Search for Articles:
Title / Keyword
Author / Affiliation / Email
Journal
Article Type
 
 
Advanced Search
 
Section
Special Issue
Volume
Issue
Number
Page
 
5.4
3.9
Journals
JCM
Special Issues
Small-Vessel Vasculitis
share announcement

Small-Vessel Vasculitis

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 (1 April 2021) | Viewed by 7055

Special Issue Editor

Dr. Marc Hilhorst

E-Mail
Guest Editor
Department of Nephrology, Amsterdam UMC, Meibergdreef 9, 1105AZ Amsterdam, The Netherlands
Interests: immunologically mediated renal disease; autoimmunity; ANCA

Special Issue Information

Dear Colleagues,

The etiology of small-vessel vasculitis remains enigmatic. The inflammation of small vessels can be seen in a range of diseases, most notably, of the autoimmune spectrum. Some are characterized by immune complex deposition such as systemic lupus erythematosus and some are the result of antibody-mediated autoimmunity such as anti-GBM disease and ANCA vasculitis. In the last thirty years, the pathophysiology of many vasculitides has become more comprehensible and treatment has steadily improved. Nevertheless, their etiology remains unknown and the risk of relapse with severe consequences remains.

The Special Issue entitled “Small-Vessel Vasculitis” of the Journal of Clinical Medicine is now open for submissions. This issue welcomes all types of papers on the clinical characteristics, prognosis, pathophysiology, and treatment of small-vessel vasculitides, including but not limited to IgA vasculitis, cryoglobulinemic vasculitis, anti-GBM disease, and ANCA-associated vasculitides. The goal of this Special Issue is to further enhance awareness of small-vessel vasculitis and boost the knowledge on its pathophysiology in order to generate new therapeutic options. An important topic we wish to address is the clinical and pathophysiological differences between subtypes of ANCA vasculitis, specifically between PR3-ANCA and MPO-ANCA.

Dr. Marc Hilhorst
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

  • anti-neutrophil cytoplasmic antibodies (ANCA)
  • vasculitis
  • glomerulonephritis
  • immune complexes
  • therapy
  • prognosis

Published Papers (3 papers)

Download All Papers
Order results
Result details
Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:

Research

13 pages, 1778 KiB  
Article
Proteinuria Indicates Decreased Normal Glomeruli in ANCA-Associated Glomerulonephritis Independent of Systemic Disease Activity
by Désirée Tampe, Peter Korsten, Philipp Ströbel, Samy Hakroush and Björn Tampe
J. Clin. Med. 2021, 10(7), 1538; https://doi.org/10.3390/jcm10071538 - 6 Apr 2021
Cited by 12 | Viewed by 2048
Abstract
Background: Renal involvement is a common and severe complication of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), potentially resulting in a pauci-immune necrotizing and crescentic ANCA glomerulonephritis (GN) with acute kidney injury (AKI), end-stage renal disease (ESRD) or death. There is recent evidence that [...] Read more.
Background: Renal involvement is a common and severe complication of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), potentially resulting in a pauci-immune necrotizing and crescentic ANCA glomerulonephritis (GN) with acute kidney injury (AKI), end-stage renal disease (ESRD) or death. There is recent evidence that the degree of proteinuria at diagnosis is associated with long-term renal outcome in ANCA GN. Therefore, we here aimed to systematically describe the association between proteinuria and clinicopathological characteristics in 53 renal biopsies with ANCA GN and corresponding urinary samples at admission. Methods: A total number of 53 urinary samples at admission and corresponding renal biopsies with confirmed renal involvement of AAV were retrospectively included from 2015 to 2021 in a single-center study. Results: Proteinuria correlated with myeloperoxidase (MPO) subtype, diagnosis of microscopic polyangiitis (MPA) and severe deterioration of kidney function. Proteinuria was most prominent in sclerotic class ANCA GN and ANCA renal risk score (ARRS) high risk attributed to nonselective proteinuria, including both glomerular and tubular proteinuria. Finally, there was no association between proteinuria and systemic disease activity, suggesting that proteinuria reflected specific renal involvement in AAV rather that systemic disease activity. Conclusions: In conclusion, proteinuria correlated with distinct clinicopathological characteristics in ANCA GN, mostly attributed to a reduced fraction of normal glomeruli. Furthermore, proteinuria in ANCA GN reflected specific renal involvement in AAV rather than systemic disease activity. Therefore, urinary findings could further improve our understanding of mechanisms promoting kidney injury and progression of ANCA GN. Full article
(This article belongs to the Special Issue Small-Vessel Vasculitis)
►▼ Show Figures

Figure 1

10 pages, 2260 KiB  
Article
Systematic Histological Scoring Reveals More Prominent Interstitial Inflammation in Myeloperoxidase-ANCA Compared to Proteinase 3-ANCA Glomerulonephritis
by Samy Hakroush, Ingmar Alexander Kluge, Philipp Ströbel, Peter Korsten, Désirée Tampe and Björn Tampe
J. Clin. Med. 2021, 10(6), 1231; https://doi.org/10.3390/jcm10061231 - 16 Mar 2021
Cited by 19 | Viewed by 2363
Abstract
Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic vasculitis, most frequently presenting as microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA). Kidney involvement is a common and severe complication of ANCA AAV which is observed in a considerable subset of patients, [...] Read more.
Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic vasculitis, most frequently presenting as microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA). Kidney involvement is a common and severe complication of ANCA AAV which is observed in a considerable subset of patients, mainly affecting glomeruli. However, tubulointerstitial lesions have also been described in ANCA glomerulonephritis (GN). Therefore, we aim to describe active and chronic tubulointerstitial lesions in ANCA GN subtypes by systematic scoring analogous to the Banff scoring system while also utilizing clinical and laboratory findings. Methods: A total of 49 kidney biopsies with ANCA GN were retrospectively included in a single-center cohort study between 2015–2020. Results: We report that MPO-ANCA GN is associated with more severe deterioration of kidney function independent of systemic markers of AAV disease activity, and is also associated with increased proteinuria in MPO-ANCA GN and a decreased fraction of normal glomeruli. Finally, MPO-ANCA GN showed distinct, active, and chronic tubulointerstitial lesions. Conclusion: New insights into the pathophysiology of both entities, as well as differences in the clinical presentation of MPO- versus PR3-ANCA GN, could potentially pave the way for more precise treatment regimens. Therefore, it is important to understand the differences in histopathological presentation, especially in yet underestimated active tubulointerstitial lesions of ANCA GN subtypes. This research could further improve our understanding of distinct pathophysiological mechanisms. Full article
(This article belongs to the Special Issue Small-Vessel Vasculitis)
►▼ Show Figures

Figure 1

11 pages, 753 KiB  
Article
Incidence and Risk Factors of Venous Thromboembolic Events in Patients with ANCA-Glomerulonephritis: A Cohort Study from the Maine-Anjou Registry
by Nicolas Henry, Benoit Brilland, Samuel Wacrenier, Assia Djema, Anne Sophie Garnier, Renaud Gansey, Jean-Philippe Coindre, Virginie Besson, Agnès Duveau, Jean-François Subra, Maud Cousin, Giorgina Barbara Piccoli and Jean-François Augusto
J. Clin. Med. 2020, 9(10), 3177; https://doi.org/10.3390/jcm9103177 - 30 Sep 2020
Cited by 9 | Viewed by 1804
Abstract
(1) Introduction: The incidence of venous thromboembolisms (VTE) has not been extensively analyzed in patients with antineutrophil cytoplasmic antibody (ANCA)-glomerulonephritis (ANCA-GN). Thus, the aim of the present study was to assess the frequency and the risk factors of VTE in patients with ANCA-GN. [...] Read more.
(1) Introduction: The incidence of venous thromboembolisms (VTE) has not been extensively analyzed in patients with antineutrophil cytoplasmic antibody (ANCA)-glomerulonephritis (ANCA-GN). Thus, the aim of the present study was to assess the frequency and the risk factors of VTE in patients with ANCA-GN. (2) Methods: Patients from the Maine-Anjou ANCA-associated vasculitis (AAV) registry with a biopsy showing pauci-immune glomerulonephritis were included. VTE events, site, and interval from AAV diagnosis were analyzed. (3) Results: 133 patients fulfilled the inclusion criteria of the study and were analyzed. VTE episodes were diagnosed in 23/133 (17.3%) patients at a median delay of 3 months from ANCA-GN diagnosis. Patients with VTE had lower serum albumin (p = 0.040), were less frequently on statin therapy (p = 0.009) and had less frequently proteinase-3 (PR3)-ANCAs (p = 0.078). Univariate analysis identified higher age (p = 0.022), lower serum albumin (p = 0.030), lack of statin therapy (p = 0.009), and rituximab treatment (p = 0.018) as significant risk factors of VTE. In multivariate analysis, only lack of statin therapy (HR 4.873; p = 0.042) was significantly associated with VTE. (4) Conclusion: Patients with ANCA-GN are at high risk of VTE, especially within the first months following AAV diagnosis. Our results suggest that statin therapy is associated with a lower risk of VTE in ANCA-GN patients. Full article
(This article belongs to the Special Issue Small-Vessel Vasculitis)
►▼ Show Figures

Figure 1

Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:
 
Displaying articles 1-3
Back to TopTop