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Introduction: Coronaritis is a rare but serious complication of giant-cell arteritis (GCA), with an estimated prevalence of less than 1%, however difficult to establish, and of early onset., Methods: We describe 2 cases of GCA presenting with coronaritis and present a review of the literature on this complication., Results: The first patient presented with stable angina on common trunk coronaritis with ostial stenosis. Corticosteroid combined with tocilizumab from the outset resulted in improvement. Angioplasty was performed at 6months with good outcome. The second patient presented with asymptomatic tritruncular ostial coronaritis. Corticosteroid allowed clinic-biological improvement of GCA. Two years later, he presented relapse with an acute coronary syndrome, with favorable evolution after angioplasty, increase of corticosteroids and addition of tocilizumab., Conclusion: Patients presented were successfully treated with corticosteroids combined with tocilizumab and angioplasty of their coronary stenoses. Efficacy of tocilizumab in GCA has not been evaluated especially on coronaritis due to the rarity of this complication. Our experience and the cases reported in the literature suggest good results of angioplasty in this indication. Studies with long-term follow-up will be necessary to evaluate the risk of restenosis., (Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Juvenile temporal arteritis (JTA) is a recently-described and little-known inflammatory disease and its etiology is undetermined. Less than forty cases have been published. This paper is aimed at reporting the largest JTA series and to compare it to literature data to better evaluate its characteristics at diagnosis, its evolution and treatment options., Material and Methods: We conducted a retrospective and descriptive multicentric study in France by identifying adult patients under the age of 50 which had a pathological temporal artery biopsy owing to the presence of a temporal arteritis. Patients with temporal arteritis as a manifestation of systemic vasculitis were excluded., Results: We included 12 patients and the literature review identified 32 cases described in 27 articles, thus a total of 44 patients - 34 men and 10 women - with a median age of 30 and a maximum of 44. All patients presented either a lump in the temporal region or prominent temporal arteries, and 47.7% of patients suffered from headaches. Only 11.4% of patients presented general symptoms and 6.8% a biological inflammatory syndrome; 34.1% had peripheral blood eosinophilia; 83.7% presented a single episode and complete excision without further treatment was documented for 72.7%. Pathology analysis revealed infiltrate of inflammatory cells in the arterial wall in 97.6% of patients but also sparse giant cells for 25% and granuloma for 22.9%, perivascular extension of the inflammation for 82.6%, and presence of lymphoid follicles or germinal centres for 60%. Clinical relapses were present in 16.3% of cases., Conclusion: JTA is a rare, localized and benign disease. The majority of cases have only one episode which is cured by local surgery., (Copyright © 2019 Elsevier B.V. All rights reserved.)

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., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objectives: Large-vessel involvement (LVI) can occur in giant-cell arteritis (GCA) and may represent a distinct disease subgroup with a higher risk for aortic dilation. This study aimed to better characterize the presentation and evolution of LVI in patients with GCA., Patients and Methods: A retrospective multicenter study enrolled 248 GCA patients with LVI and 301 GCA patients without LVI on imaging. Factors associated with aortic dilation were identified in a multivariable model., Results: The patients with LVI were younger (p<0.0001), more likely to be women (p=0.01), and showed fewer cephalic symptoms (p<0.0001) and polymyalgia rheumatica (p=0.001) but more extracranial vascular symptoms (p=0.05) than the patients without LVI. Glucocorticoids (GC) management did not differ between the two groups, but the GC discontinuation rate was lower in the patients with LVI (p=0.0003). Repeated aortic imaging procedures were performed at 19months [range: 5-162months] and 17months [range: 6-168months] after diagnosis in 154 patients with LVI and 123 patients without LVI, respectively, of whom 21% and 7%, respectively, presented new aortic dilations (p=0.0008). In the patients with LVI, aortic dilation occurred on an aorta segment shown to be inflammatory on previous imaging in 94% of patients. In the multivariate analysis, LVI was the strongest predictor of aortic dilation (hazard ratio: 3.16 [range: 1.34-7.48], p=0.009)., Conclusions: LVI represents a distinct disease pattern of GCA with an increased risk of aortic dilation. Control of the aortic morphology during follow-up is required., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objective: To describe characteristics and long-term outcomes of the rare symptomatic lower-limb giant-cell arteritis (LL-GCA)., Methods: Retrospective analysis of 8 patients (6 women; mean±SD age, 63.6±10.9 years; follow-up, 137.3±57.3 months)., Results: Four patients satisfied≥3 American College of Rheumatology (ACR) GCA-classification criteria; 4 had<3 criteria and histological LL-GCA proof or associated typical upper-limb involvement. Patients had 2.1±1.1 vascular risk factors. Bilateral and rapidly progressive arterial claudication was the first LL sign; 2 had rest ischemia. Imaging-visualized most-to-least frequent inflammatory lesion localizations were: superficial femoral, popliteal, tibiofibular trunk, posterior tibial arteries. All received corticosteroids for 132±76.2 months; 2 required immunosuppressive agent adjunction. Only 3 required 1-6 revascularizations: twice during the first month post-diagnosis and twice later. Thromboendarterectomy and endovascular procedures did not work while bypasses were successful. LL-claudication regressed for 7 (10.5±12.1 months) and disappeared for 5 (16.8±9.8 months). Three patients stopped corticosteroids (26-90 months), 1 restarted 45 months later. Every patient relapsed once (23.9±26.7 months, mean corticosteroid dose: 0.28±0.30mg/kg/day). LL-GCA caused only 1 of the 4 deaths., Conclusion: Symptomatic LL-GCA attributable long-term mortality seems to be low despite frequent relapses and corticosteroid-dependence., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

The diagnosis of large-vessel vasculitis has experienced substantial improvement in recent years. While Takayasu arteritis diagnosis relies on imaging, the involvement of epicranial arteries by giant-cell arteritis facilitates histopathological confirmation. When appropriately performed temporal artery biopsy has high sensitivity and specificity. However, an optimal biopsy is not always achievable and, occasionally, the superficial temporal artery may not be involved. Imaging in its various modalities including colour-duplex ultrasonography, computed tomography angiography, magnetic resonance angiography and positron emission tomography, are emerging as important procedures for the diagnosis and assessment of disease extent in large-vessel vasculitis. Recent contributions to the better performance and interpretation of temporal artery biopsies as well as advances in imaging are the focus of the present review.