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Vasculitis in Adults and Children: Symptoms, Diagnosis and Treatment
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Vasculitis in Adults and Children: Symptoms, Diagnosis and Treatment

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Vascular Medicine".

Deadline for manuscript submissions: closed (15 February 2024) | Viewed by 9015

Special Issue Editor

Dr. Ryu Watanabe

E-Mail Website
Guest Editor
Department of Clinical Immunology, Osaka Metropolitan University Graduate School of Medicine, 1-4-3 Asahi-machi, Abeno-ku, Osaka 545-8585, Japan
Interests: vasculitis; rheumatoid arthritis; giant cell arteritis; Takayasu arteritis; ANCA-associated vasculitis
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Vasculitis is classified into large-vessel, medium-vessel, and small-vessel vasculitis according to the size of the affected arteries, and causes serious organ damage through ischemia, infarction, bleeding, dissection, and aneurysm formation. Treatment previously relied on high-dose glucocorticoids and cyclophosphamide, regardless of the size of the affected arteries, with substantial side effects; however, in recent years molecularly targeted therapeutic agents, such as tocilizumab, rituximab, mepolizumab, and avacopan, have enabled remission to be effectively induced and/or maintained, and have also reduced the total amount of glucocorticoids required for the corresponding vasculitis. This Special Issue, “Vasculitis in Adults and Children: Symptoms, Diagnosis and Treatment”, summarizes recent topics on the symptoms, diagnosis, and treatment of vasculitis in both adults and children. Clinical research regarding epidemiology, gender difference, prognostic factors, complications, classification criteria, and treatment, but also basic/translational research which tries to identify novel therapeutic targets, are welcomed. In this way, we should be able to better understand how to diagnose, and how to treat, vasculitis. We also discuss how future therapeutic strategies resolve the current unmet medical needs. Thus, authors are encouraged to submit original research, review articles, systematic reviews, and meta-analyses.

Dr. Ryu Watanabe
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. Journal of Clinical Medicine 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
  • glucocorticoids
  • cyclophosphamide
  • molecularly targeted therapy
  • unmet clinical needs
  • potential therapeutic targets

Related Special Issue

Published Papers (6 papers)

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Review

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15 pages, 295 KiB  
Review
New Biomarkers for Systemic Necrotizing Vasculitides
by Russka Shumnalieva, Plamena Ermencheva, Georgi Kotov, Iva Parvova-Hristova, Konstantina Bakopoulou, Issa El Kaouri, Niya Mileva and Tsvetelina Velikova
J. Clin. Med. 2024, 13(8), 2264; https://doi.org/10.3390/jcm13082264 - 13 Apr 2024
Viewed by 423
Abstract
Systemic necrotising vasculitides (SNVs) pose significant challenges due to their diverse clinical manifestations and variable outcomes. Therefore, identifying reliable biomarkers holds promise for improving precision medicine in SNVs. This review explores emerging biomarkers aiming to enhance diagnostic accuracy, prognostic assessment, and disease monitoring. [...] Read more.
Systemic necrotising vasculitides (SNVs) pose significant challenges due to their diverse clinical manifestations and variable outcomes. Therefore, identifying reliable biomarkers holds promise for improving precision medicine in SNVs. This review explores emerging biomarkers aiming to enhance diagnostic accuracy, prognostic assessment, and disease monitoring. We discuss recent advances in immunological biomarkers, inflammatory indicators, and other parameters that exhibit potential diagnostic and prognostic utility. A comprehensive understanding of these biomarkers may facilitate earlier and more accurate SNV detection, aiding in timely intervention and personalized treatment strategies. Furthermore, we highlight the evolving landscape of disease monitoring through innovative biomarkers, shedding light on their dynamic roles in reflecting disease activity and treatment response. Integrating these novel biomarkers into clinical practice can revolutionize the management of SNVs, ultimately improving patient outcomes and quality of life. Full article
(This article belongs to the Special Issue Vasculitis in Adults and Children: Symptoms, Diagnosis and Treatment)
9 pages, 644 KiB  
Review
Different Types of Vasculitis Complicated by Heparin-Induced Thrombocytopenia—Analysis of Four Cases and Literature Review
by Adam Rytel, Mateusz Nowak, Monika Kukawska-Rytel, Katarzyna Morawiec and Stanisław Niemczyk
J. Clin. Med. 2023, 12(19), 6176; https://doi.org/10.3390/jcm12196176 - 24 Sep 2023
Cited by 1 | Viewed by 996
Abstract
Vasculitis and HIT have different etiologies, although both involve autoimmune mechanisms. Treatment of vasculitis often requires the use of an anticoagulant such as heparin, which can lead to the development of HIT and subsequent life-threatening complications. The analysis covered patients hospitalized in the [...] Read more.
Vasculitis and HIT have different etiologies, although both involve autoimmune mechanisms. Treatment of vasculitis often requires the use of an anticoagulant such as heparin, which can lead to the development of HIT and subsequent life-threatening complications. The analysis covered patients hospitalized in the Department of Internal Medicine, Nephrology and Dialysis in the period from September 2020 to March 2023. After analyzing the data, we selected four patients in whom vasculitis treatment was complicated by HIT. These included two patients with ANCA vasculitis and two patients with anti-GBM disease. We also described similar cases reported in the literature. Full article
(This article belongs to the Special Issue Vasculitis in Adults and Children: Symptoms, Diagnosis and Treatment)
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17 pages, 1722 KiB  
Review
Eosinophilic Granulomatosis with Polyangiitis: Latest Findings and Updated Treatment Recommendations
by Ryu Watanabe and Motomu Hashimoto
J. Clin. Med. 2023, 12(18), 5996; https://doi.org/10.3390/jcm12185996 - 15 Sep 2023
Cited by 3 | Viewed by 3260
Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA) causes necrotizing vasculitis and eosinophil-rich granulomatous inflammation in small- to medium-sized vessels, resulting in multiple organ damage. EGPA is classified as an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, with myeloperoxidase-ANCA detected in approximately one-third of the patients. Conventional treatment [...] Read more.
Eosinophilic granulomatosis with polyangiitis (EGPA) causes necrotizing vasculitis and eosinophil-rich granulomatous inflammation in small- to medium-sized vessels, resulting in multiple organ damage. EGPA is classified as an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, with myeloperoxidase-ANCA detected in approximately one-third of the patients. Conventional treatment of EGPA relies on systemic glucocorticoids (GCs) in combination with cyclophosphamide when poor prognostic factors are present; however, the dilemma between disease control and drug-related adverse effects has long been a challenge. Recent studies have revealed that the genetic background, pathophysiology, and clinical manifestations differ between ANCA-positive and ANCA-negative patients; however, mepolizumab, an interleukin (IL)-5 inhibitor, is effective in both groups, suggesting that the IL-5-eosinophil axis is deeply involved in the pathogenesis of both ANCA-positive and ANCA-negative EGPA. This review summarizes the latest knowledge on the pathophysiology of EGPA and focuses on the roles of eosinophils and ANCA. We then introduce the current treatment recommendations and accumulated evidence for mepolizumab on EGPA. Based on current unmet clinical needs, we discuss potential future therapeutic strategies for EGPA. Full article
(This article belongs to the Special Issue Vasculitis in Adults and Children: Symptoms, Diagnosis and Treatment)
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15 pages, 6559 KiB  
Review
Refractory Takayasu’s Arteritis with Severe Coronary Involvement—Case Report and Literature Review
by Claudia Oana Cobilinschi, Elena Grădinaru, Ioana Săulescu, Nicolae Cârstea, Simona Caraiola, Andra Rodica Bălănescu and Daniela Opriș-Belinski
J. Clin. Med. 2023, 12(13), 4394; https://doi.org/10.3390/jcm12134394 - 29 Jun 2023
Cited by 2 | Viewed by 1271
Abstract
This report presents the case of a female patient diagnosed with Takayasu arteritis from childhood, with severe, refractory coronary involvement, leading to two acute coronary syndromes and multiple anginous episodes. Consequently, the patient suffered aorto-bicarotid bypass two times, multiple interventional procedures with stent [...] Read more.
This report presents the case of a female patient diagnosed with Takayasu arteritis from childhood, with severe, refractory coronary involvement, leading to two acute coronary syndromes and multiple anginous episodes. Consequently, the patient suffered aorto-bicarotid bypass two times, multiple interventional procedures with stent implantation, balloon angioplasty, and up to ten repeated in-stent restenosis that required reinterventions, despite being on maximal immunosuppressive treatment. In recent years, various studies have been reported that aim to best characterize this particular type of vascular damage and to indicate optimal therapeutic options for treatment. The latter should be based on the activity of the underlying disease; however, no reliable markers are available in TA. The management of TA patients with coronary involvement continues to be a challenge and requires both drug and interventional techniques to avoid life-threatening events. Full article
(This article belongs to the Special Issue Vasculitis in Adults and Children: Symptoms, Diagnosis and Treatment)
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Other

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10 pages, 3531 KiB  
Case Report
Not Everything Is as It Seems: A Case Series and Overview of Diseases Mimicking Antineutrophil Cytoplasmic Antibody-Associated Vasculitis
by Eline Houben, Pieter F. de Groot, Yosta Vegting, Josephine M. I. Vos, Erfan Nur, Marc L. Hilhorst, A. E. (Liesbeth) Hak and Arjan J. Kwakernaak
J. Clin. Med. 2023, 12(19), 6144; https://doi.org/10.3390/jcm12196144 - 23 Sep 2023
Viewed by 1017
Abstract
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare heterogeneous disease in which treatment must be initiated early to prevent irreversible organ damage and death. There are several diseases that can mimic AAV, even in the presence of positive ANCA serology and/or histological [...] Read more.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare heterogeneous disease in which treatment must be initiated early to prevent irreversible organ damage and death. There are several diseases that can mimic AAV, even in the presence of positive ANCA serology and/or histological evidence of vasculitis, as demonstrated in this case series. We reflect on the diagnostic approach of patients with AAV and provide an overview of AAV-mimicking diseases that can be considered in patients with atypical disease presentation or course. Full article
(This article belongs to the Special Issue Vasculitis in Adults and Children: Symptoms, Diagnosis and Treatment)
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19 pages, 659 KiB  
Systematic Review
Systemic and Local Medical or Surgical Therapies for Ear, Nose and/or Throat Manifestations in ANCA-Associated Vasculitis: A Systematic Literature Review
by Roline M. Krol, Hilde H. F. Remmelts, Ruth Klaasen, Annelies Frima, E. Christiaan Hagen, Digna M. A. Kamalski, Marloes W. Heijstek and Julia Spierings
J. Clin. Med. 2023, 12(9), 3173; https://doi.org/10.3390/jcm12093173 - 28 Apr 2023
Cited by 2 | Viewed by 1610
Abstract
Background: Ear, nose and throat (ENT) manifestations are common in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), yet how to treat these manifestations remains controversial. Therefore, we systematically reviewed the literature on the efficacy of therapies on ENT manifestations in AAV. Methods: [...] Read more.
Background: Ear, nose and throat (ENT) manifestations are common in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), yet how to treat these manifestations remains controversial. Therefore, we systematically reviewed the literature on the efficacy of therapies on ENT manifestations in AAV. Methods: A systematic review was conducted in accordance with the PRISMA guidelines, searching Medline, Embase and Cochrane libraries, including clinical studies between January 2005 and January 2022, in adults with AAV and ENT involvement, reporting on the effects of local and systemic therapy. The critical appraisal was performed using tools provided by the Cochrane Library and the level of evidence (LoE) was scored according to the Oxford Centre for Evidence-based Medicine. Results: After screening 5609 identified studies, 136 full-text articles were assessed. Finally, 31 articles were included for critical appraisal and data-extraction. Nearly all studies (n = 29) were retrospective and scored low on LoE. The included studies evaluated local interventions (n = 11), glucocorticoids combined with conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs) (n = 8), rituximab (n = 6), or mepolizumab (n = 6). Due to heterogeneity across studies meta-analysis was not performed. Four studies on mepolizumab for sinonasal symptoms (n = 92) showed response in 33–100% and relapse in 35%. Local therapy for subglottic stenosis was effective in 80–100% of patients in 11 studies (n = 157), but relapses were common (up to 83%). In five studies, hearing improvement was observed in 56–100%, with better outcomes when glucocorticoids were combined with csDMARDs compared to glucocorticoids only. Conclusion: Response rates of ENT manifestations varied widely in studies and relapses were observed frequently. Heterogeneity among studies impaired comparison. Full article
(This article belongs to the Special Issue Vasculitis in Adults and Children: Symptoms, Diagnosis and Treatment)
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