Diagnostics

Editorial

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Open AccessEditorial

Advances in Diagnosing and Managing Primary Systemic Vasculitides: A Transforming Landscape

by Alicia Rodriguez-Pla

Diagnostics 2026, 16(5), 788; https://doi.org/10.3390/diagnostics16050788 - 6 Mar 2026

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Abstract

As we conclude this Special Issue of Diagnostics, “Advances in the Diagnosis and Management of Vasculitis,” we reflect on a vibrant collection of eleven articles that span the globe from Spain and Poland to the USA, Germany, Romania, and Turkey [...] Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Management of Vasculitis)

Research

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14 pages, 2136 KB

Open AccessArticle

Long-Term Follow-Up in Patients with Large-Vessel Vasculitis Applying Extracranial and Transcranial Duplex Sonography

by Johanna Härtl, Sebastian Lambrecht, Felix Hess, Achim Berthele, Silke Wunderlich and Enayatullah Baki

Diagnostics 2026, 16(3), 455; https://doi.org/10.3390/diagnostics16030455 - 1 Feb 2026

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Abstract

Background: Although large-vessel vasculitis (LVV) can affect both the anterior and posterior intracranial circulation, routine neurosonographic follow-up, including transcranial duplex sonography, has not been established. We aimed to characterize patients with giant cell arteritis (GCA) and Takayasu arteritis (TAK) regarding the detection of [...] Read more.

Background: Although large-vessel vasculitis (LVV) can affect both the anterior and posterior intracranial circulation, routine neurosonographic follow-up, including transcranial duplex sonography, has not been established. We aimed to characterize patients with giant cell arteritis (GCA) and Takayasu arteritis (TAK) regarding the detection of progressive or new-onset inflammatory vessel changes by using neurosonography, and to assess the impact on medical or interventional treatment strategies. Methods: We retrospectively identified all patients with LVV treated at our neurological department between January 2015 and October 2025 with at least one neurosonographic follow-up examination. Baseline and follow-up sonographic data, clinical characteristics, medical therapy, and interventional treatments were analyzed. Results: In total, 21 LVV patients (GCA, n = 16; TAK, n = 5) underwent sonographic follow-up (GCA: median 28 (2–106) months, 4.5 (2–33) sonographic assessments; TAK: 75 (33–255) months, 14 (4–60) sonographic assessments). Isolated or combined, progressive or new-onset intra- and extracranial arterial disease was detected in seven of the 16 GCA patients (43.8%), of whom three (18.8%) presented with ischemic stroke. Medical treatment was adapted in four progressive cases. In two patients, additional interventional treatment was performed. Among TAK, two of five (40%) patients showed progressive sonographic changes, with one patient experiencing an ischemic stroke requiring endovascular treatment for progressive common carotid artery stenosis and one patient showing asymptomatic intracranial ICA involvement. Conclusions: Progressive and symptomatic involvement of intracranial carotid and vertebral arteries is a frequent finding in patients with LVV. These changes can be effectively detected through comprehensive neurosonographic follow-up, including transcranial ultrasound assessment. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Management of Vasculitis)

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13 pages, 240 KB

Open AccessArticle

Disease Course and Long-Term Outcomes in Adult IgA Vasculitis Nephritis: A Prospective Observational Study

by Fatih Yıldırım, Muhammet Emin Kutu, Yalkın Çalık, Kübra Kalkan, Gamze Akkuzu, Duygu Sevinç Özgür, Bilgin Karaalioğlu, Rabia Deniz, Gül Güzelant Özköse, Burak İnce and Cemal Bes

Diagnostics 2025, 15(8), 957; https://doi.org/10.3390/diagnostics15080957 - 10 Apr 2025

Cited by 3 | Viewed by 3693

Abstract

Background/Objectives: A limited number of previous studies have reported high rates of end-stage renal disease (ESRD) in adults with IgA vasculitis nephritis (IgAVN). Despite the high prevalence of the disease and the high rates of ESRD reported in the literature, no specific [...] Read more.

Background/Objectives: A limited number of previous studies have reported high rates of end-stage renal disease (ESRD) in adults with IgA vasculitis nephritis (IgAVN). Despite the high prevalence of the disease and the high rates of ESRD reported in the literature, no specific guidelines for adult patients have been established and there is no consensus on the management of the disease. This study aimed to prospectively investigate adults with IgAVN from a broad perspective. Methods: This investigation was designed as a prospective observational study and was conducted between 01.02.2022 and 01.10.2024. A total of 49 newly diagnosed adult (>18 years) patients with IgAVN were regularly followed up. At the end of the study, the renal remission rates, factors influencing remission, treatment data, treatment-related adverse events, and disease outcomes were determined. Results: The median follow-up time was 22 (IQR: 11–24) months. A total of 42 patients (87%) received immunosuppressive treatment in addition to the initial glucocorticoid treatment. Azathioprine (AZA) was the preferred (41%) first steroid-sparing agent. ESRD occurred in only one patient (2%), while a total of ten patients (20%) had an unfavorable outcome. The rate of nephrotic-range proteinuria (NRP) was significantly higher in the patients who did not achieve renal remission at the end of the 12-month follow-up period (9,7% vs. 60%; p = 0.02) and NRP was an independent risk factor for unfavorable outcomes [OR: 17.18; 95% CI: 1.31–224.95; p = 0.03]. A total of 16% of the patients developed an infection that required hospitalization during follow-up; these patients had a higher rate of IgAVN-associated acute kidney injury (62.5% vs. 22%; p = 0.02) and were significantly older (mean: 46 ± 15.3 vs. 65 ± 13.3; p = 0.002). One patient died of sepsis at 4 months and another died of a myocardial infarction at 32 months. Conclusions: These results suggest that adults with IgVAN do not have a high rate of ESRD if they receive effective immunosuppressive therapy. However, immunosuppressive therapy is associated with an increased risk of infection, particularly in the elderly. The presence of NRP is associated with lower long-term remission rates and has a predictive value for unfavorable outcomes. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Management of Vasculitis)

11 pages, 770 KB

Open AccessArticle

Distribution of Airway Findings in ANCA-Associated Vasculitis: A 20-Year Observational Analysis

by Megan M. Sullivan, Maximiliano Diaz Menindez, Hassan Baig, Anushka Irani, Ronald Butendieck, Benjamin Wang, Florentina Berianu, Carolyn Mead-Harvey, Andy Abril and Vikas Majithia

Diagnostics 2025, 15(1), 74; https://doi.org/10.3390/diagnostics15010074 - 31 Dec 2024

Cited by 1 | Viewed by 1575

Abstract

Objective: Pulmonary involvement is commonly observed in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), presenting with manifestations such as diffuse alveolar hemorrhage, inflammatory infiltrates, pulmonary nodules, and tracheobronchial disease. We aimed to identify distinct subgroups of tracheobronchial disease patterns in patients with anti-neutrophil cytoplasmic [...] Read more.

Objective: Pulmonary involvement is commonly observed in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), presenting with manifestations such as diffuse alveolar hemorrhage, inflammatory infiltrates, pulmonary nodules, and tracheobronchial disease. We aimed to identify distinct subgroups of tracheobronchial disease patterns in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) using latent class analysis (LCA), and to evaluate their clinical characteristics and outcomes. Methods: We conducted a retrospective cohort study using electronic medical records of patients aged >18 years diagnosed with AAV and tracheobronchial disease between 1 January 2002 and 6 September 2022. Patients with follow-up <6 months were excluded. LCA was employed to identify disease subtypes based on 10 pre-defined indicators. Maximum likelihood estimation with 10 repetitions per model ensured robustness in model selection, guided by the Akaike information criterion (AIC). Patient and disease characteristics were summarized and compared across predicted classes. Statistical analyses included Kruskal–Wallis and Fisher’s exact tests for continuous and categorical variables, respectively. The primary outcome was time to relapse of the tracheobronchial inflammation after starting immunosuppressive medication, analyzed using the Kaplan–Meier method and log-rank tests. Secondary outcomes included severity of pulmonary disease on pulmonary function tests, endoscopic interventions, tracheostomy, or mortality during follow-up. Results: Among 136 identified AAV patients assessed for tracheobronchial involvement, 111 (81.6%) were included after excluding 25 without tracheal or bronchial disease. Predominant findings included subglottic stenosis (91.0%), lower tracheal stenosis (16.2%), and bronchial stenosis (17.1%). LCA identified a three-class model as optimal: tracheal predominant (n = 94), tracheobronchial (n = 12), and bronchial predominant (n = 5). Tracheal predominant patients showed reduced risk of ear, eye, and lower respiratory manifestations, with milder obstruction on pulmonary function testing (PFT). Tracheobronchial-class patients were prone to saddle nose deformity (50%), extensive lower respiratory involvement (91.7%), and renal disease (66.7%). Bronchial predominant patients exhibited severe obstructive disease (median forced expiratory volume in 1 s (FEV1)% predicted: 58, IQR 34–66; FEV1/forced vital capacity (FVC) ratio: 56.9, interquartile range (IQR) 43–63.3) but lacked systemic AAV manifestations. LCA classes did not predict outcomes such as endoscopic intervention, tracheostomy, recurrent tracheobronchial narrowing, or mortality. Conclusion: LCA shows promise in subtype stratification of AAV patients, yet its utility in predicting outcomes and guiding treatment remains limited based on our analysis. Future studies with enhanced phenotypic data and larger cohorts are warranted to improve predictive accuracy. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Management of Vasculitis)

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18 pages, 528 KB

Open AccessArticle

Comparative Analysis of Classification Criteria in IgG4-Related Disease and Evaluating Diagnostic Accuracy from a Retrospective Cohort in Clinical Practice

by Marta Lopez-Gomez, Patricia Moya-Alvarado, Hye Sang Park, Mar Concepción Martín, Sara Calleja, Helena Codes-Mendez, Berta Magallares, Iván Castellví, Antonio J. Barros-Membrilla, Ana Laiz, César Diaz-Torné, Luis Sainz, Julia Bernárdez, Laura Martínez-Martinez and Hèctor Corominas

Diagnostics 2024, 14(22), 2583; https://doi.org/10.3390/diagnostics14222583 - 17 Nov 2024

Cited by 5 | Viewed by 6182

Abstract

Introduction: We conducted a comprehensive comparative analysis of the Okazaki, Umehara, and American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for diagnosing immunoglobulin G4-related disease (IgG4-RD). Materials and Methods: A retrospective study was conducted in a single tertiary hospital, using expert [...] Read more.

Introduction: We conducted a comprehensive comparative analysis of the Okazaki, Umehara, and American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for diagnosing immunoglobulin G4-related disease (IgG4-RD). Materials and Methods: A retrospective study was conducted in a single tertiary hospital, using expert clinical judgment as the gold standard. We compared the diagnostic accuracy of the Okazaki, Umehara, and ACR/EULAR criteria in a cohort of 41 patients with suspected IgG4-RD. We assessed sensitivity, specificity, and positive and negative predictive values for each criterion, and conducted a separate analysis based on four IgG4-RD subtypes. Results: A total of 30 patients were confirmed to have IgG4-RD and 11 were identified as mimickers. The Umehara criteria demonstrated the highest sensitivity (83.33%), followed by the ACR/EULAR 2019 (66.67%) and Okazaki (60.0%) criteria. All three criteria exhibited 100% specificity, with overall diagnostic accuracy ranging from 70% to 88%. The areas under the curve (AUC) were 0.917 (Umehara), 0.800 (Okazaki), and 0.833 (ACR/EULAR 2019), indicating significant diagnostic effectiveness (p < 0.000). Subtype analysis revealed that the Umehara and ACR/EULAR 2019 criteria were more effective in diagnosing pancreato-hepato-biliary involvement (subtype 1), while the Okazaki and ACR/EULAR 2019 criteria were more effective in diagnosing retroperitoneal fibrosis and/or aortitis (subtype 2). Conclusions: Our study provides valuable insights into the diagnostic performance of the Okazaki, Umehara, and ACR/EULAR criteria for a cohort of patients with suspected IgG4-RD. The Umehara criterion demonstrated the highest sensitivity, suggesting its potential utility for screening purposes, while all three criteria showed consistent specificity. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Management of Vasculitis)

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Review

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30 pages, 2010 KB

Open AccessReview

Diagnostic Challenges and Modern Therapeutic Strategies in Giant Cell Arteritis

by Alicia Rodriguez-Pla

Diagnostics 2026, 16(3), 470; https://doi.org/10.3390/diagnostics16030470 - 3 Feb 2026

Cited by 1 | Viewed by 1804

Abstract

Giant cell arteritis (GCA) represents one of the most diagnostically challenging systemic vasculitides, characterized by its heterogeneous clinical presentation, lack of pathognomonic features, and potential for devastating complications, with a special concern for irreversible vision loss. This comprehensive review synthesizes current evidence regarding [...] Read more.

Giant cell arteritis (GCA) represents one of the most diagnostically challenging systemic vasculitides, characterized by its heterogeneous clinical presentation, lack of pathognomonic features, and potential for devastating complications, with a special concern for irreversible vision loss. This comprehensive review synthesizes current evidence regarding the multifaceted diagnostic challenges in GCA, incorporating recent advances in classification criteria, imaging technologies, biomarker research, and emerging therapeutic strategies. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Management of Vasculitis)

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22 pages, 931 KB

Open AccessReview

Neutrophils and Platelets as Key Players in the Pathogenesis of ANCA-Associated Vasculitis and Potential Sources of Disease Activity Biomarkers

by Anna Drynda, Marcin Surmiak, Stanisława Bazan-Socha, Katarzyna Wawrzycka-Adamczyk, Mariusz Korkosz, Jacek Musiał and Krzysztof Wójcik

Diagnostics 2025, 15(15), 1905; https://doi.org/10.3390/diagnostics15151905 - 29 Jul 2025

Cited by 4 | Viewed by 4339

Abstract

Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a heterogeneous group of small-vessel vasculitides, characterized by the presence of antibodies binding to myeloperoxidase (MPO) and proteinase-3 (PR3) found in neutrophil granules. Apart from being the target of ANCA, neutrophils actively contribute to the vicious [...] Read more.

Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a heterogeneous group of small-vessel vasculitides, characterized by the presence of antibodies binding to myeloperoxidase (MPO) and proteinase-3 (PR3) found in neutrophil granules. Apart from being the target of ANCA, neutrophils actively contribute to the vicious cycle of inflammation and vascular damage in AAV. On the other hand, platelets have recently been recognized as essential for thrombosis and as inflammatory effectors that collaborate with neutrophils, reinforcing the generation of reactive oxygen species (ROS) and the formation of neutrophil extracellular traps (NETs) in those diseases. Neutrophils exhibit morphological and functional heterogeneity in AAV, reflecting the complexity of their contribution to disease pathogenesis. Since long-term immunosuppression may be related to serious infections and malignancies, there is an urgent need for reliable biomarkers of disease activity to optimize the management of AAV. This review summarizes the current understanding of the role of neutrophils and platelets in the pathogenesis of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), focusing on their crosstalk, and highlights the potential for identifying novel biomarkers relevant for predicting the disease course and its relapses. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Management of Vasculitis)

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12 pages, 286 KB

Open AccessReview

Relapse Predictors in Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis

by Katarzyna Wawrzycka-Adamczyk, Mariusz Korkosz, Jacek Musiał and Krzysztof Wójcik

Diagnostics 2024, 14(17), 1849; https://doi.org/10.3390/diagnostics14171849 - 24 Aug 2024

Cited by 6 | Viewed by 5151

Abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of rare diseases with a chronic and relapsing course. Recent treatment guidelines offer many therapeutic options depending mainly on the type of diagnosis and disease manifestations. Areas that remain under discussion include whether all [...] Read more.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of rare diseases with a chronic and relapsing course. Recent treatment guidelines offer many therapeutic options depending mainly on the type of diagnosis and disease manifestations. Areas that remain under discussion include whether all patients diagnosed with AAV belong to a homogeneous group with a similar prognosis at baseline or if the type and duration of remission-inducing treatment should depend on factors other than just diagnosis and disease severity. The aim of this review is to present the recent literature on the tools available to use while evaluating the risk of relapse in patients upon presentation as well as potential biomarkers of proceeding flare in patients upon remission. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Management of Vasculitis)

15 pages, 1013 KB

Open AccessReview

Positron Emission Tomography/Computed Tomography in Polymyalgia Rheumatica: When and for What—A Critical Review

by Elena Heras-Recuero, Teresa Blázquez-Sánchez, Laura Cristina Landaeta-Kancev, Marta Martínez de Bourio-Allona, Arantxa Torres-Roselló, Fernando Rengifo-García, Claritza Caraballo-Salazar, Raquel Largo, Santos Castañeda and Miguel Ángel González-Gay

Diagnostics 2024, 14(14), 1539; https://doi.org/10.3390/diagnostics14141539 - 17 Jul 2024

Cited by 7 | Viewed by 2412

Abstract

Polymyalgia rheumatica (PMR) is an inflammatory disease common in people aged 50 years and older. This condition is characterized by the presence of pain and stiffness involving mainly the shoulder and pelvic girdle. Besides the frequent association with giant cell arteritis (GCA), several [...] Read more.

Polymyalgia rheumatica (PMR) is an inflammatory disease common in people aged 50 years and older. This condition is characterized by the presence of pain and stiffness involving mainly the shoulder and pelvic girdle. Besides the frequent association with giant cell arteritis (GCA), several conditions may mimic PMR or present with PMR features. Since the diagnosis is basically clinical, an adequate diagnosis of this condition is usually required. Positron emission tomography/computed tomography (PET-CT) has proved to be a useful tool for the diagnosis of PMR. The use of 18F-FDG-PET imaging appears promising as it provides detailed information on inflammatory activity that may not be evident with traditional methods. However, since PET-CT is not strictly necessary for the diagnosis of PMR, clinicians should consider several situations in which this imaging technique can be used in patients with suspected PMR. Full article

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Other

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12 pages, 3492 KB

Open AccessCase Report

Hepatic Vascular Involvement in Adenosine Deaminase 2 Deficiency (DADA2): Case Reports and Literature Review

by Mihaela Sparchez, Laura Damian, Mihai Adrian Socaciu, Otilia Fufezan and Zeno Sparchez

Diagnostics 2026, 16(2), 189; https://doi.org/10.3390/diagnostics16020189 - 7 Jan 2026

Cited by 1 | Viewed by 743

Abstract

Background and Clinical Significance: Deficiency of Adenosine Deaminase 2 (DADA2) is a rare monogenic vasculopathy characterised by systemic inflammatory and immunodeficiency features. Although neurological and haematological manifestations are well-documented, hepatic vascular involvement remains underappreciated. This report aims to describe the clinical and [...] Read more.

Background and Clinical Significance: Deficiency of Adenosine Deaminase 2 (DADA2) is a rare monogenic vasculopathy characterised by systemic inflammatory and immunodeficiency features. Although neurological and haematological manifestations are well-documented, hepatic vascular involvement remains underappreciated. This report aims to describe the clinical and imaging characteristics of hepatic vascular involvement in a patient with DADA2 and to illustrate the evolution of hepatic lesions during long-term Etanercept therapy. In addition, we provide a synthesis of the available evidence on hepatic manifestations in DADA2, emphasising vascular pathology, clinical presentation, and therapeutic implications. Case Presentation: We describe a girl with early-onset DADA2 presenting with recurrent systemic inflammation, hypogammaglobulinaemia, vasculopathy, and two childhood strokes, followed by the development of multiple FNH-like hepatic nodules on CEUS and MRI with persistently elevated GGT. Genetic testing confirmed biallelic ADA2 mutations, and treatment with Etanercept led to sustained clinical stabilisation and marked regression of liver lesions over a nine-year follow-up period. Her older sister, carrying the same mutations, showed a milder phenotype without hepatic involvement but experienced a mesenteric vascular event. Conclusions: Large regenerative nodules with an FNH-like appearance on CEUS or MRI have not been previously reported in this setting. In our patient, Etanercept therapy produced a favourable hepatic response, reflected by a significant reduction in both the number and size of the lesions. Our case contributes to the understanding of liver disease in DADA2 and the influence of imaging and treatment on the hepatic manifestations of the condition. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Management of Vasculitis)

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5 pages, 1155 KB

Open AccessInteresting Images

Giant Cell Arteritis: Can Simple Ultrasound Examination Prevent Complex Consequences?

by Wiktoria Stańska, Robert Kruszewski, Aleksandra Juszkiewicz, Artur Bachta and Witold Tłustochowicz

Diagnostics 2024, 14(18), 2071; https://doi.org/10.3390/diagnostics14182071 - 19 Sep 2024

Cited by 2 | Viewed by 2283

Abstract

Giant cell arteritis (GCA) is a rare disease of the arteries, occurring mainly in the elderly. Although the involvement of temporal arteries can be mostly symptomatic, the occlusion of ophthalmic arteries has severe consequences. GCA affecting temporal arteries is an emergency requiring quick [...] Read more.

Giant cell arteritis (GCA) is a rare disease of the arteries, occurring mainly in the elderly. Although the involvement of temporal arteries can be mostly symptomatic, the occlusion of ophthalmic arteries has severe consequences. GCA affecting temporal arteries is an emergency requiring quick commencement of treatment with glucocorticoids due to the serious consequences of neglect—blindness. According to the new guidelines released by EULAR, ultrasound is the tool of choice in support of the clinical diagnosis of giant cell arteritis, replacing temporal artery biopsy (TAB), as it is a sensitive and non-invasive method that is widely available. The main limitation is that the reliability of this imaging is based on the technical expertise of ultrasonographers. However, performing imaging should not delay commencing the treatment. In this work, we present ultrasound images from a case report of a 74-year-old female patient where difficulties in establishing a diagnosis led to vision loss in both eyes. In this example, we describe the ultrasound findings in giant cell arteritis, emphasizing its usefulness in supporting a diagnosis of GCA. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Management of Vasculitis)

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7 pages, 4416 KB

Open AccessInteresting Images

Central Retinal Artery Occlusion Associated with Takayasu Arteritis

by Sehreen Mumtaz, Claire Wilson, Prasanna Vibhute, Eric R. Eggenberger, Florentina Berianu and Andy Abril

Diagnostics 2024, 14(13), 1329; https://doi.org/10.3390/diagnostics14131329 - 23 Jun 2024

Cited by 1 | Viewed by 2880

Abstract

Takayasu arteritis is a chronic inflammatory vasculitis with granulomatous panarteritis particularly impacting large vessels including the aorta and its branches, especially the subclavian arteries, with clinical manifestation dependent on the involved artery. Sequelae of the active disease vary, including stenosis, occlusions, or aneurysmal [...] Read more.

Takayasu arteritis is a chronic inflammatory vasculitis with granulomatous panarteritis particularly impacting large vessels including the aorta and its branches, especially the subclavian arteries, with clinical manifestation dependent on the involved artery. Sequelae of the active disease vary, including stenosis, occlusions, or aneurysmal dilatations of the large vessels. The prevalence of Takayasu arteritis is higher in the Asian population and in Japan, but quite low in the United States, varying from 0.9–8.4 per million people. Ocular manifestations are rare and lead to a delay in diagnosis and appropriate treatment. Ocular manifestations include Takayasu retinopathy, anterior ischemic optic neuropathy (AION), retinal artery occlusion (RAO) and retinal vein occlusion (RVO). We present two cases in which central retinal artery occlusion (CRAO) was associated with Takayasu arteritis. CRAO is an ophthalmic emergency with an incidence of 1.9 per 100,000 person years in the United States; only 5% of cases are arteritic, which can be observed with inflammatory vasculitides secondary to the formation of immune deposits. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Management of Vasculitis)

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