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CONTEXT: Stroke related to giant cell arteritis (GCA) is rare and is associated with a poor outcome. One of the putative ischemic mechanisms is narrowing of the arterial lumen due to wall infiltration by inflammation and intimal proliferation, leading to reduced distal blood flow. It was hypothesized that GCA-related stroke could predominate in watershed areas (WA). METHODS: Literature review including all cases of GCA-related stroke with brain images. RESULTS: Among 75 cases of GCA-related stroke, the anterior and posterior territories were involved in 48 % and 62.6 %, respectively. Up to 88.9 % of cases of anterior stroke probably involved WA. WA lesions in the posterior territories were as follows: uni/bilateral middle cerebellar peduncle (MCP) lesions in 25.5 %, and with less confidence, non-wedge-shaped cerebellar lesions in 46.8 %, or combined lesions in 61.7 %. Stenosis or occlusion of the afferent artery was almost always observed. A few lesions were not easily explained by low flow. DISCUSSION: Despite the limitations of arterial territory allocation especially in the posterior circulation, ischemic lesions mainly occurred in WA. MCP lesions, which were typically WA, were highly characteristic of GCA. Low flow downstream focal stenosis was the main, but not the unique, ischemic mechanism of GCA stroke.

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BACKGROUND: This report presents the management of patient with extracranial internal carotid artery pseudoaneurysm due to giant cell arteritis. CASE PRESENTATION: Left internal carotid artery pseudoaneurysm was diagnosed in a 57-year-old Ukrainian woman, which became a direct indication for surgical treatment involving aneurysm resection and internal carotid artery reimplantation. The used reconstruction technique with oblique cutting of internal carotid artery, aneurysm resection, ellipse-form anastomosis formation, and distal intima fixation prevents the dissection, restenosis, and aneurysm of anastomosis in the long-term postoperative period. Histopathological examination revealed the giant cell arteritis of the internal carotid artery. CONCLUSION: This case emphasizes the importance of open surgical treatment of extracranial carotid artery aneurysms, which allows to perform optimal carotid artery reconstruction and also define the rare etiology of disease.

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We systematically reviewed the literature to investigate the clinical features of isolated arteritic retinal artery occlusion (A-RAO) associated with giant cell arteritis (GCA). The four primary types of A-RAO were central retinal artery occlusion (CRAO), hemi-central retinal artery occlusion (hCRAO), branch retinal artery occlusion (BRAO), and cilioretinal artery occlusion (CLRAO). The most reported presentation was unilateral CRAO, followed by bilateral CRAO, unilateral CLRAO, and bilateral BRAO. Most RAOs were accompanied by typical GCA signs and symptoms, which can help distinguish them from non-arteritic RAOs. When reported, temporal artery biopsy confirmed GCA in most cases. Patients with GCA may present with a broad spectrum of isolated unilateral and bilateral A-RAOs. [Ophthalmic Surg Lasers Imaging Retina 2024;55:536-540.].

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OBJECTIVES: To evaluate differences in presentation and outcome of giant cell arteritis (GCA) patients with and without polymyalgia rheumatica (PMR) symptoms. METHODS: Consecutive patients diagnosed with GCA between 2000 and 2020 and followed for ≥12 months at the University Hospitals Leuven (Belgium), were included retrospectively. RESULTS: We included 398 GCA patients, of which 181 (45%) with PMR symptoms. Patients with PMR symptoms had a longer symptom duration (11 vs 6 weeks, p < 0.001). They less frequently reported fever (19% vs 28%, p = 0.030) and fatigue (52% vs 64%, p = 0.015) and tended to have less permanent vision loss (12% vs 19%, p = 0.052). There was no difference in the cumulative oral GC dose at 2 years (4.4 vs 4.3 g methylprednisolone, p = 0.571). However, those with PMR symptoms were treated with higher GC doses during subsequent follow-up (p < 0.05 from 38 months after diagnosis) and had a lower probability of stopping GC (62% vs 71%, HR 0.74 [95%CI 0.58-0.94], p = 0.018) with a longer median duration of GC treatment (29 vs 23 months, p = 0.021). In addition, presence of PMR symptoms was associated with an increased risk of relapse (64% vs 51%, HR 1.38 [95%CI 1.06-1.79], p = 0.017) with a higher number of relapses (1.47 [95%CI 1.30-1.65] vs 1.16 relapses [95%CI 1.02-1.31], p = 0.007). Patients with PMR symptoms less frequently developed thoracic aortic aneurysms during follow-up (3% vs 11%, p = 0.005). CONCLUSION: GCA patients with PMR symptoms had more recalcitrant disease with a higher risk of relapse and longer duration of GC treatment with need for higher GC doses.

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INTRODUCTION: Giant cell arteritis (GCA) presents two major phenotypes - cranial (cGCA) and extracranial (exGCA). exGCA may be overlooked. The study aimed to compare the clinical characteristics between cGCA and exGCA. METHODS: Electronic medical records of patients treated between January 2015 and July 2023 at the Department of Rheumatology were searched for the diagnosis of GCA. The clinical characteristics of patients with cGCA, exGCA, and overlapping GCA manifestations were compared. RESULTS: Out of 32 patients with GCA, 20 had cGCA, 7 had exGCA, and 5 had overlap manifestations. The groups did not differ significantly in demographics, clinical signs/symptoms, or laboratory test results. Importantly, the combined group of patients with exGCA and overlap GCA had a statistically significant delay in initiating treatment (median 12 weeks) compared to patients with cGCA (median 4 weeks; p = 0.008). CONCLUSION: Our study confirmed the insidious nature of exGCA, which lacks distinctive clinical symptoms and consequently leads to delayed treatment.

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Giant Cell Arteritis (GCA), also known as Temporal Arteritis, is a type of large vessel vasculitis primarily affecting the elderly population. It typically manifests with headaches, visual impairment, and jaw claudication. Although third nerve palsy as the primary presentation of GCA is rare, it has been reported in previous instances. In this report, we describe the case of a patient presenting with pupil-sparing third nerve palsy, ultimately diagnosed with GCA, and successfully managed with steroids and tocilizumab. A lady in her 80s with past medical history of well-controlled hypertension, bladder cancer in remission, a twenty-pack year smoking history, cervical and lumbar spine stenosis, and recent immunizations presented with acute onset of right-sided pupil-sparing third nerve palsy. Labs were pertinent for an elevated ESR and CRP. Brain imaging was without acute abnormalities. A temporal artery biopsy established evidence of healed arteritis and a diagnosis of GCA was made. The patient was treated with pulse-dose steroids followed by an oral steroid taper and tocilizumab. At one month follow-up, there was partial resolution in her ophthalmoplegia. We underscore the importance of considering temporal arteritis as a potential cause of third nerve palsy in the elderly before attributing it solely to microvascular ischemia, particularly in patients with constitutional features. Additionally, in our comprehensive literature review, we aim to consolidate the existing data from similar presentations, shedding light on the clinical manifestation and disease trajectory.

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Different types of vasculitis can be distinguished according to the blood vessel's size that is preferentially affected: large-vessel, medium-vessel, and small-vessel vasculitides. Giant cell arteritis (GCA) and Takayasu's arteritis (TAK) are the main forms of large-vessel vasculitis, and may lead to lumen narrowing. Clinical manifestations of arterial narrowing on the short- and long term include vision loss, stroke, limb ischemia, and heart failure. Imaging tools are well established diagnostic tests for large-vessel vasculitis and may aid therapy monitoring in selected cases while providing important information regarding the occurrence of vascular damage, tissue and organ complications. This review aims to provide the current status of multimodality imaging for the diagnosis and identification of vascular complications in the field of large vessel vasculitis.

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OBJECTIVES: To determine the incidence rate (IR) of myocardial infarction (MI), relative risk of MI, and impact of incident MI on mortality in individuals with biopsy-confirmed giant cell arteritis (GCA). METHODS: MIs in individuals diagnosed with GCA 1998-2016 in Skåne, Sweden were identified by searching the SWEDEHEART register, a record of all patients receiving care for MI in a coronary care unit (CCU). The regional diagnosis database, with subsequent case review, identified GCA patients receiving care for MI outside of a CCU. A cohort of 10 reference subjects for each GCA case, matched for age, sex and area of residence, was used to calculate the incidence rate ratio (IRR) of MI in GCA to that in the general population. RESULTS: The GCA cohort comprised 1134 individuals. During 7958 person-years of follow-up, 102 were diagnosed with incident MI, yielding an IR of 12.8 per 1000 person-years (95% CI 10.3 to 15.3). The IR was highest in the 30 days following GCA diagnosis and declined thereafter. The IRR of MI in GCA to that of the background population was 1.29 (95% CI 1.05 to 1.59). Mortality was higher in GCA patients who experienced incident MI than in those without MI (HR 2.8; 95% CI 2.2 to 3.6). CONCLUSIONS: The highest incidence of MI occurs within the 30 days following diagnosis of GCA. Individuals with GCA have a moderately increased risk of MI compared with a reference population. Incident MI has a major impact on mortality in GCA.

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INTRODUCTION: Parasitic infections could be an important triggering factor for autoimmune diseases. We present a clinical case of concomitant polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) induced with cystic echinococcosis (CE). CASE PRESENTATION: A 74-year-old male was admitted with a 2-month history of progressive pain at the shoulders and hip, movement restriction, and constitutional symptoms. As a result of the examinations performed due to rheumatological complaints, PMR and GCA were diagnosed. The cystic appearance, which was incidentally detected in the liver 6 months ago and not examined at that time, was found to be hydatid cyst. Medical treatment was initiated for all three conditions and the patient's symptoms improved significantly. DISCUSSION: Parasite infections may cause various autoimmune diseases because of molecular mimicry or sustained immune activation. Echinococcus granulosus is a very complex multicellular parasite and highly immunogenic for humans. Some body parts of the parasite, the outer surface and secreted particles, stimulate the host immune system strongly. CONCLUSION: The first case in the literature of coexistence of PMR and GCA associated with CE. Autoimmune diseases should be evaluated in patients with CE. Furthermore, CE should be considered in patients with autoimmune diseases in the presence of a cyst.

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OBJECTIVES: Giant cell arteritis (GCA) in patients with systemic sclerosis (SSc) is rare, and optimal treatment strategies for this group of patients have not been defined. We aim to describe the first case series of GCA/SSc overlap. METHODS: A single-institution retrospective study was performed reviewing all patients that had diagnosis codes for both SSc and GCA between January 1, 1996, and December 31, 2020. Demographic characteristic, clinical presentation, diagnostic modality, treatment, and outcome data were abstracted. Diagnosis of both SSc and GCA by a rheumatologist was required for inclusion. RESULTS: Eight patients were retrospectively identified, all of which were female. Seven patients fully met both respective ACR/EULAR classification criteria sets. One patient fulfilled GCA criteria and had 8/9 points for SSc criteria plus an oesophagogram which was consistent with clinical diagnosis of SSc. Three patients had a previous history of scleroderma renal crisis (SRC) prior to glucocorticoid initiation for GCA. No episodes of SRC occurred following initiation of glucocorticoids. Three patients were treated with tocilizumab. One patient developed a diverticular perforation while on tocilizumab requiring colonic resection and colostomy, one patient discontinued tocilizumab after a medication-unrelated complication and one patient has remained on treatment and in remission. CONCLUSIONS: Herein we present the largest single-institution series of patients with a history of GCA and SSc, an uncommon combination. Glucocorticoid treatment for GCA did not precipitate SRC, even in those with prior history of SRC. Further investigation regarding the benefit of tocilizumab in patients with SSc and GCA is required.

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ABSTRACT: Giant cell arteritis is a medium to large vessel vasculitis involving most commonly temporal arteries, vertebral and ophthalmic arteries. Giant cell arteritis causing mesenteric ischemia is not so frequent, more so if it occurs without cranial manifestations. Here we present an unusual case of a 26-year-old male patient presenting with subacute intestinal obstruction due to giant cell arteritis leading to mesenteric ischemia.

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