Advances in the Diagnosis and Management of Vasculitis

A special issue of Diagnostics (ISSN 2075-4418). This special issue belongs to the section "Pathology and Molecular Diagnostics".

Deadline for manuscript submissions: 30 April 2025 | Viewed by 4067

Special Issue Editor


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Guest Editor
Sierra Pacific Arthritis and Rheumatology Centers, Visalia, CA, USA
Interests: vasculitis; scleroderma; IgG4-related disease

Special Issue Information

Dear colleagues, 

Research in the field of primary systemic vasculitides is an evolving topic. Advances in their diagnosis and management are quickly improving patient outcomes. In recent years, less invasive imaging studies have become available to assist with the diagnosis and treatment guidance of vasculitis, especially for those with large-vessel involvement. Advances in the understanding of immunopathogenesis, the search for biomarkers, the increased recognition of steroids’ toxicity, and the increased interest in clinical trials have advanced the use of targeted therapies that have revolutionized the management of vasculitides. To keep up with these advances, this Special Issue is dedicated to updates on the diagnosis and management of primary systemic vasculitides. Proposals in agreement with this broad topic are welcome. In this Special Issue, original research articles and reviews are welcome. Research areas may include (but are not limited to) the following: imaging studies on vasculitis, biomarkers, treatment approaches, patient outcomes, and epidemiologic studies.

We look forward to receiving your contributions.

Dr. Alicia Rodríguez-Pla
Guest Editor

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Keywords

  • vasculitis
  • advances
  • updates
  • management
  • treatment
  • therapy

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Published Papers (5 papers)

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Research

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18 pages, 528 KiB  
Article
Comparative Analysis of Classification Criteria in IgG4-Related Disease and Evaluating Diagnostic Accuracy from 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
Viewed by 451
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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12 pages, 286 KiB  
Review
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
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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 KiB  
Review
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
Viewed by 676
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
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Vasculitis)
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Other

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5 pages, 1155 KiB  
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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
Viewed by 630
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 KiB  
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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
Viewed by 1023
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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