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Molecular Mechanisms in Vasculitis
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Molecular Mechanisms in Vasculitis

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: closed (31 May 2023) | Viewed by 10256

Special Issue Editors

Dr. Kim M. O'Sullivan

E-Mail Website
Guest Editor
Centre for Inflammatory Diseases, Department of Medicine, Monash University, Clayton, VIC 3168, Australia
Interests: TLRs; NETs; autoimmune kidney diseases; ANCA vasculitis; CD4 T cells; microbiome in autoimmune kidney disease
Special Issues, Collections and Topics in MDPI journals
Dr. Poh Yi Gan

E-Mail Website
Guest Editor
Centre for Inflammatory Diseases, Department of Medicine, Monash University, Clayton, VIC 3168, Australia
Interests: immunomodulation; ANCA vasculitis; T regulatory cells; mast cells; glomerulonephritis; acute kidney injury; nanoparticle
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Vasculitis is a rare heterogenous disorder which can target any organ and is characterised by inflammation of both small and large blood vessels. There are many types of vasculitis affecting different groups of people: Kawasaki disease and Henoch Schönlein (HSP) is more common in children; giant cell arteritis (GCA) affects mainly adults over the age of 50 and affects the temporal arteries in the head; anti-neutrophil cytoplasmic antibody vasculitis (AAV) is an autoimmune disease that mainly affects older adults and can cause vasculitis rashes, renal failure, and inflammation of the lungs; newly described VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a serious autoinflammatory disorder which only affects men (or women with monosomy of the X chromosome) and is characterised by mutant myeloid cells, skin rashes, pulmonary inflammation and chondritis; Behçet’s disease is an autoimmune disease characterised by inflammation of the blood vessels (arteries and veins of all sizes) throughout the body and affects mainly people in the 20s and 30s; Takayasu’s arteritis affects the aorta and is predominantly in woman less than 50; and Sjögren’s is an autoimmune disease that affects the entire body, which is predominantly found in women of any age. What is common with all these vasculitis disorders is that many of them are of unknown aetiology and lack effective therapy. Current therapy is nonspecific with multiple undesirable side effects. Understanding the molecular mechanisms which underlie the pathogenesis of the disease is critical for identifying new therapeutic targets and improving outcomes for patients living with vasculitis.

This Special Issue of the International Journal of Molecular Sciences welcomes both original and review articles on the molecular mechanisms that underlie the pathogenesis in vasculitis, aetiology, identification of biomarkers, and novel therapeutic targets for treatment.

Dr. Kim M. O'Sullivan
Dr. Poh Yi Gan
Guest Editors

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. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. There is an Article Processing Charge (APC) for publication in this open access journal. For details about the APC please see here. 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
  • ANCA vasculitis
  • VEXAS
  • Behçet’s
  • Kawasaki disease
  • Takayasu’s arteritis
  • Sjögren’s
  • pathogenesis
  • aetiology

Published Papers (4 papers)

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Research

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17 pages, 2880 KiB  
Article
Toll-like Receptor 9 Induced Dendritic Cell Activation Promotes Anti-Myeloperoxidase Autoimmunity and Glomerulonephritis
by Sharon L. Ford, Kim M. O’Sullivan, A. Richard Kitching, Stephen R. Holdsworth, Poh Yi Gan and Shaun A. Summers
Int. J. Mol. Sci. 2023, 24(2), 1339; https://doi.org/10.3390/ijms24021339 - 10 Jan 2023
Cited by 2 | Viewed by 2002
Abstract
ANCA-associated vasculitis (AAV) is intricately linked with infections. Toll-like receptors (TLR) provide a potential link between infection and anti-myeloperoxidase (MPO) autoimmunity. TLR9 ligation has been shown to promote anti-MPO autoimmunity and glomerular vasculitis in murine MPO-AAV. This study investigates dendritic cell TLR9 ligation [...] Read more.
ANCA-associated vasculitis (AAV) is intricately linked with infections. Toll-like receptors (TLR) provide a potential link between infection and anti-myeloperoxidase (MPO) autoimmunity. TLR9 ligation has been shown to promote anti-MPO autoimmunity and glomerular vasculitis in murine MPO-AAV. This study investigates dendritic cell TLR9 ligation in murine experimental anti-MPO glomerulonephritis. We analyzed autoimmune responses to MPO following transfer of TLR9 stimulated, MPO pulsed dendritic cells and kidney injury following a sub-nephritogenic dose of sheep anti-mouse glomerular basement membrane globulin. TLR9 ligation enhanced dendritic cell activation upregulating CD40 and CD80 expression, promoting systemic anti-MPO autoimmunity and T cell recall responses and exacerbating kidney injury. CD40 upregulation by TLR9 was critical for the induction of nephritogenic autoimmunity. The presence of DEC205, which transports the TLR9 ligand to TLR9 located in the endosome, also promoted kidney injury. This confirms TLR9 mediated dendritic cell activation as a mechanism of anti-MPO autoimmunity in AAV and further defines the link between infection and the generation of MPO specific autoimmune inflammation. Full article
(This article belongs to the Special Issue Molecular Mechanisms in Vasculitis)
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13 pages, 849 KiB  
Article
Circulating Endothelial Cells: A New Possible Marker of Endothelial Damage in Kawasaki Disease, Multisystem Inflammatory Syndrome in Children and Acute SARS-CoV-2 Infection
by Marianna Fabi, Biljana Petrovic, Laura Andreozzi, Elena Corinaldesi, Emanuele Filice, Carlotta Biagi, Alessia Rizzello, Bianca Elisa Mattesini, Simone Bugani and Marcello Lanari
Int. J. Mol. Sci. 2022, 23(17), 10106; https://doi.org/10.3390/ijms231710106 - 3 Sep 2022
Cited by 10 | Viewed by 1899
Abstract
Background: Kawasaki Disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) are pediatric diseases characterized by systemic inflammation and vascular injury, potentially leading to coronary artery lesions (CALs). Data on vascular injury occurring during acute COVID-19 (AC19) in children are still lacking. The [...] Read more.
Background: Kawasaki Disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) are pediatric diseases characterized by systemic inflammation and vascular injury, potentially leading to coronary artery lesions (CALs). Data on vascular injury occurring during acute COVID-19 (AC19) in children are still lacking. The aim of our study was to investigate endothelial injury in KD-, MIS-C- and AC19-dosing circulating endothelial cells (CECs). Methods: We conducted a multicenter prospective study. CECs were enumerated by CellSearch technology through the immunomagnetic capture of CD146-positive cells from whole blood. Results: We enrolled 9 KD, 20 MIS-C and 10 AC19. During the acute stage, the AC19 and KD patients had higher CECs levels than the MIS-C patients. From the acute to subacute phase, a significant CEC increase was observed in the KD patients, while a mild decrease was detected in the MIS-C patients. Cellular clusters/syncytia were more common in the KD patients. No correlation between CECs and CALs were found in the MIS-C patients. The incidence of CALs in the KD group was too low to investigate this correlation. Conclusions: Our study suggests a possible role of CECs as biomarkers of systemic inflammation and endothelial dysfunction in KD and MIS-C and different mechanisms of vascular injury in these diseases. Further larger studies are needed. Full article
(This article belongs to the Special Issue Molecular Mechanisms in Vasculitis)
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17 pages, 4311 KiB  
Article
Effect of Mortalin on Scar Formation in Human Dermal Fibroblasts and a Rat Incisional Scar Model
by Bok Ki Jung, Tai Suk Roh, Hyun Roh, Ju Hee Lee, Chae-Ok Yun and Won Jai Lee
Int. J. Mol. Sci. 2022, 23(14), 7918; https://doi.org/10.3390/ijms23147918 - 18 Jul 2022
Cited by 3 | Viewed by 2734
Abstract
Wound healing is a complicated cascading process; disequilibrium among reparative processes leads to the formation of pathologic scars. Herein, we explored the role of mortalin in scar formation and its association with the interleukin-1α receptor using in vitro and in vivo models. To [...] Read more.
Wound healing is a complicated cascading process; disequilibrium among reparative processes leads to the formation of pathologic scars. Herein, we explored the role of mortalin in scar formation and its association with the interleukin-1α receptor using in vitro and in vivo models. To investigate the effects of mortalin, we performed an MTT cell viability assay, qRT-PCR, and Western blot analyses, in addition to immunofluorescence and immunoprecipitation studies using cultured fibroblasts. A rat incisional wound model was used to evaluate the effect of a mortalin-specific shRNA (dE1-RGD/GFP/shMot) Ad vector in scar tissue. In vitro, the mortalin-treated human dermal fibroblast displayed a significant increase in proliferation of type I collagen, α-smooth muscle actin, transforming growth factor-β, phospho-Smad2/3-complex, and NF-κB levels. Immunofluorescence staining revealed markedly increased mortalin and interleukin-1α receptor protein in keloid tissue compared to those in normal tissue, suggesting that the association between mortalin and IL-1α receptor was responsible for the fibrogenic effect. In vivo, mortalin-specific shRNA-expressing Ad vectors significantly decreased the scar size and type-I-collagen, α-SMA, and phospho-Smad2/3-complex expression in rat incisional scar tissue. Thus, dE1-RGD/GEP/shMot can inhibit the TGF-β/α-SMA axis and NF-κB signal pathways in scar formation, and blocking endogenous mortalin could be a potential therapeutic target for keloids. Full article
(This article belongs to the Special Issue Molecular Mechanisms in Vasculitis)
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Review

Jump to: Research

20 pages, 1075 KiB  
Review
The Future of Targeted Treatment of Primary Sjögren’s Syndrome: A Focus on Extra-Glandular Pathology
by Weizhen Zeng, Xinyao Zhou, Sulan Yu, Ruihua Liu, Chrystie Wan Ning Quek, Haozhe Yu, Ryan Yong Kiat Tay, Xiang Lin and Yun Feng
Int. J. Mol. Sci. 2022, 23(22), 14135; https://doi.org/10.3390/ijms232214135 - 16 Nov 2022
Cited by 4 | Viewed by 3006
Abstract
Primary Sjögren’s syndrome (pSS) is a chronic, systemic autoimmune disease defined by exocrine gland hypofunction resulting in dry eyes and dry mouth. Despite increasing interest in biological therapies for pSS, achieving FDA-approval has been challenging due to numerous complications in the trials. The [...] Read more.
Primary Sjögren’s syndrome (pSS) is a chronic, systemic autoimmune disease defined by exocrine gland hypofunction resulting in dry eyes and dry mouth. Despite increasing interest in biological therapies for pSS, achieving FDA-approval has been challenging due to numerous complications in the trials. The current literature lacks insight into a molecular-target-based approach to the development of biological therapies. This review focuses on novel research in newly defined drug targets and the latest clinical trials for pSS treatment. A literature search was conducted on ClinicalTrials.gov using the search term “Primary Sjögren’s syndrome”. Articles published in English between 2000 and 2021 were included. Our findings revealed potential targets for pSS treatment in clinical trials and the most recent advances in understanding the molecular mechanisms underlying the pathogenesis of pSS. A prominent gap in current trials is in overlooking the treatment of extraglandular symptoms such as fatigue, depression, and anxiety, which are present in most patients with pSS. Based on dryness and these symptom-directed therapies, emerging biological agents targeting inflammatory cytokines, signal pathways, and immune reaction have been studied and their efficacy and safety have been proven. Novel therapies may complement existing non-pharmacological methods of alleviating symptoms of pSS. Better grading systems that add extraglandular symptoms to gauge disease activity and severity should be created. The future of pSS therapies may lie in gene, stem-cell, and tissue-engineering therapies. Full article
(This article belongs to the Special Issue Molecular Mechanisms in Vasculitis)
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