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Abstract Background Metabolic imaging is routinely used to demonstrate aortitis in patients with giant-cell arteritis. We aimed to investigate the preclinical model of aortitis in BALB/c IL1rn −/− mice using [18F]fluorodeoxyglucose ([18F]FDG) positron emission tomography–magnetic resonance (PET-MR), gamma counting and immunostaining. We used 15 first-generation specific and opportunistic pathogen-free (SOPF) 9-week-old IL1rn −/− mice, 15 wild-type BALB/cAnN mice and 5 s-generation specific pathogen-free (SPF) 9-week-old IL1rn −/− . Aortic [18F]FDG uptake was assessed as the target-to-background ratio (TBR) using time-of-flight MR angiography as vascular landmarks. Results [18F]FDG uptake measured by PET or gamma counting was similar in the first-generation SOPF IL1rn −/− mice and the wild-type group (p > 0.05). However, the first-generation IL1rn −/− mice exhibited more interleukin-1β (p = 0.021)- and interleukin-6 (p = 0.019)-positive cells within the abdominal aorta than the wild-type mice. In addition, the second-generation SPF group exhibited significantly higher TBR (p = 0.0068) than the wild-type mice on the descending thoracic aorta, unlike the first-generation SOPF IL1rn −/− mice. Conclusions In addition to the involvement of interleukin-1β and -6 in IL1rn −/− mouse aortitis, this study seems to validate [18F]FDG PET-MR as a useful tool for noninvasive monitoring of aortitis in this preclinical model.

Background: Metabolic imaging is routinely used to demonstrate aortitis in patients with giant-cell arteritis. We aimed to investigate the preclinical model of aortitis in BALB/c IL1rn−/− mice using [18F]fluorodeoxyglucose ([18F]FDG) positron emission tomography–magnetic resonance (PET-MR), gamma counting and immunostaining. We used 15 first-generation specific and opportunistic pathogen-free (SOPF) 9-week-old IL1rn−/− mice, 15 wild-type BALB/cAnN mice and 5 s-generation specific pathogen-free (SPF) 9-week-old IL1rn−/−. Aortic [18F]FDG uptake was assessed as the target-to-background ratio (TBR) using time-of-flight MR angiography as vascular landmarks. Results: [18F]FDG uptake measured by PET or gamma counting was similar in the first-generation SOPF IL1rn−/− mice and the wild-type group (p > 0.05). However, the first-generation IL1rn−/− mice exhibited more interleukin-1β (p = 0.021)- and interleukin-6 (p = 0.019)-positive cells within the abdominal aorta than the wild-type mice. In addition, the second-generation SPF group exhibited significantly higher TBR (p = 0.0068) than the wild-type mice on the descending thoracic aorta, unlike the first-generation SOPF IL1rn−/− mice. Conclusions: In addition to the involvement of interleukin-1β and -6 in IL1rn−/− mouse aortitis, this study seems to validate [18F]FDG PET-MR as a useful tool for noninvasive monitoring of aortitis in this preclinical model. [ABSTRACT FROM AUTHOR]

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. We describe 2 cases of GCA presenting with coronaritis and present a review of the literature on this complication. 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 6 months 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. 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. [ABSTRACT FROM AUTHOR]

A 93-year-old woman presented with a 1-day history of sudden painless vision loss in her right eye with a background of new temporal headaches and jaw claudication. Dilated fundus examination of the right eye showed mild retinal whitening and edema throughout the right fundus with selective sparing in the distribution of the cilioretinal artery. A diagnosis of giant-cell arteritis (GCA) was made and confirmed with temporal artery biopsy. Oral prednisone was initiated and her vision improved to 20/200 in the right eye at 3-month follow-up. GCA-related cilioretinal artery sparing central retinal artery occlusion is rare and may have mild improvement with promt treatment.

This article discusses a case of giant-cell arteritis (GCA) in a 78-year-old woman who presented with a low-grade fever of unknown origin. The patient initially visited a dental clinic for toothache and jaw claudication, but no dental issues were found. The patient was then referred to a clinic where a slightly enlarged left temporal artery without pulsation and tenderness was observed. Ultrasonography revealed thickened walls in the temporal artery frontal branches, confirming the diagnosis of GCA. Prompt diagnosis and treatment with prednisolone led to the resolution of symptoms. The article emphasizes the importance of considering GCA in elderly Asian patients with fever and temple pain and highlights the usefulness of ultrasonography for diagnosis. [Extracted from the article]

Although great improvements have been achieved in the fields of diagnosing and treating patients with giant-cell arteritis (GCA) in the last decades, several questions remain unanswered. The progressive increase in the number of older people, together with growing awareness of the disease and use of advanced diagnostic tools by healthcare professionals, foretells a possible increase in both prevalence and number of newly diagnosed patients with GCA in the coming years. A thorough clarification of pathogenetic mechanisms and a better definition of clinical subsets are the first steps toward a better understanding of the disease and, subsequently, toward a better use of existing and future therapeutic options. Examination of the role of different imaging techniques for GCA diagnosing and monitoring, optimization, and personalization of glucocorticoids and other immunosuppressive agents, further development and introduction of novel drugs, identification of prognostic factors for long-term outcomes and management of treatment discontinuation will be the central topics of the research agenda in years to come. [ABSTRACT FROM AUTHOR]

A 93-year-old woman presented with a 1-day history of sudden painless vision loss in her right eye with a background of new temporal headaches and jaw claudication. Dilated fundus examination of the right eye showed mild retinal whitening and edema throughout the right fundus with selective sparing in the distribution of the cilioretinal artery. A diagnosis of giant-cell arteritis (GCA) was made and confirmed with temporal artery biopsy. Oral prednisone was initiated and her vision improved to 20/200 in the right eye at 3-month follow-up. GCA-related cilioretinal artery sparing central retinal artery occlusion is rare and may have mild improvement with promt treatment. [ABSTRACT FROM AUTHOR]

Objectives The treatment of GCA relies on corticosteroids but is burdened by a high rate of relapses and adverse effects. Anti-IL-6 treatments show a clear benefit with a significant steroid-sparing effect, but late relapses occur after treatment discontinuation. In addition to IL-6, IL-1 also appears to play a significant role in GCA pathophysiology. We report herein the efficacy of anakinra, an IL-1 receptor antagonist, in six GCA patients exhibiting corticosteroid dependence or resistance, specifically analysing the outcome of aortitis in four of them. Methods This retrospective study analysed the cases of all GCA patients treated with anakinra from the French Study Group for Large Vessel Vasculitis. Results After a median duration of anakinra therapy of 19 (18–32) months, all six patients exhibited complete clinical and biological remission. Among the four patients with large-vessel involvement, one had a disappearance of aortitis under anakinra and three showed a decrease in vascular uptake. After a median follow-up of 56 (48–63) months, corticosteroids were discontinued in four patients, and corticosteroid dosage could be decreased to 5 mg/day in two patients. One patient relapsed 13 months after anakinra introduction in the context of increasing the daily anakinra injection interval to every 48 h. Three patients experienced transient injection-site reactions, and one patient had pneumonia. Conclusion In this short series, anakinra appears to be an efficient and safe steroid-sparing agent in refractory GCA, with a possible beneficial effect on large-vessel involvement. [ABSTRACT FROM AUTHOR]

Little is known about the impact of giant cell arteritis (GCA) and its treatment on patient-reported physical, mental, and psychic quality of life (QoL). In this monocentric study, a questionnaire was sent to the 100 last patients diagnosed with GCA and followed-up in a single tertiary center. Their physical, mental and psychic status were self-assessed via close-ended questions, the 12-item short form survey (SF-12) and the 15-item geriatric depression scale (GDS). We aimed to identify parameters that were significantly associated with moderate-to-severe disability in both physical and mental domains. Ninety patients were analyzable. Moderate to severe physical disability was found in 41 (46%) patients. In multivariate analysis, walking difficulties (OR, 95% CI 8.42 [2.98–26.82], p 80 (OR, 95% CI 4.21 [1.44–13.61], p = 0.008) were independent findings associated with moderate to severe physical disability. Moderate to severe mental disability was found in 30 (33%) patients. In multivariate analysis, depressive mood (OR, 95% CI 11.05 [3.78–37.11], p < 0.0001), felt adverse events attributable to glucocorticoids (OR, 95% CI 10.54 [1.65–213.1], p = 0.01) and use of immune-suppressants (OR, 95% CI 3.50 [1.14–11.87], p = 0.03) were independent findings associated with moderate to severe mental disability. There was a statistically significant negative correlation between GDS and the physical and/or mental disability scores (GDS and PCS-12: r = −0.33, p = 0.0013; GDS and MCS-12: r = −0.36, p = 0.0005). In conclusion, this study identified via a self-assessment of patients with GCA some medical and modifiable findings that significantly affect their physical and mental quality of life. A better knowledge of these factors may help improve the care of GCA patients.

Abstract Background Giant cell arteritis is a vasculitis of large and middle-sized arteries that affects patients aged over 50 years. It can show a typical clinical picture consisting of cranial manifestations but sometimes nonspecific symptoms and large-vessel involvement prevail. Prompt diagnosis and treatment is essential to avoid irreversible damage. Discussion There has been an increasing knowledge on the occurrence of the disease without the typical cranial symptoms and its close relationship and overlap with polymyalgia rheumatica, and this may contribute to reduce the number of underdiagnosed patients. Although temporal artery biopsy is still the gold-standard and temporal artery ultrasonography is being widely used, newer imaging techniques (FDG-PET/TAC, MRI, CT) can be of valuable help to identify giant cell arteritis, in particular in those cases with a predominance of extracranial large-vessel manifestations. Conclusions Giant cell arteritis is a more heterogeneous condition than previously thought. Awareness of all the potential clinical manifestations and judicious use of diagnostic tests may be an aid to avoid delayed detection and consequently ominous complications.

• Giant-cell arteritis-related aortic dissection occurs earlier in patients with aortitis than in those without. • 70% of patients presented Stanford type A aortic dissection. • Half of patients with type a dissection had a previous thoracic aorta dilation. • Aortic surgery is the single predictive factor for survival in patients with GCA-related aortic dissection. To describe characteristics and outcomes of patients with giant cell arteritis (GCA)-related aortic dissection. We retrospectively included, through a nationwide GCA network, all patients who had an aortic dissection either revealing GCA or occurring during follow-up. A total of 46 patients were included in this study. Aortic dissection was inaugural and led to GCA diagnosis in 21 patients, whereas it occurred during follow-up in the 25 others, at a median of 53 [1–265] months after GCA diagnosis. Large-vessel vasculitis (LVV) was diagnosed through imaging before or at the time of aortic dissection in 31 (67%) patients. In patients who developed an aortic dissection during follow-up, the aortic event occurred 22 [1–143] months post GCA diagnosis in the patients with previous aortitis, whereas it occurred after 72 [19–265] months in patients without previously diagnosed aortitis (p = 0.005). Aortic surgery was performed in 27 (59%) patients and 23 of them survived. A total of 15 (32%) patients died following the aortic dissection, including 11 who were not operated on. In a multivariate analysis, aortic surgery was the single predictor of survival (HR: 4.3; 95% CI: 1.47— 15.7; p = 0.007). Patients with prior LVV are more prone to develop early aortic dissection and require close monitoring of aortic morphology. One third of patients died from the aortic dissection. Surgery remains the best predictive factor for survival. [ABSTRACT FROM AUTHOR]

COVID-19 shares some features of giant-cell arteritis, in which the diagnosis needs a high suspicion for prompt investigation and therapy. When the diseases coexist this might lead to diagnosis delay with grave consequences. We reported a case of a post-COVID-19 giant cell arteritis and polymyalgia rheumatica with visual loss. We treated the patient with pulse methylprednisolone 1 gm daily for 3 consecutive days followed by 60 mg prednisolone for 4 weeks until normalization of ESR, and then, gradual withdrawal. Oral Paracetamol, vitamin-D3, and calcium carbonate were added to the treatment regimen. The headache continued, so, we started perineural injection therapy (PIT) once daily, for 6 sessions, at which the headache was completely resolved after the third injection. The vision was regained completely after the sixth injection. [ABSTRACT FROM AUTHOR]

The issue of improved diagnosis of both rheumatic diseases of the elderly and aortic diseases does not lose its relevance. In terms of aortic aneurysms, dissection and ruptures and their attended pathogenesis, both inflammation and structural wall damages may be detected with imaging methods whose role is vital. A number of international guidelines deal with the management of polymyalgia rheumatica, giant cell arteritis, or aortic aneurysms. Aortitis is associated with up to 40 % of polymyalgia rheumatica’s cases. The clinical suspicion of aortitis is based on the detection of blood pressure and pulse asymmetry, aortic regurgitation murmur, vascular bruits, as well as persistent polymyalgia rheumatica or inflammatory dorsalgia, pelvis or leg pain. In 2020, the positron emission tomography/computed tomography’s use is approved by the Italian Society for Rheumatology for the diagnosis of vasculitis attended by polymyalgia rheumatica at the secondary healthcare level and by the European Headache Federation for the diagnosis of large vessel giant cell arteritis in the neurological practice. A review of the guideline by the European Association of Nuclear Medicine, the Society of Nuclear Medicine and Molecular Imaging, and the American Society of Nuclear Cardiology (2018) was performed in terms of positron emission tomography with fluorodesoxyglucose combined with computed tomography (angiography) imaging in large vessel vasculitis and polymyalgia rheumatica. It is further compared with the clinical guidelines, other guidelines by the societies of nuclear medicine and new scientific data. Both procedure and patient’s preparation for examination are decribed. The criteria for assessing vasculitis proposed for either clinical practice or clinical studies are considered, as well as the factors influencing the test results and their interpretation (such as atherosclerosis, diabetes, age, body mass index, glucemia’s and acute phase markers’ levels). The guideline substantiates the benefit of both positron emission tomography’s use and its combination with computed tomography to detect extracranial vasculitis, as well as the value of performing computed tomography-angiography at different stages of the disease. There is a need to strengthen evidence on both standard time of fluorodesoxyglucose exposure and the benefit of combining positron emission tomography with computed tomography-angiography, in particular for detection of vasculitis relapses. Finding a consensus for early test’s performing is needed, as well as its score standardization, ensuring reimbursement and implementation of new imaging techniques for the cranial vessels. In the future, the evidence-based approach to managing vasculitis will be supplemented by teranostics. [ABSTRACT FROM AUTHOR]

For decades, the treatment of GCA has relied on glucocorticoids. Work over the past two decades has supported a modest efficacy of MTX but no clear benefit from anti-TNF-based therapies. More recently, the therapeutic armamentarium for GCA has expanded. The availability of agents targeting specific cytokines, cytokine receptors or signalling pathways, along with a better, although still limited, understanding of the immunopathology of GCA, are opening further therapeutic possibilities. Blocking IL-6 receptor with tocilizumab has been effective in maintaining remission and reducing glucocorticoid exposure and tocilizumab has been approved for the treatment of GCA. However, nearly half of the patients do not benefit from tocilizumab and additional options need to be investigated. This review focuses on standard therapeutic approaches and on targeted therapies that have been or are currently under investigation. [ABSTRACT FROM AUTHOR]

To describe the characteristics and outcome of patients with giant-cell arteritis (GCA)-related ophthalmologic involvement at diagnosis. In a retrospective single-center cohort of 409 consecutive patients with GCA, we retrieved 104 patients with visual symptoms at GCA diagnosis and we compared them to 104 age- and sex-matched controls without ophthalmologic involvement. Each visual symptom was associated to an ophthalmologic diagnosis that was centrally re-assessed by an ophthalmologist. Compared to controls, patients with visual symptoms showed less fever (p = 0.0006), less polymyalgia rheumatica (p = 0.02) and lower acute phase reactants (p = 0.004). Blurred vision (in 60% of patients), amaurosis fugax (in 18%), diplopia (in 13%) and permanent visual loss (in 9%) were the four visual symptoms described by patients before GCA diagnosis. Anterior ischemic optic neuropathy (AION) was found in 47 (45%) patients, followed by central retinal artery occlusion (CRAO) in 15 (15%). Two patients had both involvements. The delay of glucocorticoids initiation was not different between patients with and without visual symptoms (p = 0.06). Among the 60 patients with initial AION and/or CRAO, 39 (65%) kept definite blindness or important visual damage, although 45 (75%) had received intravenous (IV) pulses of methylprednisolone. A new ischemic event (AION in all cases) occurred in 4% of patients with visual symptoms despite the initiation of treatment. Ophthalmologic involvement was observed in one-quarter of our GCA patients. AION is still associated with the worst visual prognosis, and IV methylprednisolone pulses did not reduce the risk of blindness in our study. [ABSTRACT FROM AUTHOR]

• GCA patients with scalp, tongue and/or lip necrosis have more cephalic symptoms but not more ocular involvement after matching for sex, age at diagnosis and diagnosis period. • GCA patients with scalp, tongue and/or lip necrosis display shorter overall survival. • Scalp tenderness and cognitive disorder at diagnosis seem to be factors significantly associated to this very rare complication of GCA. Scalp, tongue and/or lip necrosis are rare complications of GCA. To describe characteristics and outcome of patients with giant cell arteritis (GCA) -related scalp, tongue and/or lip necrosis. A retrospective nationwide multicenter study included 20 GCA patients with scalp, tongue, and/or lip necrosis diagnosed between 1998 and 2021 and 80 GCA control patients matched for age, sex and management period. Logistic regression analyses were conducted to identify baseline characteristics associated with scalp, tongue and/or lip necrosis. Compared to controls, patients with scalp, tongue and/or lip necrosis showed significantly more cranial manifestations (headache, p=0.045; scalp tenderness, p=0.006; jaw claudication, p=0.02). No differences were observed between both groups regarding the occurrence of visual symptoms or large vessel involvement. At diagnosis, GCA patients with necrosis more likely received IV methylprednisolone infusions and higher doses of oral prednisone. There were no differences regarding vascular complications during follow up. Compared to controls, survival was decreased in GCA patients with necrosis (p=0.003). In a multivariable logistic regression model, scalp tenderness [odds ratio (OR) 4.81(95 % CI: 1.57, 14.79), p = 0.006] and cognitive disorder [OR 6.42 (95 % CI: 1.01, 40.60), p=0.048] were identified as factors associated to scalp, tongue, and/or lip necrosis. Our results suggest that scalp, tongue, and/or lip necrosis is associated to higher mortality in GCA patients. Scalp tenderness and cognitive disorder were significant factors associated to this very rare complication of GCA. [ABSTRACT FROM AUTHOR]

Giant-cell arteritis is associated with a higher risk of aortic aneurysm and aortic dissection formation. We present a women with aortic dissection type B treated with a stent graft and bare-metal stent implantation. After the stent deployment we noticed aortic rupture, which was successfully treated with implantation of an additional stent graft. This report highlights the difficulty of endovascular therapy in patients with giant-cell arteritis. We have to bear in mind that chronic inflammation of the aorta leads to a more fragile aortic wall than normal. We recommend the use of a stent graft over a bare-metal stent and gentle use of a balloon catheter.

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. 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. 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. JTA is a rare, localized and benign disease. The majority of cases have only one episode which is cured by local surgery. • Juvenile temporal arteritis, is a rare, localized, and benign disease affecting the temporal artery in patients aged less than <50 years. • JTA presenting as a lump in the forehead, sometimes painful, without systemic symptoms or biological inflammation. • JTA is most frequently cured by biopsy or excision. • Kimura disease and angiolymphoid hyperplasia with eosinophilia must be considered as alternate diagnosis. [ABSTRACT FROM AUTHOR]

BackgroundGiant-cell arteritis (GCA) is considered a T helper (Th)1- and Th17-mediated disease. Interleukin (IL)-12 is a heterodimeric cytokine (p35/p40) involved in Th1 differentiation. When combining with p19 subunit, p40 compose IL-23, a powerful pro-inflammatory cytokine that maintains Th17 response.ObjectivesThe aims of this study were to investigate p40, p35, and p19 subunit expression in GCA lesions and their combinations to conform different cytokines, to assess the effect of glucocorticoid treatment on subunit expression, and to explore functional roles of p40 by culturing temporal artery sections with a neutralizing anti-human IL-12/IL-23p40 antibody.Methods and resultsp40 and p19 mRNA concentrations measured by real-time RT-PCR were significantly higher in temporal arteries from 50 patients compared to 20 controls (4.35 ± 4.06 vs 0.51 ± 0.75; p

We describe a 58-year-old woman presenting with headache and an elevated erythrocyte sedimentation rate (ESR), who was diagnosed with and successfully treated for giant-cell arteritis (GCA). Seven months after the end of treatment, ovarian GCA was incidentally found after ovariectomy for a simple cyst. GCA of extracranial vessels like the ovarian arteries is rare. Nevertheless, we stress that extracranial GCA should be considered in patients older than 50 years with an elevated ESR, even if a temporal artery biopsy is negative or specific symptoms are absent. Moreover, we discuss the importance of imaging techniques when GCA of the extracranial large vessels is suspected.

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Systemic vasculitides (SV) are a heterogeneous group of acute and chronic diseases, the most important pathomorphological sign of which is inflammation and necrosis of the vascular wall. The basis for the classification of SV is their etiology and pathogenesis, the type of affected vessels and the pattern of inflammation, predominant organ involvements, clinical manifestations, a genetic predisposition, and demographic characteristics. To diagnose vasculitis is a difficult task due to a broad spectrum of its clinical manifestations that depend on the predominant size of affected vessels, the involved organs and systems, and the magnitude of the inflammatory process. There are no specific laboratory tests for most forms of SV; nonspecific inflammatory measures and activated autoimmune reactions are noted to be higher. Screening for SV determines the levels of anti-neutrophil cytoplasmic antibodies (ANCA), cryoglobulins, anti-glomerular basement membrane antibodies, and anti-complement C1q antibodies. However, biopsy with its specimen being pathomorphologically characterized, as well as current noninvasive or minimally invasive diagnostic techniques remain relevant because of a large number of seronegative variants of vasculitis. The ANCA-associated vasculitis management algorithm developed by the British Society of Rheumatology has been presented in 2015. The timely diagnosis and treatment at the onset of SV is the basis for quality of life and survival improvement.

Abstract 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. [ABSTRACT FROM AUTHOR]

Purpose: We report three cases of ocular inflammation and polymyalgia rheumatica without concomitant giant-cell arteritis.Methods: Report of three cases.Results: Polymyalgia rheumatica onset was at a mean age of 66.7 years, and ocular inflammation, which developed 7-21 months later, was bilateral in all patients. Ocular inflammation presented as episcleritis, scleritis, or anterior uveitis, and it emerged during the tapering of low-dose prednisolone prescribed for polymyalgia rheumatica in all patients. Recurrence of ocular inflammation was observed in two patients.Conclusions: Ocular inflammation associated with polymyalgia rheumatica was often bilateral and occurred during steroid tapering. Although this presentation is relatively uncommon, polymyalgia rheumatica should be considered in the differential diagnosis of older patients presenting with ocular inflammation, especially those with proximal myalgia and elevated inflammatory markers. [ABSTRACT FROM AUTHOR]

Background: Giant-cell arteritis (GCA) is considered a T helper (Th)1- and Th17-mediated disease. Interleukin (IL)-12 is a heterodimeric cytokine (p35/p40) involved in Th1 differentiation. When combining with p19 subunit, p40 compose IL-23, a powerful pro-inflammatory cytokine that maintains Th17 response. Objectives: The aims of this study were to investigate p40, p35, and p19 subunit expression in GCA lesions and their combinations to conform different cytokines, to assess the effect of glucocorticoid treatment on subunit expression, and to explore functional roles of p40 by culturing temporal artery sections with a neutralizing anti-human IL-12/IL-23p40 antibody. Methods and results: p40 and p19 mRNA concentrations measured by real-time RT-PCR were significantly higher in temporal arteries from 50 patients compared to 20 controls (4.35 ± 4.06 vs 0.51 ± 0.75; p < 0.0001 and 20.32 ± 21.78 vs 4.17 ± 4.43 relative units; p < 0.0001, respectively). No differences were found in constitutively expressed p35 mRNA. Contrarily, p40 and p19 mRNAs were decreased in temporal arteries from 16 treated GCA patients vs those from 34 treatment-naïve GCA patients. Accordingly, dexamethasone reduced p40 and p19 expression in cultured arteries. Subunit associations to conform IL-12 and IL-23 were confirmed by proximity-ligation assay in GCA lesions. Immunofluorescence revealed widespread p19 and p35 expression by inflammatory cells, independent from p40. Blocking IL-12/IL-23p40 tended to reduce IFNγ and IL-17 mRNA production by cultured GCA arteries and tended to increase Th17 inducers IL-1β and IL-6. Conclusion: IL-12 and IL-23 heterodimers are increased in GCA lesions and decrease with glucocorticoid treatment. p19 and p35 subunits are much more abundant than p40, indicating an independent role for these subunits or their potential association with alternative subunits. The modest effect of IL-12/IL-23p40 neutralization may indicate compensation by redundant cytokines or cytokines resulting from alternative combinations. [ABSTRACT FROM AUTHOR]

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 19 months [range: 5–162 months] and 17 months [range: 6–168 months] 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. [ABSTRACT FROM AUTHOR]

The case of a giant-cell arteritis is presented. First clinical signs of the disease were fewer and development of infarction in the basin of the left vertebral artery. Magnetic resonance angiography showed its prolonged diminution. Laboratory results were remarkable for the high rate of erythrocyte sedimentation and the increase of C-reactive protein (CRP) concentration. Physical examination revealed acrotism in temporal arteries. Diagnosis was proven by biopsy results which included giant multinucleate cells. Authors discuss problems of diagnosis of the disease, the role of radiological methods (angio-ultrasonography, magnetic resonance and computed tomography aided angiography, positron-emission tomography) and the necessity to pay particular attention to the elderly patients with high rate of erythrocyte sedimentation and the increased CRP concentration.

Objective 18 F-FDG PET/CT can detect large-vessel involvement in giant-cell arteritis (GCA) with a good sensitivity. In patients with clinically and biologically controlled disease, we aimed to assess how vascular uptakes evolve on repetitive FDG-PET/CT. Patients and methods All included patients had to satisfy the 4 following criteria: 1) diagnosis of GCA was retained according to the criteria of the American College of Rheumatology or based on the satisfaction of 2 criteria associated with the demonstration of large-vessel involvement on FDG-PET/CT; 2) all patients had a positive PET/CT that was performed at diagnosis before treatment or within the first 10 days of treatment; 3) another FDG-PET/CT was performed after at least 3 months of controlled disease without any relapse; 4) patients were followed-up at least for 12 months. Results Twenty-five patients (17 [68%] women, median age: 69 [65–78]) with large-vessel inflammation on a baseline FDG-PET/CT and with repetitive imaging during the period with controlled disease were included and followed-up for 62 [25–95] months. Four repeated procedures revealed total extinction of vascular uptakes at 11.5 [8–12] months after the first FDG-PET/CT. Eight PET/CT revealed decreased numbers of vascular uptakes, and 10 procedures revealed no changes. The 3 remaining procedures indicated worsening of the numbers of vascular uptakes in the absence of relapse. Conclusions Our study revealed long-term persistent vascular uptake on repeated FDG-PET/CT in > 80% of our GCA patients with large-vessel inflammation and clinical-biological controlled disease. Prospective studies are required to confirm these findings. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of our study was to assess the concordance of aortic CT angiography (CTA) and FDG-PET/CT in the detection of large-vessel involvement at diagnosis in patients with giant-cell arteritis (GCA). Methods: We created a multicenter cohort of patients with GCA diagnosed between 2010 and 2015, and who underwent both FDG-PET/CT and aortic CTA before or in the first ten days following treatment introduction. Eight vascular segments were studied on each procedure. We calculated concordance between both imaging techniques in a per-patient and a per-segment analysis, using Cohen's kappa concordance index. Results: We included 28 patients (21/7 women/men, median age 67 [56-82]). Nineteen patients had large-vessel involvement on PET/CT and 18 of these patients also presented positive findings on CTA. In a per-segment analysis, a median of 5 [1-7] and 3 [1-6] vascular territories were involved on positive PET/CT and CTA, respectively ( p = 0.03). In qualitative analysis, i.e., positivity of the procedure suggesting a large-vessel involvement, the concordance rate between both procedures was 0.85 [0.64-1]. In quantitative analysis, i.e., per-segment analysis in both procedures, the global concordance rate was 0.64 [0.54-0.75]. Using FDG-PET/CT as a reference, CTA showed excellent sensitivity (95%) and specificity (100%) in a per-patient analysis. In a per-segment analysis, sensitivity and specificity were 61% and 97.9%, respectively. Conclusions: CTA and FDG-PET/CT were both able to detect large-vessel involvement in GCA with comparable results in a per-patient analysis. However, PET/CT showed higher performance in a per-segment analysis, especially in the detection of inflammation of the aorta's branches. [ABSTRACT FROM AUTHOR]

Dermatologic manifestations are observed in almost all systemic vasculitides, even in large-and medium-vessel vasculitides, although such vessels are not found in the skin. Cutaneous manifestations may be related to a direct skin localization of the systemic vasculitis or a non-specific process associated with the vasculitis. According to the 2012 International Chapel Hill consensus, the two major variants of large-vessel vasculitides are Takayasu arteritis and giant-cell arteritis. In Europe and North America, acute inflammatory nodules or erythema nodosum-like lesions are the most commonly observed skin lesions with Takayasu arteritis. Medium-sized arteriole vasculitis of the dermis or subcutis but also septal or lobular panniculitis may be found during pathological examination. In Japan, widespread pyoderma gangrenosum-like lesions are more frequent. Cutaneous manifestations of giant-cell arteritis are rare; they are ischemic, linked to arterial occlusions, or non-ischemic, with various mechanisms. The two major medium-vessel vasculitides are Kawasaki disease and polyarteritis nodosa. Kawasaki disease is characterized by a mucocutaneous lymph node syndrome without skin vasculitis. Two subsets of polyarteritis nodosa with different skin manifestations are described, without transition from one to the other. In the systemic subset, the most frequent skin lesions are in the order of frequency purpura, livedo, and nodules. Cutaneous polyarteritis nodosa mainly features nodules, livedo racemosa, and ulcerations. Genetic screening and measurement of plasma levels of adenosine deaminase 2 should be considered for patients with uncommon systemic polyarteritis nodosa or early-onset cutaneous polyarteritis nodosa. [ABSTRACT FROM AUTHOR]

Large-vessel vasculitis (LVV) is a group of diseases mainly comprised of giant-cell arteritis (GCA), Takayasu arteritis, and a series of rare diseases like Behçet's disease, IgG4-related disease, infectious aortitis, and other unfrequent entities. Besides clinical and laboratory features, Doppler sonography (DS) can assist in establishing the diagnosis. Its diagnostic sensitivity has been evaluated in various studies, most of them, however, in temporal arteritis (TA) respectively in LVV with involvement of the temporal artery. Little is known in extracranial LVV. We retrospectively evaluated the diagnostic accuracy of DS in 30 patients with extracranial, non-temporal LVV using the highly sensitive PET/CT as method of reference in comparison to 20 controls who were found to have no LVV. We investigated ten arterial sites and documented the presence of the sonographic halo sign. Sensitivities of DS for LVV were highest in the subclavian and axillary arteries (71.4%/72.2%) and low in the abdominal aorta (26.1%) and the common femoral artery (16.7%). DS detected 24 out of 30 cases of LVV (overall sensitivity 80.0%). The LVV cases where DS was completely negative did not significantly differ in leukocyte count, C-reactive protein, or erythrocyte sedimentation rate from LVV cases with positive DS. DS is a potent method in diagnosing extracranial LVV especially in the axillary and the subclavian arteries. Aortic, intraabdominal, and lower extremity artery manifestations, however, are often missed by DS. A second imaging modality (e.g., PET/CT) is therefore required. [ABSTRACT FROM AUTHOR]

Purpose: We aimed to describe the initial treatment that was used in a common hospital-based practice in patients with giant-cell arteritis with and without large-vessel involvement at diagnosis as well as the outcomes in both groups.Methods: This retrospective multi-center cohort included patients with giant-cell arteritis diagnosed between 2005 and 2015, all of whom had fluorodeoxyglucose (FDG) positron emission tomography combined with computed tomography (FDG-PET/CT) performed at giant-cell arteritis diagnosis and were followed up for ≥12 months. We compared the features, treatment, and outcomes of patients with large-vessel involvement demonstrated on FDG-PET/CT with those of patients with a negative PET/CT.Results: Eighty patients (50 women, median age: 71 [53-87] years) were included, 40 of whom had large-vessel involvement demonstrated on FDG-PET/CT and 40 who did not. After a median 56-month follow-up time, 42 (53%) patients had discontinued glucocorticoid (GC) treatment. Patients with and without large-vessel involvement were indistinguishable in the initial median dose of prednisone (0.74 mg/kg vs 0.75 mg/kg, P = .56), overall GC duration (P = .77), GC discontinuation rate (P = .65), relapse rate (P = .50), frequency of GC-dependent disease requiring GC-sparing treatments (P = .62), and fatality rate (P = .06).Conclusion: In the setting of tertiary hospital recruitment, large-vessel involvement at giant-cell arteritis diagnosis using a PET/CT study had no influence on the choice of initial GC dose and had no impact on outcomes. Prospective studies are required to confirm these findings. [ABSTRACT FROM AUTHOR]

Giant-cell arteritis is associated with a higher risk of aortic aneurysm and aortic dissection formation. We present a women with aortic dissection type B treated with a stent graft and bare-metal stent implantation. After the stent deployment we noticed aortic rupture, which was successfully treated with implantation of an additional stent graft. This report highlights the difficulty of endovascular therapy in patients with giant-cell arteritis. We have to bear in mind that chronic inflammation of the aorta leads to a more fragile aortic wall than normal. We recommend the use of a stent graft over a bare-metal stent and gentle use of a balloon catheter. [ABSTRACT FROM AUTHOR]

Giant-cell arteritis (GCA), commonly known as temporal arteritis, is a chronic granulomatous vasculitis that affects predominantly the extracranial branches of the carotid artery. Although an algorithm for diagnosing GCA that includes both biopsy and imaging examinations has been recently proposed, it harbors the possibility of false negatives. Hence, jaw claudication caused by ischemia of the masticatory muscles is one of the important clinical predictors for implementation of temporal artery biopsy (TAB). We describe a case of GCA in which jaw claudication was the only clinical predictor for implementation of TAB. A 78-year-old man was referred to our department with facial pain associated with mastication. He had been admitted 3 weeks previously to another department to investigate an unidentified fever. A blood test revealed an elevated C-reactive protein level and a high erythrocyte sedimentation rate (70 mm/h). Although ultrasonography and computed tomography angiography of the temporal region showed no findings specific for GCA, based on our examinations we determined that his facial pain arose from jaw claudication rather than temporomandibular disorder. Histopathological examination by temporal artery biopsy showed intima thickening with disruption of elastic lamina and inflammatory cell infiltration, and we consequently diagnosed GCA. Clinical symptoms immediately resolved after prescription of prednisolone 40 mg/day for 2 days. In this case of GCA the imaging examinations of the superficial temporal artery were false-negative, and jaw claudication was the most important predictor of GCA. [ABSTRACT FROM AUTHOR]