; P = 0.008], strokes at GCA diagnosis (HR 2.54; 95% CI 1.05, 5.24; P = 0.04), any infection during follow-up (HR 1.93; 95% CI 1.24, 2.98; P = 0.004) and fever at GCA diagnosis (HR 1.99; 95% CI 1.16, 3.28; P = 0.01). CONCLUSION: Our study provides real-life insight on the cause-specific mortality in GCA patients.

Incidence Trends and Mortality of Giant Cell Arteritis in Southern Norway.

OBJECTIVE: Southern Norway consists of a homogeneous population of nearly 300,000 inhabitants and is an ideal epidemiologic setting. We aimed to explore potential changes in incidence of giant cell arteritis (GCA) in Southern Norway from 2000-2013, with comparisons of previous reports from the same population cohort from 1987-1994 and 1992-1996, and to investigate the mortality rates of GCA over a period of 14 years. METHODS: All patients diagnosed with GCA during January 1, 2000 to December 31, 2013 were identified through the electronic health records and biopsy findings databases at our clinic. The diagnosis of GCA and information about death was confirmed by reviewing the patients' hospital records. Inclusion criteria were: 1) fulfillment of the American College of Rheumatology 1990 criteria for GCA, or 2) histologically proven GCA, or 3) confirmed arteritis of the large or medium-sized vessels by imaging. RESULTS: A total of 206 patients were included, and 147 (72%) were females. The annual incidence rate of GCA per 100,000 inhabitants age ≥50 years was 16.8 (95% confidence interval [95% CI] 14.6-19.2), 24.5 for females (95% CI 19.2-26.5), and 10.2 for males (95% CI 7.9-13.2). Forty-six patients (22%) died (24 women, 22 men). The overall standardized mortality ratio was 1.05 (95% CI 0.77-1.38), 0.92 for females (95% CI 0.61-1.35), and 1.38 for males (95% CI 0.88-2.05). Overall survival rate was significantly higher in females compared to males (P < 0.001). CONCLUSION: GCA incidence is not increasing. We did not find excess mortality; however, males seem to have a worse survival rate compared to females.

Morbidity and Mortality of Large-Vessel Vasculitides.

PURPOSE OF REVIEW: Giant cell arteritis (GCA) and Takayasu arteritis (TAK) comprise the primary systemic large-vessel vasculitides. In these conditions, arterial stenosis, occlusion, aneurysm, and dissection can lead to severe disease-related consequences. This review focuses on disease-related manifestations of GCA and TAK, emphasizing the impact of these findings on long-term morbidity and mortality. RECENT FINDINGS: Vision loss remains a main contributor of morbidity in GCA. Non-invasive imaging allows for recognition of aortic disease in GCA but monitoring and intervention guidelines require further development. TAK represents a severe disease of early-onset with high risk of morbidity due to aortic, pulmonary, cardiovascular, and neurologic involvement. Overall, patients with GCA have similar mortality rates to comparators but mortality is notably higher than the general population in TAK. A multidisciplinary approach of expert subspecialists is required to assist with the complex care of patients with GCA and TAK in order to appropriately surveil, identify, and address the multi-faceted co-morbidities of these diseases.

Giant cell arteritis: a diagnostic challenge in an older adult / Arterite de células gigantes: um desafio diagnóstico em um idoso

Giant cell arteritis is a large-vessel vasculitis usually seen in older adults. The inflammatory process results in systemic, ophthalmic, and neurological lesions. It is difficult to diagnose in older adults and may present as a medical emergency. Here, we report the case of an 83-year-old woman who presented with bitemporal headache, jaw claudication, glossodynia, failure to thrive, and amaurosis fugax. The findings supported the hypothesis of giant cell arteritis. Despite receiving treatment, the patient died of an acute myocardial infarction. Headache in older adults raises the possibility of giant cell arteritis, especially when combined with an ophthalmic emergency. Many symptoms indicate the condition, but the diagnosis may be challenging, especially for the generalist physician.

A arterite de células gigantes é uma vasculite de grandes vasos geralmente observada em adultos mais velhos. O processo inflamatório resulta em lesões sistêmicas, oftalmológicas e neurológicas. É de difícil diagnose em adultos mais velhos e pode se apresentar como uma emergência médica. Apresentamos o caso de uma mulher de 83 anos que apresentou cefaleia bitemporal, claudicação da mandíbula, glossodinia, incapacidade de prosperar e amaurose fugaz. Os resultados apoiaram a hipótese de arterite de células gigantes. Apesar de receber tratamento, o paciente morreu por infarto agudo do miocárdio. Dor de cabeça em idosos aumenta a possibilidade de arterite de células gigantes, especialmente quando combinada com uma emergência oftalmológica. Muitos sintomas indicam a condição, mas o diagnóstico pode ser desafiador, especialmente para o médico generalista.

Familial aggregation of longevity in giant cell arteritis and polymyalgia rheumatica.

The long-term mortality in giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) is unexpectedly decreased or at least not increased regardless of several mortality risk factors that these diseases share with other chronic immune-mediated rheumatic diseases. The genetic and immunological profile of PMR/GCA patients is unique, therefore, the hypothesis that this profile provides some survival advantage to PMR/GCA patients should be considered. The longevity is a phenomenon that was demonstrated to be familial. The familial aggregation of longevity can be studied by analysis of life expectancy in family members. Here we test the hypothesis of the aggregation of an increased longevity in the families of PMR/GCA patients. We compared the age of death of 358 parents of 179 PMR and GCA patients with corresponding data retrieved from 506 parents of 253 randomly collected age and sex-matched controls. The number of nonagenarian (≥ 90-year -old) mothers of PMR/GCA patients was significantly higher (OR = 2.34, 95%CI 1.11-11.95, p < 0.0005) vs controls. Both nonagenarian parents were found in 6 patients (3.35%) and none in the control cohort (OR = 8.77, 95%CI 2.26-405.10, p = 0.003). Our data suggest the familial aggregation of nonagenarians in PMR/GCA patients.

Survival of patients with giant cell arteritis: a controversial issue.

OBJECTIVES: Epidemiologic studies differ regarding overall survival in giant cell arteritis (GCA). In this review we evaluated longevity and the impact of several disease parameters on survival of GCA patients. METHODS: Review of the medical literature during the period 1975-2018, using PubMed database. RESULTS: Epidemiologic studies addressing the issue of survival in GCA patients used variable methods of calculating mortality rates in relation to background population or in relation to selected controls. Several epidemiologic studies found that survival of GCA patients was similar to that of the general population. Others reported increased mortality in patients with GCA, or in subgroups of GCA patients. 5-Year and 10-year survival rates differed considerably among studies: 5-year survival rates ranged between 60-90% (except for 2 extremes of 35% and 97%), and 10-year survival rates ranged between 48-81%. Reasons for these discrepancies are unclear, and may be related to differences in populations, in the period of the study, and in study methods. Several studies found that mortality was increased in female GCA patients, and some reported increased mortality early in the course of the disease (mostly within the first 2 years after diagnosis). The deleterious effect of vision loss on survival was noted in a few studies, although most studies did not address the issue of mortality in this particular subgroup of GCA patients. CONCLUSIONS: Epidemiologic studies varied considerably in the reported outcomes of GCA patients: some found that the overall survival was similar to that of the general population while others reported increased mortality in GCA or in subgroups of GCA patients.

Giant cell arteritis-related aortitis with positive or negative temporal artery biopsy: a French multicentre study.

Objective: To compare the clinical presentation and outcome of giant cell arteritis (GCA)-related aortitis according to the results of temporal artery biopsy (TAB).Method: Patients with GCA-related aortitis diagnosed between 2000 and 2017, who underwent TAB, were retrospectively included from a French multicentre database. They all met at least three American College of Rheumatology criteria for the diagnosis of GCA. Aortitis was defined by aortic wall thickening > 2 mm on computed tomography scan and/or an aortic aneurysm, associated with an inflammatory syndrome. Patients were divided into two groups [positive and negative TAB (TAB+, TAB-)], which were compared regarding aortic imaging characteristics and aortic events, at aortitis diagnosis and during follow-up.Results: We included 56 patients with TAB+ (70%) and 24 with TAB- (30%). At aortitis diagnosis, patients with TAB- were significantly younger than those with TAB+ (67.7 ± 9 vs 72.3 ± 7 years, p = 0.022). Initial clinical signs of GCA, inflammatory parameters, and glucocorticoid therapy were similar in both groups. Coronary artery disease and/or lower limb peripheral arterial disease was more frequent in TAB- patients (25% vs 5.3%, p = 0.018). Aortic wall thickness and type of aortic involvement were not significantly different between groups. Diffuse arterial involvement from the aortic arch was more frequent in TAB- patients (29.1 vs 8.9%, p = 0.03). There were no differences between the groups regarding overall, aneurism-free, relapse-free, and aortic event-free survival.Conclusion: Among patients with GCA-related aortitis, those with TAB- are characterized by younger age and increased frequency of diffuse arterial involvement from the aortic arch compared to those with TAB+, without significant differences in terms of prognosis.

Survival and death causes of patients with giant cell arteritis in Western Norway 1972-2012: a retrospective cohort study.

BACKGROUND: Our objective was to determine the survival and causes of death in a large and well-characterized cohort of patients with giant cell arteritis (GCA). METHODS: This is a hospital-based, retrospective, observational cohort study including patients diagnosed with GCA in Western Norway during 1972-2012. Patients were identified through computerized hospital records using the International Classification of Diseases (ICD)-coding system. Medical records were reviewed. Patients were randomly assigned population controls matched on age, sex, and geography from the Central Population Registry of Norway (CPRN). Date and cause of death were obtained from the Norwegian Cause of Death Registry (NCoDR). The survival was analyzed using Kaplan-Meier methods with the Gehan-Breslow test and the causes of death using cumulative incidence and Cox models for competing risks. RESULTS: We identified 881 cases with a clinical diagnosis of GCA of which 792 fulfilled the American College of Rheumatology (ACR) 1990 classification criteria. Among those fulfilling the ACR criteria, 528 were also biopsy-verified. Cases were matched with 2577 population controls. A total of 490 (56%) GCA patients and 1517 (59%) controls died during the study period. We found no difference in the overall survival of GCA patients compared to controls, p = 0.413. The most frequent underlying causes of death in both groups were diseases of the circulatory system followed by cancer. GCA patients had increased risk of dying of circulatory disease (HR 1.31, 95% CI 1.13-1.51, p < 0.001) but lower risk of dying of cancer (HR 0.56, 95% CI 0.42-0.73, p < 0.001) compared to population controls. CONCLUSIONS: We found no difference in the overall survival of GCA patients compared to matched controls, but there were differences in the distribution of underlying death causes.

Survival predictors in biopsy-proven giant cell arteritis: a northern Italian population-based study.

OBJECTIVE: To evaluate the influence of disease-related findings and treatment outcomes on survival in a population-based cohort of Northern Italian patients with GCA. METHODS: A total of 281 patients with incident temporal artery biopsy (TAB)-proven GCA, diagnosed over a 26-year period (1986-2012) and living in the Reggio Emilia area, were retrospectively evaluated. We analysed clinical, imaging and laboratory findings at diagnosis, pathological patterns of TAB, CS treatment and therapeutic outcomes, and traditional cardiovascular risk factors as factors predictive of survival. RESULTS: Univariate analysis showed that increased mortality was associated with large vessel involvement at diagnosis [hazard ratio (HR) 5.84], while reduced mortality was associated with female sex (HR 0.66), PMR (HR 0.54), higher haemoglobin levels (HR 0.84) at diagnosis, long-term remission (HR 0.47) and inflammation limited to adventitia or to the adventitial vasa vasorum (HR 0.48) at TAB examination. Multivariate analysis confirmed the association between increased mortality and large vessel involvement (HR 5.14) at diagnosis, between reduced mortality and PMR (HR 0.57) at diagnosis and adventitial inflammation (HR 0.31) at TAB. CONCLUSION: PMR at diagnosis and inflammation limited to the adventitia at TAB appear to identify subsets of patients with more benign disease, while large vessel involvement at diagnosis is associated with reduced survival.

Giant-cell arteritis-related mortality in France: A multiple-cause-of-death analysis.

OBJECTIVES: Giant-cell arteritis (GCA) is a large vessel vasculitis. Data regarding mortality are controversial. We describe the mortality data of the French death certificates for the period of 2005 to 2014. METHODS: Using multiple-cause-of-death (MCOD) analysis, we calculated age-adjusted mortality rates for GCA, examined differences in mortality rates according to age and gender and analyzed the underlying causes of death (UCD). RESULTS: We analyzed 4628 death certificates listing a diagnosis of GCA as UCD or non-underlying cause of death (NUCD). The mean age of death was 86 (±6.8) years. The overall age-standardized mortality rate among GCA patients was 7.2 per million population. Throughout the study period, the mean age of death was significantly increased (r = 0.17, p < .0001) in both genders. There was no significant difference with age repartition of death in the general population (p = .26). When GCA was listed as the UCD, most frequent associated diseases were cardiovascular (79%) and infectious diseases (35%). When GCA was reported as the NUCD, the listed UCD was a cardiovascular event in 40% of cases, neoplasm in 13%, neurodegenerative disorder in 11% and infectious disease in 10%. When GCA was the UCD or NUCD, an age-adjusted observed/expected ratio > 1 in GCA-associated mortality compared with the general population mortality was observed for tuberculosis, pneumonia and cardiovascular diseases. CONCLUSION: In this analysis of French death certificates mentioning GCA, we observed a stable standardized mortality rate between 2005 and 2014. The most frequent associated diseases were cardiovascular diseases and infections.

Tocilizumab as an add-on therapy to glucocorticoids during the first 3 months of treatment of Giant cell arteritis: A prospective study.

BACKGROUND: The aim of this study was to evaluate tocilizumab (TCZ) as an add-on therapy to glucocorticoids (GC) during the first 3 months of treatment of giant cell arteritis (GCA). METHODS: GCA patients, as defined by ≥3/5 ACR criteria and positive temporal artery biopsy (TAB) or angio-CT-scan or PET-scan-proven aortitis, were included in this prospective open-label study. Prednisone was started at 0.7 mg/kg/day and then tapered according to a standardized protocol. All patients received four infusions of TCZ (8 mg/kg/4 weeks) after inclusion. The primary endpoint was the percentage of patients in remission with ≤0.1 mg/kg/day of prednisone at week 26 (W26). Patients were followed for 52 weeks and data prospectively recorded. RESULTS: Twenty patients with a median (IQR) age of 72 (69-78) years were included. TAB were positive in 17/19 (90%) patients and 7/16 (44%) had aortitis. Remission was obtained in all cases. At W26, 15 (75%) patients met the primary endpoint. Ten patients experienced relapse during follow-up, mainly patients with aortitis (P = 0.048), or CRP >70 mg/L (P = 0.036) or hemoglobin ≤10 g/dL (P = 0.015) at diagnosis. Among 64 adverse events (AE) reported in 18 patients, three were severe and 30, mostly non-severe infections (n = 15) and hypercholesterolemia (n = 8), were imputable to the study. CONCLUSION: This study shows that an alternative strategy using a short-term treatment with TCZ can be proposed to spare GC for the treatment of GCA. However, 50% of patients experienced relapse during the 9 months following TCZ discontinuation, especially patients with aortitis, or CRP > 70 mg/L or Hb ≤ 10 g/dL at diagnosis. TRIAL REGISTRATION: ClinicalTrials.gov (NCT01910038).

Overall and cause-specific mortality in giant cell arteritis : A meta-analysis.

OBJECTIVE: This study aimed to assess the all-cause and cause-specific standardized mortality ratios (SMRs) in patients with giant cell arteritis (GCA). METHODS: We surveyed studies examining all-cause and/or cause-specific SMRs in patients with GCA compared to the general population, using MEDLINE, EMBASE, Cochrane databases, and manual searches. We then performed a meta-analysis of all-cause, sex-specific, region-specific, and cause-specific SMRs in patients with GCA. RESULTS: In total, 8 reports including 1972 patients with GCA (including 877 patients who died) met the inclusion criteria. Compared with the general population, all-cause SMR was not increased in patients with GCA (SMR 1.081, 95% confidence interval [CI] 0.963-1.214, p = 0.184). Stratification by region showed no significant increase in all-cause SMR in Europe and USA. Sex-specific meta-analysis revealed that the pooled SMR was 1.046 (95%CI 0.834-1.314, p = 0.696) for women and 1.051 (95%CI 0.974-1.133, p = 0.204) for men. There were no sex-specific significant differences in SMR. The risk of mortality due to cardiovascular disease (CVD) was significantly increased (SMR 1.312, 95%CI 1.136-1.516, p < 0.001). However, there was no significant increase in the SMR for mortality due to cancer (SMR 0.833, 95%CI 0.613-1.132, p = 0.243). CONCLUSIONS: Patients with GCA do not show increased rates of death from all causes, regardless of sex, region, or malignancy. However, these patients are at an increased risk of death due to CVD.

Mortality in Patients With Giant Cell Arteritis: A Cohort Study in UK Primary Care.

OBJECTIVE: To examine whether giant cell arteritis (GCA) is associated with increased all-cause mortality and whether mortality differs according to age, sex, and calendar year of cohort entry. METHODS: Using the UK-based Clinical Practice Research Datalink, we identified 9,778 newly diagnosed GCA patients from 1990-2014, and up to 10 nonvasculitis patients randomly matched to each case on age, sex, practice, and years of history before cohort entry. We used Cox regression to estimate adjusted hazard ratios (HRs) for mortality of GCA patients in comparison to nonvasculitis patients, then stratified by age, sex, and calendar year of cohort entry. RESULTS: Compared with nonvasculitis patients, GCA patients had increased mortality during the first year following diagnosis (adjusted HR 1.51, 95% confidence interval [95% CI] 1.40-1.64), and marginally increased mortality between 1 and 5 years after the diagnosis (adjusted HR 1.16, 95% CI 1.09-1.23), but not >5 years after the diagnosis (adjusted HR 1.06, 95% CI 1.00-1.12). GCA patients diagnosed before age 65 years had the highest mortality risk during the first year following diagnosis (adjusted HR 2.32, 95% CI 1.60-3.35). The mortality risk did not differ substantially by sex or calendar year of cohort entry. CONCLUSION: GCA patients had an increased risk of mortality during the period shortly after the GCA diagnosis, in particular during the first year, but no increased risk after 5 years postdiagnosis. The mortality risk differed by age with an even greater increased 1-year mortality in those age <65 years at diagnosis, but not by sex or calendar year of cohort entry.

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

OBJECTIVE: To investigate the epidemiology and mortality in patients with biopsy-proven giant cell arteritis (GCA) in northern Italy. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Giant cell aortitis: clinical presentation and outcomes in 40 patients consecutively operated on.

OBJECTIVES: Giant cell arteritis (GCA) may affect mid-size and large-size arteries. Although temporal arteritis is a well-characterized clinical entity, GCA of the thoracic aorta remains ill defined. The aim of the study was to evaluate the clinical presentation, surgical and mid-term outcomes in patients operated for GCA of the thoracic aorta. METHODS: A retrospective review of patients operated for GCA of the thoracic aorta was conducted. The diagnosis of GCA was established by the pathology report. RESULTS: Forty consecutive patients (mean age of 66.6 ± 9.1 years) with a diagnosis of GCA of the thoracic aorta were operated on. A history of polymyalgia rheumatica or temporal arteritis was positive in 22.5% of patients. Emergency surgery was performed in 10% of patients (3 'type A' dissections and 1 'type B'). Mega-aorta syndrome was present in 10% of patients. Involvement of the ascending aorta was present in 100% of patients. One patient had a previous branched thoracic endovascular replacement (TEVAR) with a type I proximal endoleak. In 4 patients, the thoracic aorta was totally replaced. Eighty-five percent of patients had an arch replacement; 79.4% a hemiarch and 20.6% a full arch. The mean circulatory arrest time was 16.3 ± 12.3 min. Eighty percent of patients had an aortic valve procedure; aortic valve replacement was performed in 50% of them and Bentall-De Bono/valve sparing in 50%. Cerebrovascular accident occurred in 2.5% of patients. No patient died during hospitalization. The mean hospital stay was 8.7 ± 3.0 days. The mean postoperative follow-up time was 4.2 ± 2.3 years, with a mean of 4.2 ± 2.2 thoraco-abdominal computed tomographies (CTs)/patient. Four patients had late reinterventions: 2 were valve-related, 1 for a distal type I endoleak treated with a distal TEVAR extension and 1 type II open thoraco-abdominal replacement for disease progression. One distal type I TEVAR endoleak was treated medically. Aortic diameter progressions on CT (mm/year) were 0.7 ± 1.0 mm for the arch, 1.2 ± 2.0 mm for the isthmus, 1.1 ± 1.7 mm for the mid-descending, 0.7 ± 0.9 mm for the aortic hiatus, 0.5 ± 0.5 mm for the supra-renal aorta and 0.6 ± 0.6 mm for the infra-renal aorta. One patient who declined reoperation on the descending aorta died suddenly 3 years after her initial operation. The 5-year overall survival rate was 91%. CONCLUSIONS: GCA of the thoracic aorta may be suspected in less than 25% of patients preoperatively. Clinical presentation may be acute or chronic with localized or diffused aortic involvement but always involved the ascending aorta. Surgery may be performed with excellent outcomes. Follow-up imaging is mandatory to assess aortic progression.

Associations between polymyalgia rheumatica and giant cell arteritis and 12 cardiovascular diseases.

OBJECTIVES: Evidence of the association of polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) with the full range of cardiovascular diseases (CVDs) is limited. We examined their relationship with the first clinical presentation of the 12 most common CVDs in an unselected population-based cohort of men and women. METHODS: We analysed CArdiovascular disease research using LInked Bespoke studies and Electronic health Records (CALIBER) data, which links primary care and hospital and mortality data in England, from 1997 to 2010. We assembled a cohort of men and women initially free from CVD at baseline and included all patients with PMR and/or GCA (PMR/GCA) diagnosis, matched by age, sex and general practice with up to 10 individuals without PMR/GCA. Random effects Poisson regression analysis was used to study the association between PMR/GCA and the initial presentation of 12 types of CVDs. RESULTS: The analysis included 9776 patients with PMR only, 1164 with GCA only, 627 with PMR and GCA and 105 504 without either condition. During a median of 3.14 years of follow-up 2787 (24.1%) individuals with PMR/GCA and 21 559 (20.4%) without PMR/GCA developed CVDs. Patients with PMR/GCA had lower rates of unheralded coronary death (3.18 vs 3.61/1000 person-years; adjusted incidence ratio 0.79, 95% CI 0.66 to 0.95), transient ischaemic attack (5.11 vs 5.61/1000 person-years; 0.67, 95% CI 0.54 to 0.84) and coronary and death composite (24.17 vs 25.80/1000 person-years; 0.90, 95% CI 0.82 to 0.98). No associations were observed for other CVDs or cerebrovascular diseases, and in patients with only PMR or GCA. No evidence of interaction by age or sex was found. Estimates decreased with longer PMR/GCA duration and findings were robust to multiple sensitivity analyses. CONCLUSIONS: In this large contemporary population-based cohort the presence of PMR and/or GCA was not associated with an increased risk of CVDs or cerebrovascular diseases regardless of PMR/GCA duration.

Predictors of relapse and treatment outcomes in biopsy-proven giant cell arteritis: a retrospective cohort study.

OBJECTIVE: To evaluate characteristics of relapse, relapse rates, treatment and outcomes among patients with biopsy-proven GCA in a large, single-institution cohort. METHODS: We conducted a retrospective review of all patients with biopsy-proven GCA from 1998 to 2013. Demographic, clinical, laboratory and treatment data at presentation and during follow-up were collected. Comparisons by relapse rate were performed using chi-square tests. Prednisone discontinuation by initial oral dose ≤40 and >40 mg/day was compared using Cox models. RESULTS: The cohort included 286 patients [74% female, mean age at diagnosis 75.0 years (s.d. 7.6), median follow-up 5.1 years). During follow-up, 73 patients did not relapse, 80 patients had one relapse and 133 had two or more relapses. The first relapse occurred during the first year in 50% of patients, by 2 years in 68% and by 5 years in 79%. More patients with established hypertension (P = 0.007) and diabetes (P = 0.039) at GCA diagnosis were in the high relapse rate group ( ≥ 0.5 relapses/year) and more females were in the low or high relapse groups than in the no relapse group (P = 0.034). Patients receiving an initial oral prednisone dose >40 mg/day were able to reach a dose of <5 mg/day [hazard ratio (HR) 1.46 (95% CI 1.09, 1.96)] and discontinue prednisone [HR 1.56 (95% CI 1.09, 2.23)] sooner than patients receiving ≤40 mg/day without an increase in observed glucocorticoid-associated adverse events. CONCLUSION: Females and patients with hypertension or diabetes at GCA diagnosis have more relapses during follow-up. Patients treated with an initial oral prednisone dose >40 mg/day achieved earlier prednisone discontinuation.