Giant Cell Arteritis Research Articles

Tocilizumab for Giant Cell Arteritis: Clinical Outcomes Following Relapses and Tocilizumab Discontinuation Due to Adverse Events.

Tocilizumab (TCZ) is effective for giant cell arteritis (GCA). However, little is known regarding treatment modification and clinical outcomes after unfavorable events, such as GCA relapses or TCZ discontinuation due to adverse events (AEs). This multicenter retrospective study included GCA patients who initiated TCZ from 2008 to 2021 at five Japanese hospitals. GCA relapses and TCZ-related AEs were monitored for two years after TCZ initiation. In patients with GCA relapses, subsequent clinical courses, including relapse symptoms and treatment modification, were followed for 90 days after the relapses. Similarly, patients who discontinued TCZ due to AEs were additionally followed until one year after the TCZ discontinuation to evaluate AEs, relapses, and treatment changes. Of 62 eligible patients, 10 patients (16%) relapsed after initiating TCZ therapy. Most relapses (8/10) occurred after extending TCZ intervals or discontinuing TCZ. Combinations of adjusting TCZ intervals, glucocorticoid (GC), and/or methotrexate (MTX) could manage the relapses without serious complications. In the entire cohort, AEs occurred in 28 patients (45%), and 8 patients (13%) discontinued TCZ due to AEs. After AE-related TCZ discontinuation, six patients attempted to taper GC without other immunosuppressive treatments, and four subsequently relapsed. In contrast, two patients who used other immunosuppressants or biological therapy could decrease GC without relapses. Although GCA relapses can occur after initiating TCZ therapy, most relapses can be safely managed by adjusting TCZ, GC, and/or MTX. Adding immunosuppressants or biological treatments may potentially be related to preventing relapses when patients discontinue TCZ due to AEs.

Alkaline phosphatase is useful for predicting giant cell arthritis complications in patients with polymyalgia rheumatica.

This study determined whether alkaline phosphatase can be used to distinguish giant cell arthritis complications in patients with polymyalgia rheumatica. This retrospective study included patients diagnosed with polymyalgia rheumatica between January 2014 and October 2023 at our hospital. The predictive accuracy of biomarkers for diagnosing giant cell arthritis was evaluated. Logistic regression was performed to identify factors predicting giant cell arthritis complications. In total, 128 participants were included in this study and divided into two groups: isolated polymyalgia rheumatica (n = 111) and polymyalgia rheumatica with giant cell arthritis (n = 17). The median alkaline phosphatase level of polymyalgia rheumatica with giant cell arthritis group was significantly higher than that of the isolated polymyalgia rheumatica group (242.0 [interquartile range, 221.0-595.0] vs. 187.0 [interquartile range 97.5-254.5] U/L, P < 0.001). Setting a cut-off value of 214 U/L for alkaline phosphatase yielded a sensitivity and specificity of 0.88 and 0.55, respectively, for diagnosing giant cell arteritis. Multivariate analysis revealed that alkaline phosphatase was a significant independent variable in the complications of giant cell arteritis (odds ratio, 25.2; P = 0.032). Alkaline phosphatase can help distinguish giant cell arthritis complications in patients with polymyalgia rheumatica.

Fourth Nerve Palsy as the Presenting Manifestation of Giant Cell Arteritis.

Fourth Nerve Palsy as the Presenting Manifestation of Giant Cell Arteritis.

Invasive Aspergillosis with Cavernous Sinus Thrombosis Following High-Dose Corticosteroid Therapy: A Challenging Case of Rhino-Orbital-Cerebral Mycosis

Invasive aspergillosis is a rare but severe fungal infection primarily affecting immunocompromised individuals. The Coronavirus Disease-2019 (COVID-19) pandemic has introduced new complexities in managing aspergillosis due to the widespread use of corticosteroids for treating COVID-19-related respiratory distress, which can increase susceptibility to fungal infections. Here, we present a challenging case of progressive cerebral aspergillosis complicated by cavernous sinus thrombosis (CST) in a 67-year-old male with a history of COVID-19. The patient, initially misdiagnosed with temporal arteritis, received pulse corticosteroid therapy twice before presenting with persistent left-sided headaches and vision loss. Cranial imaging revealed findings consistent with fungal sinusitis, Tolosa–Hunt syndrome, and orbital pseudotumor, which progressed despite initial antifungal therapy. Subsequent magnetic resonance imaging indicated an invasive mass extending into the left cavernous sinus and other intracranial structures, raising suspicion of aspergillosis. A transsphenoidal biopsy confirmed Aspergillus infection, leading to voriconazole therapy. Despite aggressive treatment, follow-up imaging revealed significant progression, with extension to the right frontal region and left cavernous sinus. The patient then developed visual impairment in the right eye and was diagnosed with CST secondary to fungal sinusitis. Management included a combination of systemic antifungals and antibiotics; however, the patient declined surgical intervention. This case underscores the diagnostic challenges and rapid progression associated with cerebral aspergillosis in post-COVID-19 patients treated with corticosteroids. This report highlights the need for heightened clinical suspicion and prompt, targeted interventions in similar cases to improve patient outcomes. Further research is required to understand the optimal management of invasive fungal infections.

18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in Large-Vessel Vasculitis During Active and Inactive Disease Stages Is Associated with the Metabolic Profile, but Not the Macrophage-Related Cytokines: A Proof-of-Concept Study

Giant cell arteritis (GCA) is an autoimmune/autoinflammatory disease affecting large vessels in patients over 50 years old. The disease presents as an acute inflammatory response with two phenotypes, cranial GCA and large-vessel vasculitis (LV)-GCA, involving the thoracic aorta and its branches. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET-CT) is among the imaging techniques contributing to diagnosing patients with systemic disease. However, its association with soluble inflammatory markers is still elusive. This proof-of-concept study aims to identify novel soluble serum biomarkers in PET/CT-positive patients with LV-GCA and associate them with active (0 months) and inactive disease (6 months following treatment), in sequential samples. The most-diseased-segment target-to-background ratio (TBRMDS) was calculated for 13 LV-GCA patients, while 14 cranial GCA and 14 Polymyalgia Rheumatica patients with negative initial PET/CT scans served as disease controls. Serum macrophage-related cytokines were evaluated by cytometric bead array (CBA). Finally, previously published NMR/metabolomics data acquired from the same blood sampling were analyzed along with PET/CT findings. TBRMDS was significantly increased in active versus inactive disease (3.32 vs. 2.65, p = 0.006). The analysis identified nine serum metabolites as more sensitive to change from the active to inactive state. Among them, choline levels were exclusively altered in the LV-GCA group but not in the disease controls. Cytokine levels were not associated with PET/CT activity. Combining CRP, ESR, and TBRMDS with choline levels, a composite index was generated to distinguish active and inactive LV-GCA (20.4 vs. 11.62, p = 0.001). These preliminary results could pave the way for more extensive studies integrating serum metabolomic parameters with PET/CT imaging data to extract sensitive composite disease indexes useful for everyday clinical practice.

Diagnostic Performance of a Newly-Launched Canadian Fast-Track Ultrasound Clinic by Rheumatologists for the Diagnosis of Giant Cell Arteritis.

Giant Cell Arteritis (GCA) can present diagnostic challenges and early diagnosis is crucial due to potential ischemic complications. Recent guidelines suggest that a suspected diagnosis should be confirmed with temporal artery biopsy or imaging, including ultrasound (US). In our Canadian setting, point-of-care temporal artery US was near unavailable, and biopsy remains the standard of care. We hypothesize that launching a fast-track US clinic by rheumatologists may spare the need for a temporal artery biopsy. Therefore, this study aimed to assess the diagnostic performance of US in this newly-launched fast-track clinic. In this single-center retrospective cross-sectional analysis, 99 visits were identified from the fast-track clinic between January 2020 and July 2022. Each subject had an US according to a standard protocol for suspicion of either new-onset or relapse of GCA. Ultrasonographers were rheumatologists who acquired training on vascular US techniques before launching the clinic. For each patient presenting with suspected new-onset GCA, the pre-test probability was calculated using the Southend GCA probability score. The sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were calculated using the rheumatologist clinical diagnosis as the gold standard for GCA diagnosis. A total of 22 subjects had a diagnostic of GCA and 77 had another diagnostic. Patients with and without GCA were, respectively, 81.8% vs 72.7% females, had a mean age of 76.6 ± 7.7 vs. 74.8 ± 9.8 years and a mean CRP of 73.4 ± 57.8 vs 38.3 ± 59.9 mg/L. Temporal artery US demonstrated a a sensitivity of 86.3% [95% confidence interval (CI), 65.1%-97.1%], a specificity of 90.9% (95% CI, 82.2%-96.3%), a PPV of 73.1% (95% CI, 56.8%-84.9%) and a NPV of 95.9% (95% CI, 89.0%-98.5%). 14 patients had a suspicion of relapse and were all correctly identified by the US. Among those with suspicion of new-onset 27, 34 and 24 US were performed for high, intermediate, and low pretest probability of GCA, respectively. The high-risk subgroup demonstrated higher PPV while similar sensitivity/specificity were observed between all three subgroups. Our results highlights the benefits of US as a key diagnostic tool for GCA, particularly when combined with clinical evaluations. An excellent discriminative ability for diagnosis of GCA was shown in this newly-launched clinic suggesting that the role of TAB may need to be redefined. These findings will guide on broader implementation of US programs for GCA.

Infective agents and polymyalgia rheumatica: key discussion points emerging from a narrative review of published literature

IntroductionThe aetiology of polymyalgia rheumatica (PMR) is unknown. Recently, reports on cases of PMR following the coronavirus disease 2019 (COVID-19) have revived the role of infection as an aetiological or triggering factor. It is estimated that patients with PMR have manifestations of giant cell arteritis (GCA) in &lt; 20% of cases. To date, little is known on the potential role of infectious agents in facilitating this association. Given this background, we performed a review of published literature. Our first aim was to review and discuss the relationship between PMR and infective agents. Secondly, we compared data of PMR-only patients with PMR and overlapping GCA to seek any commonalities or differences regarding the type of infectious agent in these two subgroups.Material and MethodsWe performed a non-systematic literature search on Embase and Medline (COVID interface) with the following search terms: “polymyalgia rheumatica” AND “infections” OR “infectious agents”, both MESH headings and free-text (in each language they were written). Each paper’s reference list was scanned for additional publications meeting this study’s aim. When papers reported data partially presented in previous articles, we referred to the most recent published data. Abstracts submitted at conferences or from non-peer-reviewed sources were not included. Polymyalgia rheumatica following vaccinations was an additional exclusion criterion.ResultsSeveral infectious agents have been held responsible for PMR. However, no definite causal link has been identified so far. According to our review, the search for a specific infectious agent, however intriguing, appears to be stagnating. Genetic background and epigenetic regulation probably play a key role. However, topical studies are lacking. Polymyalgia rheumatica as an adverse event following immunization should be kept methodologically distinct from PMR following an acute infection, as the adjuvants in the vaccine can make a significant difference.ConclusionsFinally, some infectious agents are able to replicate in human arteries or have an endothelium tropism. Whilst these can theoretically trigger GCA, their role in isolated PMR seems minimal.

Norwegian society of rheumatology recommendations on diagnosis and treatment of patients with Polymyalgia Rheumatica: a narrative review

BackgroundTo provide evidence-based, up-to-date recommendations for physicians in primary and specialist healthcare setting in diagnosing and treating patients with polymyalgia rheumatica (PMR).MethodsThe PMR working group conducted a narrative review of the available evidence in the field and wrote the draft guidelines. These guidelines were discussed and revised according to the standard operating procedures within the Norwegian Society of Rheumatology. The European Alliance of Associations for Rheumatology (EULAR) and American College of Rheumatology (ACR) recommendations for the management of PMR, the British Society for Rheumatology (BSR) guidelines for the management for PMR, the treat-to-target recommendations in giant cell arteritis and PMR and the 2023 recommendations for early referral of individuals with suspected polymyalgia rheumatica were used in particular for purpose of harmonization.ResultsA total of 10 recommendations have been formulated covering initial diagnostic investigations, comorbidity assessment, imaging, specialist referral criteria, treatment involving glucocorticoids and steroid-sparing agents and follow-up care.ConclusionNorwegian recommendations for diagnostics and treatment to improve management and outcome in patients with PMR were developed.

Further clinical data on the more rapid achievement of remission without the use of steroids with tocilizumab compared to methotrexate in giant-cell arteritis.

Further clinical data on the more rapid achievement of remission without the use of steroids with tocilizumab compared to methotrexate in giant-cell arteritis.

Use of immunosuppressants and biologics in giant cell arteritis: Recommendations of the French Study Group for Large Vessel Vasculitis (GEFA)

PurposeAn updated revision of the 2016 recommendations from the French Study Group for Large Vessel Vasculitis (GEFA) was needed to better delineate the place and management of immunosuppressants or biologics in giant cell arteritis (GCA). MethodsA panel of 18 physicians, including internists and rheumatologists, constituted the task force of this project and drafted the recommendations. Twelve additional readers were asked to analyse and comment on the recommendations. Two face-to-face virtual meetings were held to discuss and validate the recommendations. Each member voted individually, and a>85% consensus was required to validate each recommendation. ResultsFrom the initial 6 questions, 26 recommendations were validated. The following main recommendations were validated. (1) Subcutaneous 162mg tocilizumab (TCZ) for at least 12months should be used first when glucocorticoid (GC)-sparing treatment is needed with the objective of discontinuing GCs within the subsequent 6months. (2) GCA patients who have experienced any of the following conditions must receive TCZ at GCA diagnosis with 6months of GC therapy: major cardiovascular event, osteoporosis with fracture, psychiatric event with GC use, complicated diabetes mellitus, or any previous>6months of GC treatment. (3) In patients in whom GC discontinuation is not possible after 12months of treatment because of persistent disease activity or in patients in whom GC-related adverse events are unacceptable, TCZ (or alternatively methotrexate) may be proposed. ConclusionsThese recommendations were constructed based on the results of the published literature and the experts’ experiences to standardise therapeutic practices in France. Further updates will likely be necessary following new publications.

Atypical Signs and Symptoms of Giant Cell Arteritis: A Systematic Review.

Giant cell arteritis can present with atypical manifestations that delay treatment and risk severe complications. To comprehensively describe all atypical signs/symptoms of giant cell arteritis. In this systematic review, we searched MEDLINE, Embase, and Cochrane Central Register of Controlled Trials from inception to October 2022. Primary research articles that included at least one participant with an atypical sign/symptom of biopsy-proven giant cell arteritis were included. Study screening and data extraction were performed in duplicate. The primary outcome was the proportion of participants with atypical giant cell arteritis features. Time to treatment was compared between participants with atypical giant features only and participants with both typical and atypical features. Of 21,828 screened records, 429 studies corresponding to 746 individuals (median [IQR] age 72 [IQR, 66-78] years, 63% female) with at least one atypical feature of GCA were included. Eighty-two percent had both atypical and at least one concurrent typical giant cell arteritis feature, whereas 18% of patients with atypical signs and symptoms only presented with atypical features. Patients with atypical symptoms presented to clinicians earlier than patients with typical features (p < 0.001). There was no difference between groups in proportion to elevated ESR and CRP (82.3% vs. 83.35%, p = 0.91) or mortality rate (8.2% vs. 10.8%, p = 0.42). Patients with atypical features only experienced greater delay in treatment initiation (p < 0.001). The most commonly reported atypical signs/symptoms were vertigo (11.9%), scalp necrosis/ulceration (7.9%), and dry cough (5.8%). Eighteen percent of biopsy-proven giant cell arteritis cases with at least one atypical feature have only atypical features and are more likely to experience delays in treatment. Clinicians should be aware of atypical signs/symptoms of giant cell arteritis and order inflammatory markers early to prevent giant cell arteritis-associated morbidity.

What is new in imaging to assist in the diagnosis of giant cell arteritis and Takayasu's arteritis since the EULAR and ACR/VF recommendations?

Over the past decades, fundamental insights have been gained to establish the pivotal role of imaging in the diagnosis of large-vessel vasculitis, including giant cell arteritis (GCA) and Takayasu's arteritis (TAK). A deeper comprehension of imaging modalities has prompted earlier diagnosis leading to expedited treatment for better prognosis. The European Alliance of Associations in Rheumatology (EULAR) recommended in 2023 that ultrasound should be the initial imaging test in suspected GCA, and Magnetic Resonance Imaging (MRI) remains the first-line imaging modality in suspected TAK. We summarize the recent advances in diagnostic imaging in large vessel vasculitis, highlighting use of combination imaging modalities, and discuss progress in newer imaging techniques such as contrast-enhanced ultrasound, shear wave elastography, ocular ultrasound, ultrasound biomicroscopy, integration of Positron Emission Tomography (PET) with MRI, novel tracer in PET, black blood MRI, orbital MRI, and implementation of artificial intelligence (AI) to existing imaging modalities. Our aim is to offer a perspective on ongoing advancements in imaging for the diagnosis of GCA and TAK, particularly innovative technology, which could potentially boost diagnostic precision.

Traditional and Emerging Strategies for Managing Polymyalgia Rheumatica: Insights into New Treatments.

Background/Objectives: Polymyalgia Rheumatica (PMR) is an inflammatory condition that primarily affects individuals aged 50 and older, especially in Western countries. Although glucocorticoids are the cornerstone of PMR treatment, these drugs are associated with side effects, making it advisable to use them for the shortest duration possible. However, tapering or discontinuation of glucocorticoids often leads to disease relapses. In this review, we focus on the traditional management of PMR, as well as the potential for therapies that may reduce glucocorticoid use. Special attention is given to the efficacy of biologic agents in PMR management. Methods: A literature review, primarily based on articles published in PubMed, was conducted. In addition to discussing various glucocorticoids and conventional disease-modifying drugs used for the management of isolated PMR, this review specifically focused on the information reported regarding new therapies, with particular emphasis on biologic agents. Results: Prednisone or prednisolone at a dose ranging between 12.5 and 25 mg/day is the agreed-upon treatment for PMR. Due to the side effects associated with prolonged glucocorticoid use and the high frequency of relapses when glucocorticoids are tapered, glucocorticoid-sparing agents have emerged as tools in the management of PMR. Methotrexate has traditionally been the conventional disease-modifying antirheumatic drug (DMARD) unanimously recommended for use in PMR. Other conventional DMARDs, such as leflunomide, have shown promising results but require further study. The use of biologic agents has marked a significant step forward in the management of PMR. While anti-TNF agents failed to provide beneficial effects in isolated PMR, anti-IL-6 receptor agents, such as tocilizumab and sarilumab, have demonstrated efficacy in reducing relapse frequency, lowering the cumulative glucocorticoid burden, and achieving long-term remission of the disease. Other biologic agents, many of which have been used in giant cell arteritis, as well as Janus kinase (JAK) inhibitors, are currently under investigation. Conclusions: Glucocorticoids are the primary treatment for isolated PMR but are associated with comorbidities, especially in patients with pre-existing conditions or frequent relapses. Glucocorticoid-sparing agents, such as methotrexate and biologics, in particular tocilizumab and sarilumab, offer alternatives, improving symptoms and reducing glucocorticoid use. While biologic agents reduce long-term side effects and help achieve disease remission, their use must consider potential side effects and higher costs compared to traditional therapies.

Baricitinib in polymyalgia rheumatica and giant cell arteritis: report of six cases.

The objective of this case series is to describe the efficacy and safety of baricitinib (BARI) in a group of patients with polymyalgia rheumatica (PMR) and/or giant cell arteritis (GCA). These patients were treated with BARI due to either a refractory disease course or the unavailability of tocilizumab because of the pandemic. A total of six patients (five females and one male, median age 64 years, range 50-83) were treated with BARI. Two of them had isolated PMR, two had PMR with associated large vessel (LV)-GCA, one had LV-GCA presenting as fever of unknown origin, and one had cranial-GCA. All patients reported improvement with BARI. At the time of starting BARI, patients were taking a median prednisone dose of 8.75 mg/day (range 0-25), and the four patients with PMR had a median PMR-AS of 23.3 (indicating high disease activity), which decreased to 1.58 after 6 months of treatment with BARI. Two of them could stop glucocorticoids (GC) and continued BARI monotherapy. One patient suffered from pneumonia, and BARI was therefore stopped. No other adverse events attributable to BARI were detected. Our case series supports previous reports suggesting efficacy of Janus kinase inhibitors as a GC-sparing strategy in PMR and GCA.

FDG PET/CT in large vessel vasculitis.

Large vessel vasculitides (LVV) such as giant cell arteritis, Takayasu arteritis and aortitis/periaortitis are characterised by immune-mediated inflammation of medium to large arteries. Clinical disease manifestations can be non-specific and diagnostic imaging plays an important role in the diagnostic pathway. In recent years, FDG PET/CT has proven to be a powerful metabolic tool that can provide a wholed body, non-invasive assessment of vascular inflammation. This review outlines the clinical features of large vessel vasculitis and the closely related entity of polymyalgia rheumatica, summarises the evidence for FDG PET/CT in the assessment of these conditions, and provides guidance for patient preparation, image acquisition and interpretation.

Comparison and Significance of Contrast-Enhanced Computed Tomographic Findings of Large-Vessel Vasculitis Before and After Treatment: Differences Between Takayasu Arteritis and Giant Cell Arteritis.

Imaging is essential for diagnosing large-vessel vasculitis (LVV). During diagnostic imaging, assessing disease activity and vascular damage separately is important. Acute-phase findings represent disease activity, while chronic-phase findings represent vascular damage; however, whether the imaging findings are acute or chronic may be unclear. We investigated how vascular lesions change before and after treatment and whether they were acute- or chronic-phase findings. Fifty-one patients with LVV who had undergone contrast-enhanced computed tomography (CT) scans from the neck to the pelvis before treatment and 1-4 months after treatment were recruited. Wall thickening, wall contrast enhancement, stenosis, occlusion, dilation, aneurysm, and calcification were semi-quantitatively assessed in 21 vessels from the common carotid to the common iliac artery. Twenty-four patients were diagnosed with Takayasu arteritis (TAK), and 27 with giant cell arteritis (GCA). Wall thickening and wall contrast enhancement improved after the treatment, which was especially significant in the GCA group. No significant differences in stenosis, occlusion, dilation, aneurysm, or calcification were observed before and after treatment. Stenosis and occlusion were more common with TAK, while calcification was more common with GCA. Wall thickening and wall contrast enhancement are acute-phase findings (activity), while stenosis, occlusion, dilation, aneurysm, and calcification are chronic-phase findings (damage). The frequencies of these findings differ between TAK and GCA.

Imaging in Large Vessel Vasculitis-A Narrative Review.

Vasculitis refers to a group of rare conditions characterized by the inflammation of blood vessels, affecting multiple systems. It presents a diagnostic and therapeutic challenge due to its broad clinical manifestations. Vasculitis is classified based on the size of the affected vessels: small, medium, large, or variable-sized. Large vessel vasculitis (LVV), particularly giant cell arteritis (GCA) and Takayasu arteritis (TAK), has garnered attention due to its significant morbidity and mortality. Both conditions involve immune-mediated inflammation of the vascular wall, despite differing in epidemiology and presentation. Early identification is crucial to prevent complications like organ ischemia and hemorrhage. Diagnostic accuracy can be hampered by false negative results, making comprehensive investigation essential. Vascular imaging, including computed tomography angiography (CTA), ultrasound (US), magnetic resonance imaging (MRI), and positron emission tomography-computed tomography (PET-CT), is key in diagnosing vasculitis, revealing vessel wall thickening and other suggestive features. This article reviews typical and atypical CT and CTA findings in LVV, discusses imaging modalities, and highlights their role in therapeutic management and prognosis. It emphasizes the importance of a multidisciplinary approach and the critical role of radiologists in improving patient outcomes in LVV.

Characteristics associated with anterior ischemic optic neuropathy (AION) in giant cell arteritis (GCA).

Giant cell arteritis (GCA) is the most common vasculitis and can result in blindness due to anterior ischemic optic neuropathy (AION). Little is known about which patients with GCA are at higher risk of AION.We did a retrospective chart review to compare demographics, past medical history, labs and imaging of GCA patients with and without AION. All patients at the University of Washington who were diagnosed with GCA by a rheumatologist, had a vascular ultrasound and met classification criteria for GCA were included. AION was diagnosed by an ophthalmologist. To compare demographics, symptoms, presentation imaging and lab findings, we used Pearson Chi-square, Fisher's exact test and t-tests to compare GCA patients with and without AION. 91 patients with GCA without vision loss were compared to 15 patients with GCA and AION.The AION group had significantly more men (p=0.03) and elevated C-reactive protein (CRP) as compared to the non-AION group (p = 0.04). Rates of hypertension, smoking, erythrocyte sedimentation rate (ESR) and classification scores were similar. Vascular ultrasound showed similar rates of small and large vessel vasculitis, but the AION group had higher halo scores, with the AION group having a mean score of 5 and patients with GCA and without AION having a mean score of 3 (p < 0.01). Older men with GCA and more than three halos on vascular ultrasound imaging of GC may be at higher risk of AION. Key Points • This is the first paper to incorporate the use of vascular ultrasound in comparing patients with anterior ischemic optic neuropathy (AION) to those without giant cell arteritis (GCA). • Male sex is associated with AION despite GCA being more common in women. • Older age and higher CRP are associated with AION. • GCA patients with AION had a higher halo score than GCA patients without AION.

The value of ultrasound for monitoring disease activity in giant cell arteritis

Giant cell arteritis (GCA) is the most common primary systemic vasculitis in the elderly. Although the diagnosis of GCA has improved, monitoring its disease activity remains challenging due to the lack of validated tools and biomarkers. The current reliance on assessing symptoms, physical signs, and inflammatory markers during disease follow-up presents limitations, notably the nonspecific nature of GCA-related symptoms and the suppressive impact of IL-6 inhibitors on inflammatory markers. Therefore, recent attention has shifted toward acknowledging imaging as a monitoring tool, particularly ultrasound, given its widespread accessibility, cost-effectiveness, and well-established role in GCA diagnosis. Research on this topic has found that ultrasound characteristics, including the number of affected arterial segments and halo size, are associated with laboratory markers and treatment response, underscoring the ultrasound’s potential as a monitoring tool for GCA. It has also been demonstrated that ultrasound abnormalities progress differently throughout the disease course, depending on the type of arterial involvement, with vessel wall changes in the axillary arteries resolving more slowly than those in the temporal arteries. Nevertheless, there are still no studies comparing the added value of regular ultrasounds for monitoring disease activity to clinical and laboratory monitoring alone; hence, this imaging modality is not yet recommended for patients with GCA in clinical and biochemical remission. This narrative review aims to synthesize the main research findings of key studies addressing the role of ultrasound for monitoring disease activity in GCA, with a focus on the pattern of arterial involvement. It highlights the potential of ultrasound, particularly halo sign assessment, for evaluating disease progression but notes that further validation and standardization of protocols are needed to improve accuracy and enable routine use.

Multimodality imaging of pulmonary artery aneurysms due to giant cell arteritis: a case series.

Pulmonary artery aneurysms (PAAs) are rare, but important vascular phenomena with nonspecific signs and symptoms. While some patients remain asymptomatic, others manifest with serious symptoms such as chest pain, dyspnea, or hemoptysis. Often diagnosed incidentally, PAAs have various underlying etiologies, including congenital, acquired, and idiopathic. Giant cell arteritis (GCA) is a very rare cause of PAAs, with limited reported cases in the literature. This case series will review the clinical presentation, multimodality imaging, and management of four patients with isolated PAA due to GCA.