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Vasculitis: Current Treatment and Future Options

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

Deadline for manuscript submissions: closed (15 September 2024) | Viewed by 17918

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Dr. Carlos García-Porrúa

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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

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Published Papers (7 papers)

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Research

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12 pages, 479 KiB

Open AccessArticle

18F-FDG-PET/CT Scan for Detection of Large Vessel Involvement in Giant Cell Arteritis: Arteser Spanish Registry

by Paula Estrada, Marta Domínguez-Álvaro, Rafael B. Melero-González, Eugenio de Miguel, Maite Silva-Díaz, Jesús A. Valero, Ismael González, Julio Sánchez-Martín, Javier Narváez, Eva Galíndez, Javier Mendizábal, Carlota L. Iñiguez-Ubiaga, Luis Rodríguez-Rodríguez, Javier Loricera, Alejandro Muñoz, Patricia Moya-Alvarado, Patricia Moran-Álvarez, Vanessa A. Navarro-Ángeles, Carlos Galisteo, Santos Castañeda, Ricardo Blanco and on behalf of ARTESER Project Collaborative Group Show full author list Hide full author list

J. Clin. Med. 2024, 13(20), 6215; https://doi.org/10.3390/jcm13206215 - 18 Oct 2024

Viewed by 1536

Abstract

Background/Objectives: Imaging studies have transformed the diagnosis of large vessel vasculitis (LVV) involvement in giant cell arteritis (GCA). A positron emission tomography/computed tomography (PET/CT) scan with 18-fluorodeoxyglucose (18F-FDG) has emerged as a valuable tool for assessing LVV. We aimed to determine the utility of an 18F-FDG-PET/CT scan in detecting LVV in GCA in the ARTESER registry. Methods: The ARTESER study is a large multicenter, retrospective, longitudinal, and observational study, promoted by the Spanish Society of Rheumatology. It included patients newly diagnosed with GCA across 26 tertiary hospitals from 1 June 2013 to 29 March 2019. Patients with a diagnosis of incidental GCA were included if they fulfilled specific criteria, including the ACR 1990 criteria, positive imaging examinations, or the expert clinical opinion of investigators. Differences between patients with positive and negative 18F-FDG-PET/CT scan results were analyzed using a bivariate model. A regression model assessed associations in patients with a positive scan, and the predictive capacity of the cumulative dose of glucocorticoids (GC) on PET scan outcomes was evaluated using ROC curve analysis. Results: Out of 1675 GCA patients included in the registry, 377 met the inclusion criteria of having an 18F-FDG-PET/CT scan. The majority were diagnosed with a cranial GCA phenotype, and 65% had LVV. The thoracic aorta was the most frequently affected. Cardiovascular disease, diabetes, and older age had a negative association with a positive scan outcome. The OR for having a positive 18F-FDG-PET/CTC scan was lower as the number of days increased. Depending on the cumulative dosage of the GC, the 18F-FDG-PET/CT scan showed an AUC of 0.74, with a Youden index > 60 mg/day. Conclusions: Younger patients showed a higher probability of presenting LVV as detected by the 18F-FDG-PET/CT scan. The timing of the examination and the cumulative dosage of the GC influenced the likelihood of a positive result, with earlier tests being more likely to detect inflammation. Full article

(This article belongs to the Special Issue Vasculitis: Current Treatment and Future Options)

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12 pages, 280 KiB

Open AccessArticle

Clinical and Serological Profiles in Cryoglobulinemia: Analysis of Isotypes and Etiologies

by Helena Codes-Méndez, Sicylle Jeria, Hye-Sang Park, Patricia Moya, Berta Magallares-López, Elisabeth Moltó, Yolanda Álvaro, Anais Mariscal, Esther Moga, Jose Luis Tandaipan, César Díaz-Torne, Ana Laiz, Luis Sainz, Ivan Castellví and Hector Corominas

J. Clin. Med. 2024, 13(20), 6069; https://doi.org/10.3390/jcm13206069 - 11 Oct 2024

Cited by 2 | Viewed by 1397

Abstract

Objectives: Cryoglobulinemia (CG) is marked by abnormal immunoglobulins (Ig) in serum, precipitating at temperatures below 37 °C. Current classification categorizes CG into three subtypes (types I, II, and III) based on Ig clonality. The features distinguishing patients with CG based on their etiology remain unidentified. Aiming to characterize clinical and serological profiles of CG individuals, we conducted an observational analysis of a large cohort of patients and compared their characteristics based on underlying causes: hepatovirus (HV) infections, rheumatic diseases (RD), hematological disorders, and unidentified etiology (essential CG). Methods: We analyzed 252 cryoglobulin-positive serum samples from 182 patients and classified these into the four etiological groups. A separate sub-analysis was carried out for 10 patients meeting criteria for multiple diseases. We collected demographic, clinical, and laboratory data: CG characterization, complement (C3 and C4) levels, antinuclear antibodies (ANA), and rheumatoid factor (RF). Kruskal–Wallis and Wilcoxon–Mann–Whitney U-tests were used for comparisons. Results: Most patients (93.3%) had mixed cryoglobulinemia (types II + III), with 6.7% having type I. HV infection, predominantly hepatitis C, was the main (52.9%) associated condition within the cohort, followed by rheumatic (27.3%) and hematological (9.8%) disorders. In our cohort, ANA were frequent (45.3%) and often associated with RF positivity (43.6%) and decreased complement levels (C3: 42.4%, C4: 32.5%). Essential CG and CG associated with RD had a higher prevalence of cutaneous manifestations (p < 0.01) and renal involvement (p = 0.017). Hematological disorder-related CG showed higher cryoglobulin and RF concentrations (p < 0.01), despite milder symptoms. Conclusions: Our study underscores a mixed prevalence of CG across disease subgroups, with hepatitis-C virus as the primary factor, followed by rheumatic and hematological disorders. Four clinical and serological profiles of CG were identified based on their etiologies. Full article

(This article belongs to the Special Issue Vasculitis: Current Treatment and Future Options)

13 pages, 4781 KiB

Open AccessArticle

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 1164

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 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

Open AccessArticle

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

Cited by 5 | Viewed by 1292

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 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

Open AccessFeature PaperArticle

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 10 | Viewed by 2057

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 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)

Review

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21 pages, 390 KiB

Open AccessFeature PaperEditor’s ChoiceReview

IgA Vasculitis (Henoch–Schönlein Purpura): An Update on Treatment

by Santos Castañeda, Patricia Quiroga-Colina, Paz Floranes, Miren Uriarte-Ecenarro, Cristina Valero-Martínez, Esther F. Vicente-Rabaneda and Miguel A. González-Gay

J. Clin. Med. 2024, 13(21), 6621; https://doi.org/10.3390/jcm13216621 - 4 Nov 2024

Cited by 1 | Viewed by 8286

Abstract

Objective: IgA vasculitis (IgAV), previously named as Henoch–Schönlein purpura, is the most frequent systemic vasculitis in children. In adults, IgAV is less common although it is associated with more severe disease. In fact, the frequency of glomerulonephritis (referred to as IgAV nephritis) in adults is higher than in children and tends to present more severely, with around 10–30% of those affected eventually progressing to end-stage renal disease. In this review, we describe the pathophysiology, main clinical features, diagnosis of the disease, and latest clinical data regarding IgAV therapy. Methods: A narrative literature review, primarily based on articles published in PubMed, was conducted. In addition to discussing the main aspects of glucocorticoids and conventional disease-modifying drugs used in the management of IgAV, this review focuses on the latest information reported regarding biologics and potential future therapies. Results: Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations. Colchicine, dapsone, and methotrexate can be useful for controlling minor manifestations. Several immunomodulatory agents, such as cyclosporine A, tacrolimus, and mycophenolate mofetil, have shown favorable results as glucocorticoid-sparing agents. Leflunomide has shown promising results but requires further study. The use of rituximab has demonstrated efficacy in reducing relapse frequency, lowering the cumulative glucocorticoid burden, and achieving long-term remission of the disease in children and adults with IgAV. Immunoglobulins and plasma exchange therapy can also be useful in difficult and life-threatening situations. Other potential therapies with encouraging results include TRF-budesonide, B-cell-directed therapy, B-cell-depleting agents, sodium–glucose cotransporter-2 inhibitors, endothelin receptor antagonists, and complement pathway inhibitors. Conclusions: Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations. The role of various immunomodulatory therapies, such as calcineurin inhibitors and mycophenolate mofetil, remains promising, while rituximab reduces the long-term side effects of glucocorticoids and can help achieve disease remission. Other potential therapies with encouraging results require further research. Full article

(This article belongs to the Special Issue Vasculitis: Current Treatment and Future Options)

21 pages, 869 KiB

Open AccessReview

Traditional and Emerging Strategies for Managing Polymyalgia Rheumatica: Insights into New Treatments

by Carlos García-Porrúa, Elena Heras-Recuero, Teresa Blázquez-Sánchez, Arantxa Torres-Roselló, Santos Castañeda and Miguel Ángel González-Gay

J. Clin. Med. 2024, 13(21), 6492; https://doi.org/10.3390/jcm13216492 - 29 Oct 2024

Viewed by 1831

Abstract

Background/Objectives: Polymyalgia Rheumatica (PMR) is an inflammatory condition that primarily affects individuals aged 50 and older, especially in Western countries. Although glucocorticoids are the cornerstone of PMR treatment, these drugs are associated with side effects, making it advisable to use them for the shortest duration possible. However, tapering or discontinuation of glucocorticoids often leads to disease relapses. In this review, we focus on the traditional management of PMR, as well as the potential for therapies that may reduce glucocorticoid use. Special attention is given to the efficacy of biologic agents in PMR management. Methods: A literature review, primarily based on articles published in PubMed, was conducted. In addition to discussing various glucocorticoids and conventional disease-modifying drugs used for the management of isolated PMR, this review specifically focused on the information reported regarding new therapies, with particular emphasis on biologic agents. Results: Prednisone or prednisolone at a dose ranging between 12.5 and 25 mg/day is the agreed-upon treatment for PMR. Due to the side effects associated with prolonged glucocorticoid use and the high frequency of relapses when glucocorticoids are tapered, glucocorticoid-sparing agents have emerged as tools in the management of PMR. Methotrexate has traditionally been the conventional disease-modifying antirheumatic drug (DMARD) unanimously recommended for use in PMR. Other conventional DMARDs, such as leflunomide, have shown promising results but require further study. The use of biologic agents has marked a significant step forward in the management of PMR. While anti-TNF agents failed to provide beneficial effects in isolated PMR, anti-IL-6 receptor agents, such as tocilizumab and sarilumab, have demonstrated efficacy in reducing relapse frequency, lowering the cumulative glucocorticoid burden, and achieving long-term remission of the disease. Other biologic agents, many of which have been used in giant cell arteritis, as well as Janus kinase (JAK) inhibitors, are currently under investigation. Conclusions: Glucocorticoids are the primary treatment for isolated PMR but are associated with comorbidities, especially in patients with pre-existing conditions or frequent relapses. Glucocorticoid-sparing agents, such as methotrexate and biologics, in particular tocilizumab and sarilumab, offer alternatives, improving symptoms and reducing glucocorticoid use. While biologic agents reduce long-term side effects and help achieve disease remission, their use must consider potential side effects and higher costs compared to traditional therapies. Full article

(This article belongs to the Special Issue Vasculitis: Current Treatment and Future Options)

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