Vasculitis: Current Treatment and Future Options

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

Deadline for manuscript submissions: 15 September 2024 | Viewed by 2973

Special Issue Editor


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Guest Editor
Servicio de Reumatología, Hospital Universitario Lucus Augusti, Lugo, Spain
Interests: vasculitis; psoriatic arthritis; rheumatoid arthritis; osteoporosis

Special Issue Information

Dear Colleagues,

Primary vasculitis constitutes a heterogeneous group of diseases characterized by blood vessel inflammation and necrosis. The most used classification was proposed by the Chapel Hill Consensus conference and is based on the size of the affected vessels, such as large- and small-vessel vasculitis. The use of glucocorticoids and conventional synthetic DMARDs has been the cornerstone of treatment. However, highly specific cytokine (e.g., tocilizumab, mepolizumab, and avacopan)-targeted synthetic DMARDs (e.g., apremilast and baricitinib) and cell-targeted drugs (e.g., rituximab and abatacept) are emerging in the treatment of vasculitis. It is therefore crucial to advance the knowledge of these treatments and their diffusion to the scientific community. Accordingly, a Special Issue titled “Vasculitis: Current Treatment and Future Options” is being launched. This Special Issue is calling for original research and reviews that investigate the clinical efficacy of current and future treatments used in vasculitis.

Dr. Carlos García-Porrúa
Guest Editor

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Keywords

  • vasculitis treatment
  • tocilizumab
  • mepolizumab
  • avacopan
  • apremilast
  • baricitinib
  • abatacept
  • rituximab

Published Papers (3 papers)

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Research

13 pages, 4781 KiB  
Article
IL-6 Receptor Expression on the Surface of T Cells and Serum Soluble IL-6 Receptor Levels in Patients with Microscopic Polyangiitis and Granulomatosis with Polyangiitis
by Taejun Yoon, Sung Soo Ahn, Eunhee Ko, Jason Jungsik Song, Yong-Beom Park and Sang-Won Lee
J. Clin. Med. 2023, 12(22), 7059; https://doi.org/10.3390/jcm12227059 - 13 Nov 2023
Viewed by 676
Abstract
We investigated the IL-6 receptor (IL-6R) expression on the surface of T cells isolated from peripheral blood mononuclear cells (PBMCs) of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) patients and measured the serum soluble IL-6R (sIL-6R) levels in these patients. Sera and [...] Read more.
We investigated the IL-6 receptor (IL-6R) expression on the surface of T cells isolated from peripheral blood mononuclear cells (PBMCs) of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) patients and measured the serum soluble IL-6R (sIL-6R) levels in these patients. Sera and PBMCs were obtained from 51 patients with MPA (n = 32) and GPA (n = 19), with 25 patients having active disease (defined as a Birmingham Vasculitis Activity Score [BVAS] ≥ 5). The median age of patients was 67.0 years, and 52.9% were women. Serum IL-6 levels were significantly correlated with the BVAS (r = 0.384); however, IL-6R expression on the surface of T cells did not significantly differ based on disease activity. Meanwhile, IL-6R expression on the surface of stimulated CD4+ (median mean fluorescence intensity [MFI] 588.0 vs. 1314.8; p < 0.001), CD4+CD25+ (MFI 853.3 vs. 1527.3; p < 0.001), and CD4+CD45RO+ (MFI 679.5 vs. 1241.5; p < 0.001) T cells was significantly reduced compared with unstimulated conditions. Conversely, patients with active disease exhibited a significantly higher median serum sIL-6R level than those with inactive disease (38.1 ng/mL vs. 34.7 ng/mL; p = 0.029). These results imply that the trans-signalling IL-6 pathway may be more activated than the classical signalling pathway in patients with MPA and GPA, suggesting the therapeutic potential of targeting sIL-6R. Full article
(This article belongs to the Special Issue Vasculitis: Current Treatment and Future Options)
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12 pages, 734 KiB  
Article
18F-Fluorodeoxyglucose Positron Emission Tomography–Computed Tomography Findings of Polymyalgia Rheumatica in Patients with Giant Cell Arteritis
by Elena Heras-Recuero, Marta Martínez de Bourio-Allona, Laura Cristina Landaeta-Kancev, Teresa Blázquez-Sánchez, Arantxa Torres-Roselló, Miguel Álvarez-Rubio, Mariam Belhaj-Gandar, Juan Antonio Martínez-López, Luis Martínez-Dhier, Javier Llorca, Raquel Largo and Miguel Ángel González-Gay
J. Clin. Med. 2023, 12(22), 6983; https://doi.org/10.3390/jcm12226983 - 8 Nov 2023
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Abstract
Objective: Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are often overlapping conditions. We studied whether 18F-fluorodeoxyglucose (FDG) positron emission tomography–computed tomography (PET-CT) is useful in identifying PMR in the setting of large vessel (LV) GCA. Methods: LV-GCA patients diagnosed by PET-CT at [...] Read more.
Objective: Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are often overlapping conditions. We studied whether 18F-fluorodeoxyglucose (FDG) positron emission tomography–computed tomography (PET-CT) is useful in identifying PMR in the setting of large vessel (LV) GCA. Methods: LV-GCA patients diagnosed by PET-CT at a tertiary care center for a population of 450,000 people over a two-year period were reviewed. Scoring was performed based on potential significant FDG uptake at up to 16 sites in nine different extravascular areas (SCORE 16). Differences in extravascular sites of significant FDG uptake were evaluated between LV-GCA with a clinical diagnosis of PMR or not. Results: Fifty-four patients were diagnosed with LV-GCA by 18F-FDG-PET-CT. Of them, 21 (38.8%) were clinically diagnosed with PMR. Significant extravascular FDG uptake was more frequently observed in those with a clinical diagnosis of PMR. In this sense, the SCORE 16 was higher in those with clinical PMR (5.10 ± 4.05 versus 1.73 ± 2.31 in those without a clinical diagnosis of PMR; p < 0.001). A SCORE 16 involving more than four sites of significant FDG uptake yielded a sensitivity of 52% and a specificity of 91% for establishing a clinical diagnosis of PMR associated with LV-GCA. The best areas of significant FDG uptake to clinically identify PMR in patients with LV-GCA were the shoulder, the greater trochanter, and the lumbar interspinous regions, with an area under the ROC curve of 0.810 (0.691–0.930). Conclusions: Significant extravascular 18F-FDG-PET-CT uptake may help establish a clinical diagnosis of PMR in patients with LV-GCA. These patients are more commonly diagnosed with PMR if they have significant FDG uptake in the shoulder, greater trochanter, and lumbar interspinous areas. Full article
(This article belongs to the Special Issue Vasculitis: Current Treatment and Future Options)
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11 pages, 284 KiB  
Article
Positron Emission Computed Tomography Spectrum of Large Vessel Vasculitis in a Tertiary Center: Differences in 18F-fluorodeoxyglucose Uptake between Large Vessel Vasculitis with Predominant Cranial and Extracranial Giant Cell Arteritis Phenotypes
by Elena Heras-Recuero, Laura Cristina Landaeta-Kancev, Marta Martínez de Bourio-Allona, Arantxa Torres-Rosello, Teresa Blázquez-Sánchez, Iván Ferraz-Amaro, Santos Castañeda, Juan Antonio Martínez-López, Luis Martínez-Dhier, Raquel Largo and Miguel Ángel González-Gay
J. Clin. Med. 2023, 12(19), 6164; https://doi.org/10.3390/jcm12196164 - 24 Sep 2023
Cited by 3 | Viewed by 1343
Abstract
(1) Objective:To assess the spectrum of PET-CT-related large vessel vasculitis (LVV) in a Spanish tertiary center and to determine whether FDG uptake by PET-CT differs between giant cell arteritis (GCA) with predominant cranial or extracranial phenotypes. (2) Methods: The spectrum of patients diagnosed [...] Read more.
(1) Objective:To assess the spectrum of PET-CT-related large vessel vasculitis (LVV) in a Spanish tertiary center and to determine whether FDG uptake by PET-CT differs between giant cell arteritis (GCA) with predominant cranial or extracranial phenotypes. (2) Methods: The spectrum of patients diagnosed with LVV by PET-CT in a tertiary referral hospital that cares for 450,000 people over a period of two years was reviewed. Moreover, differences in FDG uptake between LVV-GCA with predominantly cranial and extracranial phenotype were analyzed. (3) Results: Eighty patients were diagnosed with LVV by PET-CT. Most were due to systemic vasculitis (n = 64; 80%), especially GCA (n = 54; 67.5%). Other conditions included the presence of rheumatic diseases (n = 4; 3.2%), tumors (n = 9; 7.2%) and infections (n = 3; 2.4%). LVV-GCA patients with predominant extracranial GCA phenotype were younger (mean ± SD: 68.07 ± 9.91 vs. 75.46 ± 7.64 years; p = 0.017) and had a longer delay to the diagnosis (median [interquartile range] 12 [4–18] vs. 4 [3–8]; p = 0.006), but had polymyalgia rheumatica symptoms more frequently than those with predominantly cranial GCA phenotype (46.3% vs. 15.4%, p = 0.057). When FDG uptake was compared according to the two different disease patterns, no statistically significant differences were observed. However, patients with extracranial LVV-GCA showed a non-significantly higher frequency of vasculitic involvement of lower-extremity arteries. (4) Conclusions: Regardless of the predominant phenotype, LVV identified by PET-CT is more commonly due to GCA in the Spanish population. In these GCA patients, younger age, PMR, and a higher frequency of lower-extremity artery vasculitis suggest the presence of LVV. Full article
(This article belongs to the Special Issue Vasculitis: Current Treatment and Future Options)
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