[Clinical characteristics and follow-up of 60 patients with recent diagnosis of giant cell arteritis, NEWTON study]. / Caractéristiques et profils évolutifs des patients avec un diagnostic récent d'artérite à cellules géantes, étude NEWTON.

INTRODUCTION: The management of giant cell arteritis (GCA) has evolved with the arrival of tocilizumab (TCZ) and the use of PET/CT. Our objective is to describe the characteristics and followup of patients with recent diagnosis of GCA in current care. PATIENTS AND METHODS: The NEWTON cohort is a monocentric retrospective cohort based on data collected from 60 GCA patients diagnosed between 2017 and 2022 according to the ACR/EULAR 2022 criteria. RESULTS: The median age at diagnosis was 73 [68.75; 81] years old. At diagnosis, the main manifestations were unusual temporal headaches in 48 (80 %) and an inflammatory syndrome in 50 (83 %) patients. Temporal artery biopsy confirmed the diagnosis in 49/58 (84 %) patients. Doppler of the temporal arteries found a halo in 12/23 (52 %) patients. The PET/CT found hypermetabolism in 19/43 (44 %) patients. Prednisone was stopped in 17.5 [12.75; 24.25] months. During follow-up, 22 (37 %) patients received TCZ. At least one complication of corticosteroid therapy was observed in 22 (37 %) patients. After a median follow-up of 24 [12; 42] months, 25 (42 %) patients relapsed. At the end of the follow-up, 29 (48.3 %) patients were weaned from corticosteroid therapy and 15 (25 %) were on TCZ. CONCLUSION: Despite the increasing use of TCZ in the therapeutic arsenal and of the PET/CT in the imaging tools of GCA patients, relapses and complications of corticosteroid therapy remain frequent, observed in more than a third of patients.

Use of intravenous lidocaine in a patient with refractory chest pain secondary to Takayasu arteritis. Case report / Uso da lidocaína endovenosa em paciente com dor torácica refratária secundária à arterite de Takayasu. Relato de caso

ABSTRACT BACKGROUND AND OBJECTIVES: Takayasu's arteritis (TA) is a rare form of chronic inflammatory disease involving large vessels, with uncertain etiology, with chest pain as a common and challenging symptom, resulting from inflammation in the aortic root or arch, pulmonary artery or coronary arteries. The objective of this study was to describe the use of intravenous lidocaine to treat severe and refractory chest pain secondary to TA. CASE REPORT: A 33-year-old female patient diagnosed with TA, with severe chest pain that was difficult to manage, was admitted after consulting an emergency department. The pain was unresponsive to traditional treatment after a week of drug adjustments. As a therapeutic option, a Sympathetic Venous Blockade (SVB) with lidocaine was chosen, achieving a reduction in pain from 10 to 3 on the Visual Analog Scale. Infliximab was administered before discharge. The patient was re-evaluated at an outpatient appointment after 30 days. CONCLUSION: This strategy for the treatment of severe chest pain allowed for pain reduction and relief.

RESUMO JUSTIFICATIVA E OBJETIVOS: A arterite de Takayasu (AT) é uma forma rara de doença inflamatória crônica envolvendo grandes vasos, com etiologia incerta, tendo a dor torácica como um sintoma comum e desafiador, consequente à inflamação na raiz ou arco aórtico, artéria pulmonar ou coronárias. O objetivo deste estudo foi relatar a utilização da lidocaína por via endovenosa na abordagem da dor torácica intensa e refratária secundária à AT. RELATO DO CASO: Paciente do sexo feminino, 33 anos, com diagnóstico de AT, dor torácica intensa de difícil manejo, internada após consulta em serviço de emergência. Dor não responsiva ao tratamento tradicional após uma semana de ajustes em fármacos. Como opção terapêutica, foi escolhido o Bloqueio Simpático Venoso (BSV) com lidocaína, obtendo redução da dor de 10 para 3 na Escala Analógica Visual. Antes da alta hospitalar foi administrado infliximabe. Paciente foi reavaliada em consulta ambulatorial após 30 dias. CONCLUSÃO: Esta estratégia fora tratamento da dor torácica intensa permitiu redução e alívio da dor.

[A late onset diagnosis of giant cell arteritis in remission]. / Diagnostic tardif d'une artérite à cellules géantes en rémission.

Giant-cell arteritis (GCA) is a classical cause of chronical inflammation (CI) in the elderly, causing headaches, scalp hypersensitivity and jaw claudication. We describe a patient with a GCA revealed with a year-long biological inflammation and weight loss. Diagnosis was performed on a systematic temporal artery biopsy showing typical histological features. No treatment was intended as the patient had a spontaneous remission, maintained at one year of follow-up. This case highlights the benefit of a systematic temporal artery biopsy to explore CI and reminds us that GCA may undergo spontaneous remission.

[Hospitalization for infection in patients treated for giant cell arteritis: A single-centre retrospective study]. / Hospitalisation pour infection chez les patients suivis pour une artérite à cellules géantes : étude monocentrique rétrospective.

INTRODUCTION: Infections are associated with morbimortality of patients with giant cell arteritis (GCA). The aim of this work was twofold: the identification of factors predisposing to the risk of infection and the description of patients hospitalized with an infection occurring during the treatment period of CAG. METHODS: A monocentric retrospective study was conducted in GCA patients, comparing patients hospitalized for infection with patients without infection. The analysis included 21/144 (14.6%) patients with 26 infections (cases) and 42 control matched on sex, age, and diagnosis of GCA. RESULTS: Both groups were similar except for a higher frequency of seritis in cases (15% vs. 0%, p=0.03). Relapses of GCA were less common in cases (23.8% vs 50.0%, p=0.041). Hypogammaglobulinemia was present during infection. More than half of the infections (53.8%) occurred in the first year of follow-up with an average dose of 15mg/day of corticosteroids. Infections were mainly pulmonary (46.2%) and cutaneous (26.9%). CONCLUSION: Factors associated with infectious risk were identified. This preliminary monocentric work will continue with a national multicentre study.

Atteintes coronariennes et artérite a cellules géantes : à propos de 2 cas et revue de la littérature.

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.

[Hearing loss in giant cell arteritis: A case report]. / Surdité dans l'artérite à cellules géantes : à propos d'une observation.

INTRODUCTION: Hearing loss is a rare manifestation in giant cell arteritis. The different types of deafness are possible with a predominance of sensorineural deafness. CASE REPORT: We report a 75-year-old woman who presented with typical manifestations of giant cell arteritis associated concomitantly with the occurrence of bilateral mixed hearing loss confirmed on the audiogram. Corticosteroids allowed a rapidly favorable clinical and biological outcome. The follow-up audiogram at 3 months was markedly improved and showed a decrease in sensorineural hearing loss and disappearance of conductive hearing loss. CONCLUSION: Any rapid onset deafness in an inflammatory context in the elderly should lead to a search for giant cell arteritis. The diagnosis can be difficult in the absence of other typical manifestations, especially since the biopsy of the temporal artery most often comes back negative. Corticosteroids are usually effective.

Tongue Necrosis due to Giant Cell Arteritis / Necrose da língua por arterite de células gigantes

Giant Cell Arteritis is a vasculitis that mainly affects women over 50. The most common manifestations are headache, jaw claudication, and amaurosis. If not diagnosed early, it can lead to rare irreversible ischemic consequences, with tongue necrosis being one of these. We report a case of a previously undiagnosed patient with lateral tongue necrosis who responded well to oral corticosteroid treatment. The diagnosis is clinical, laboratory and histological and may be aided by imaging exams. Initial treatment is with oral corticosteroids, with methotrexate and tocilizumab as alternatives. Diagnostic suspicion and quick start of treatment favorably influence the prognosis of the disease (AU)

Apresentamos um caso de arterite de células gigantes com amaurose, seguida de necrose da língua, diagnosticado clinicamente com boa resposta terapêutica ao corticoide oral. Salientamos que os casos de necrose da língua são extremamente raros. A suspeita diagnóstica de arterite de células gigantes é de fundamental importância e mesmo na impossibilidade de realizar a biópsia de artéria temporal, não se deve retardar o início do tratamento sob pena de acarretar sequelas definitivas ou manifestações mais graves da doença (AU)

[Gynecologic vasculitis revealing a giant cell arteritis: A case report and literature review]. / Vascularite des artères utérines révélant une artérite à cellules géantes : cas clinique et revue de la littérature.

INTRODUCTION: Genital vasculitis are uncommon. They may be localized or be a manifestation of a systemic vasculitis. We report a patient with a giant cell arteritis (GCA) involving uterine arteries and a literature review on genital vasculitis. CASE REPORT: A 65-year-old woman was referred to a gynecologist for a cervical intraepithelial neoplasia (CIN) associated with an ovarian mass. An unexpected diagnosis of GCA involving small to medium sized uterine arteries was made through the anatomopathological analysis while the patient was asymptomatic. Two weeks later, she presented typical cranial symptoms of giant cell arteritis (GCA). PET-scanner confirmed the diagnosis of GCA with an involvement of the ascending aorta, and the axillary and the subclavian arteries. CONCLUSION: Gynecologic vasculitis are rare and usually an asymptomatic manifestations of GCA.

Giant cell arteritis with ocular involvement successfully treated with tocilizumab and very short-course glucocorticoids: A case report.

PURPOSE: To report the case of a patient with arteritic anterior ischemic optic neuropathy (AAION) with a history of glucocorticoids (GC) hypersensitivity treated with monthly intravenous tocilizumab (IV TCZ) and short course of GC. CASE REPORT: A 71-year-old Caucasian patient presented with AAION related to giant cell arteritis (GCA) confirmed by temporal artery biopsy. Past medical history was significant for GC hypersensitivity proven by allergy evaluation and the patient was managed with IV TCZ and very short-course GC. CONCLUSION: TCZ therapy with very short-course GC could be effective in GCA with ophthalmic involvement as a first-line strategy. Clinical trials are needed for thorough evaluation of the efficiency of TCZ as a first-line treatment to induce and maintain remission in patients with GCA and ocular involvement.

[Management of patients with a suspicion of giant cell arteritis: Survey among general practitioners and specialists]. / Prise en charge d'une suspicion d'artérite à cellules géantes : enquête auprès des médecins généralistes et spécialistes.

INTRODUCTION: Headache, visual disturbances and shoulder and hip girdle pain are frequent symptoms of consultation and the diagnosis of giant cell arteritis (GCA) can be evoked in these situations. However, GCA is a rare disease, and the management modalities of a clinical suspicion of GCA are poorly described, which warranted this study. METHODS: This is a survey evaluating the management of a suspected case of GCA. The questionnaires were sent to general practitioners (GPs), members of the French Rheumatology Society (SFR) and the French National Society of Internal Medicine (SNFMI) RESULTS: One thousand four hundred and fifty two physicians responded to the survey, including 967 GPs (66.6 %) and 485 other specialists (33.4 %). GPs immediately referred the patient to the emergency room in 42 % of cases, and to a specialist colleague in 72 % of cases in the presence of visual symptoms. GPs and other specialists reported performing temporal artery biopsy (TAB) respectively in 46.7 % and 69.7 % of cases (P<0.05). GPs and other specialists reported using diagnostic imaging in 7.4 % and 16.2 % of cases, respectively (P<0.05). Temporal artery ultrasound was the most used diagnostic imaging. The average prednisone equivalent dose prescribed as initial treatment was 1mg/kg/day for GPs and 0.7mg/kg/day for other specialists (P<0.05). CONCLUSION: Some suspected GCA patients would be managed by their GPs. Imaging was little used for the diagnosis of GCA and TAB remained the preferred diagnostic examination. The initial prednisone equivalent dose varied between GP and other specialists.

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.

[Delays in the management of ocular complications of giant cell arteritis: A retrospective monocentric study of 33 patients]. / Délais de prise en charge des complications visuelles d'artérite à cellules géantes : étude rétrospective monocentrique de 33 patients.

INTRODUCTION: Ocular complications of giant cell arteritis (GCA) can lead to irreversible bilateral blindness and represent a therapeutic emergency. Recommendations for the management of GCA have recently been updated. The objective of the study was to evaluate delays in appropriate management of the ocular complications of GCA and its determinants. METHOD: Retrospective, monocentric study, conducted over the period January 2013-November 2018. All consecutive patients with a final diagnosis of GCA and related visual impairment (permanent visual loss and/or alteration of visual field) were included. RESULTS: Thirty-three patients were included (women: 21, men: 12; mean age at diagnosis: 79). Twenty-seven patients (82%) presented with symptoms suggestive of ACG prior to the visual complication, ranging from a few weeks to several months. Seventeen patients (52%) had a known biological inflammatory syndrome (median CRP at 64 mg/L) prior to hospital consultation. The median time from the onset of permanent ophthalmologic manifestations to appropriate corticosteroid management was 3 days (range: 0-134). Two of the 21 patients who consulted an out-of-hospital ophthalmologist received corticosteroid therapy before referral to hospital. Three patients (9%) were treated within 24 h of the onset of the disorders. CONCLUSION: There is a significant delay in the appropriate management of ophthalmological complications of ACG and deviations from current recommendations. Numerous actions must therefore be taken to improve the visual prognosis of patients with ACG, both preventively (i.e. early diagnosis and treatment of ACG before the possible occurrence of visual complications), and curatively (rapid recognition and immediate treatment of ocular complications). These elements support the relevance of specific fast-track pathways for GCA.

[The current place of non-invasive large-vessel imaging in the diagnosis and follow-up of giant cell arteritis]. / Place actuelle de l'imagerie non invasive des artères de gros calibre dans l'artérite à cellules géantes: du diagnostic au suivi.

Large vessel involvement in giant cell arteritis has long been described, although its right frequency and potential prognostic value have only been highlighted for two decades. Large vessel involvement not only is associated with a high incidence of late aortic aneurysms, but also might cause greater resistance to glucocorticoids and longer treatment duration, as well as worse late cardiovascular outcomes. These data were brought to our attention, thanks to substantial progress recently made in large vessel imaging. This relies on four single, often complementary, approaches of varying availability: colour Doppler ultrasound, contrast-enhanced computed tomography with angiography and, magnetic resonance imaging, which all demonstrate homogeneous circumferential wall thickening and describe structural changes; 18F-fluorodeoxyglucose positron emission tomography-computed tomography (PET/CT), which depicts wall inflammation and assesses many vascular territories in the same examination. In addition, integrated head-and-neck PET/CT can accurately and reliably diagnose cranial arteritis. All four procedures exhibit high diagnostic performance for a large vessel arteritis diagnosis so that the choice is left to the physician, depending on local practices and accessibility; the most important is to carry out the chosen modality without delay to avoid false or equivocal results, due to early vascular oedema changes as a result of high dose glucocorticoid treatment. Yet, ultrasound study of the superficial cranial and subclavian/axillary arteries remains a first line assessment aimed at strengthening and expediting the clinical diagnosis as well as raising suspicion of large-vessel involvement. In treated patients, vascular imaging results are poorly correlated with clinical-biological controlled disease so that it is strongly recommended not to renew imaging studies unless a large vessel relapse or complication is suspected. On the other hand, a structural monitoring of aorta following giant cell arteritis is mandatory, but uncertainties remain regarding the best procedural approach, timing of first control and spacing between controls. Individuals at greater risk of developing aortic complication, e.g. those with classic risk factors for aneurysm and/or visualised aortitis, should be monitored more closely.

[Ischemic optic neuropathies]. / Neuropathies optiques ischémiques.

Ischemic optic neuropathies include any vascular disease of the optic nerve. Anterior ischemic optic neuropathies are classically distinguished from posterior ischemic optic neuropathies by the presence of optic disc edema in the former, and the absence thereof in the latter. Non-arteritic acute anterior ischemic optic neuropathy is the most common ischemic optic nerve disease. Its exact cause remains unknown. A disc at risk (small and crowded optic nerve) is a typical backdrop for the development of non-arteritic ischemic optic neuropathy. There is no curative or preventive treatment. Posterior ischemic optic neuropathy is exceedingly rare, compared to anterior ischemic optic neuropathy. It is more frequent in patients with cardiovascular risk factors or in the perioperative period. There is no treatment. In any case of ischemic optic neuropathy, an arteritic cause must be ruled out urgently through clinical and paraclinical examinations. The most frequent cause is giant-cell arteritis. In this case, emergency treatment with intravenous methylprednisolone is required in order to limit vision loss in the affected eye and to prevent vision loss in the other eye.