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Advances in the Diagnosis and Management of Vasculitis
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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 7978

Special Issue Editor

Dr. Alicia Rodríguez-Pla

E-Mail Website
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

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Diagnostics is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

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

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Published Papers (7 papers)

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Research

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13 pages, 240 KiB  
Article
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
Viewed by 262
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 KiB  
Article
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
Viewed by 676
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 KiB  
Article
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 1 | Viewed by 1149
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
Viewed by 1332
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
Cited by 3 | Viewed by 1096
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  
Interesting 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
Viewed by 1049
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  
Interesting 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
Viewed by 1478
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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