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Autoimmune and Autoinflammatory Disorders: Current Issue and Future Perspectives

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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: 30 June 2024 | Viewed by 10215

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Special Issue Editors

Prof. Giuseppe Lopalco

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Guest Editor

Department of Precision and Regenerative Medicine and Ionian Area (DiMePre-J), University "Aldo Moro" of Bari, 70121 Bari, Italy
Interests: adaptive and innate immunity; spondyloarthritis; precision medicine

Prof. Luca Cantarini

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Guest Editor

Rheumatology Unit, Department of Medical, Surgical Sciences and Neurosciences, University Hospital of Siena, 53100 Siena, Italy
Interests: innate immunity; IL-1 mediated diseases; inflammasome

Dr. Daniela Marotto

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Guest Editor

Interdistrict Rheumatology Unit, ASL Gallura, 07026 Olbia, Tempio Pausania, Italy
Interests: innate and adaptive immunity; gender medicine; IL1 mediated diseases

Special Issue Information

Dear Colleagues,

Extreme, unrestricted activation of the immune system has pathologic consequences. In some cases, the uncontrolled activation is restricted to adaptive immune cells, leading to autoimmune disorders; in others, the innate immune response can result in clinical phenotypes and organ damage. At times, both processes may be dysregulated.

Recent findings in autoimmune and autoinflammatory disorders are providing unique insights into key immune pathways that, when dysregulated, drive pathologic immune phenotypes. These studies provide clues that not only disclose targets for treatment, but also point to the knitting and coordination of innate and adaptive immune processes in causing immune dysregulation and tissue pathology.

This Special Issue pursues studies focusing on autoimmune and autoinflammatory diseases that may lead to improve our knowledge on these disorders. We welcome contributions aimed at covering the pathogenetic, clinical and therapeutic aspects in relation to autoimmune and autoinflammatory disorders in the field of rheumatology.

Prof. Giuseppe Lopalco
Prof. Luca Cantarini
Dr. Daniela Marotto
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

spondyloarthritis
innate immunity
adaptive immunity
interleukin-1
IL-1-mediated diseases
inflammasome
precision medicine
gender medicine

Published Papers (5 papers)

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Research

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17 pages, 2941 KiB

Open AccessArticle

Rheumatologist’s Perspective on Non-Infectious Uveitis: Patterns from Tertiary Referral Rheumatologic Clinics in Italy

by Paola Triggianese, Mauro Fatica, Francesco Caso, Luisa Costa, Arianna D’Antonio, Marco Tasso, Elisabetta Greco, Paola Conigliaro, Alberto Bergamini, Claudia Fabiani, Luca Cantarini and Maria Sole Chimenti

Int. J. Mol. Sci. 2023, 24(11), 9690; https://doi.org/10.3390/ijms24119690 - 02 Jun 2023

Cited by 2 | Viewed by 1236

Abstract

Non-infectious uveitis (NIU) can be an early or even the first extra-articular manifestation of systemic rheumatic diseases, or the first one; thus, rheumatologists are often involved in the diagnostic and therapeutic assessment of NIU. We evaluated 130 patients with a diagnosis of NIU who were admitted to two Italian rheumatologic clinics (Tor Vergata University Hospital in Rome, and Federico II University in Naples) from January 2018 to December 2021. Anterior uveitis (AU) occurred in 75.4% of patients, followed by posterior uveitis (PU, 21.5%); acute (54.6%) and recurrent (35.4%) NIU were more documented than chronic NIU (10%), and a bilateral involvement was observed in 38.7% of cases. Half of NIU cases were associated with spondyloarthritis (SpA); the remaining were affected by Behçet disease (BD)-related uveitis (13.9%) and idiopathic NIU (9.2%). HLA-B27⁺ patients (34.8%) had a higher prevalence of anterior and unilateral NIU (p = 0.005) with acute course (p = 0.04) than HLA-B27^– patients. On the contrary, HLA-B51⁺ patients (19.6%) had mostly PU and bilateral NIU (p < 0.0001) and recurrent course (p = 0.04) than HLA-B51^– patients. At the first rheumatologic referral, 117 patients (90%) received systemic treatments. Findings from this study demonstrate that rheumatologic referral has a pivotal role in the diagnostic work-up of NIU and may dramatically influence NIU-treatment strategies. Full article

(This article belongs to the Special Issue Autoimmune and Autoinflammatory Disorders: Current Issue and Future Perspectives)

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12 pages, 1306 KiB

Open AccessArticle

Serum Interleukin-36 α as a Candidate Biomarker to Distinguish Behçet’s Syndrome and Psoriatic Arthritis

by Alessandra Bettiol, Filippo Fagni, Irene Mattioli, Giacomo Bagni, Gianfranco Vitiello, Alessia Grassi, Chiara Della Bella, Marisa Benagiano, Arianna Troilo, Katarzyna Stella Holownia, David Simon, Flavia Rita Argento, Jurgen Sota, Claudia Fabiani, Matteo Becatti, Claudia Fiorillo, Georg Schett, Giuseppe Lopalco, Luca Cantarini, Domenico Prisco, Elena Silvestri, Giacomo Emmi and Mario Milco D’Elios Show full author list Hide full author list

Int. J. Mol. Sci. 2023, 24(10), 8817; https://doi.org/10.3390/ijms24108817 - 16 May 2023

Cited by 3 | Viewed by 1249

Abstract

Behçet’s syndrome (BS) is a rare systemic vasculitis characterized by different clinical manifestations. As no specific laboratory tests exist, the diagnosis relies on clinical criteria, and the differential diagnosis with other inflammatory diseases can be challenging. Indeed, in a relatively small proportion of patients, BS symptoms include only mucocutaneous, articular, gastrointestinal, and non-typical ocular manifestations, which are frequently found also in psoriatic arthritis (PsA). We investigate the ability of serum interleukin (IL)-36α—a pro-inflammatory cytokine involved in cutaneous and articular inflammatory diseases—to differentiate BS from PsA. A cross-sectional study was performed on 90 patients with BS, 80 with PsA and 80 healthy controls. Significantly lower IL-36α concentrations were found in patients with BS as compared to PsA, although in both groups IL-36α was significantly increased compared to healthy controls. An empirical cut-off of 420.6 pg/mL displayed a specificity of 0.93, with a sensitivity of 0.70 (AUC 0.82) in discriminating PsA from BS. This cut-off displayed a good diagnostic performance also in BS patients lacking highly specific BS manifestations. Our results indicate that IL-36α might be involved in the pathogenesis of both BS and PsA, and might be a candidate biomarker to support the differential diagnosis of BS. Full article

(This article belongs to the Special Issue Autoimmune and Autoinflammatory Disorders: Current Issue and Future Perspectives)

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10 pages, 842 KiB

Open AccessCommunication

Immunogenicity and Safety of Adjuvanted Recombinant Zoster Vaccine in Rheumatoid Arthritis Patients on Anti-Cellular Biologic Agents or JAK Inhibitors: A Prospective Observational Study

by Vincenzo Venerito, Pasquale Stefanizzi, Luca Cantarini, Marlea Lavista, Maria Grazia Galeone, Antonio Di Lorenzo, Florenzo Iannone, Silvio Tafuri and Giuseppe Lopalco

Int. J. Mol. Sci. 2023, 24(8), 6967; https://doi.org/10.3390/ijms24086967 - 09 Apr 2023

Cited by 5 | Viewed by 3138

Abstract

Rheumatoid arthritis (RA) patients on JAK inhibitors (JAKi) have an increased HZ risk compared to those on biologic DMARDs (bDMARDs). Recently, the Adjuvanted Recombinant Zoster Vaccine (RZV) became available worldwide, showing good effectiveness in patients with inflammatory arthritis. Nevertheless, direct evidence of the immunogenicity of such a vaccine in those on JAKi or anti-cellular bDMARDs is still lacking. This prospective study aimed to assess RZV immunogenicity and safety in RA patients receiving JAKi or anti-cellular bDMARDs that are known to lead to impaired immune response. Patients with classified RA according to ACR/EULAR 2010 criteria on different JAKi or anti-cellular biologics (namely, abatacept and rituximab) followed at the RA clinic of our tertiary center were prospectively observed. Patients received two shots of the RZV. Treatments were not discontinued. At the first and second shots, and one month after the second shot, from all patients with RA, a sample was collected and RZV immunogenicity was assessed and compared between the treatment groups and healthy controls (HCs) receiving RZV for routine vaccination. We also kept track of disease activity at different follow-up times. Fifty-two consecutive RA patients, 44 females (84.61%), with an average age (±SD) of 57.46 ± 11.64 years and mean disease duration of 80.80 ± 73.06 months, underwent complete RZV vaccination between February and June 2022 at our center. At the time of the second shot (1-month follow-up from baseline), anti-VZV IgG titer increased significantly in both groups with similar magnitude (bDMARDs: 2258.76 ± 897.07 mIU/mL; JAKi: 2059.19 ± 876.62 mIU/mL, p < 0.001 for both from baseline). At one-month follow-up from the second shot, anti-VZV IgG titers remained stable in the bDMARDs group (2347.46 ± 975.47) and increased significantly in the JAKi group (2582.65 ± 821.59 mIU/mL, p = 0.03); still, no difference was observed between groups comparing IgG levels at this follow-up time. No RA flare was recorded. No significant difference was shown among treatment groups and HCs. RZV immunogenicity is not impaired in RA patients on JAKi or anti-cellular bDMARDs. A single shot of RZV can lead to an anti-VZV immune response similar to HCs without discontinuing DMARDs. Full article

(This article belongs to the Special Issue Autoimmune and Autoinflammatory Disorders: Current Issue and Future Perspectives)

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Review

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21 pages, 1040 KiB

Open AccessReview

“Neuroimmunoendocrinology” in Children with Rheumatic Diseases: How Glucocorticoids Are the Orchestra Director

by Maria Cristina Maggio, Angela Miniaci, Romina Gallizzi and Adele Civino

Int. J. Mol. Sci. 2023, 24(17), 13192; https://doi.org/10.3390/ijms241713192 - 25 Aug 2023

Cited by 2 | Viewed by 1134

Abstract

The neural, the endocrine, and the immune systems are studied as distinct districts in physiological and pathological settings. However, these systems must be investigated with an integrative approach, while also considering that therapeutic agents, such as glucocorticoids, can induce a reversible or irreversible change of this homeostasis. Children and adolescents affected by rheumatic diseases frequently need treatment with corticosteroids, and the treatment must sometimes be continued for a long time. In the biological era, the treat-to-target strategy allowed a real revolution in treatment, with significant steroid dose sparing or, in many patients, steroid treatment withdrawal. In this review, the impact of glucocorticoids on endocrine, immune, and neurologic targets is analyzed, and the crosstalk between these systems is highlighted. In this narrative review, we explore the reasoning as to why glucocorticoids can disrupt this homeostasis, we summarize some of the key results supporting the impact of glucocorticoids treatment on endocrine, immune, and neurologic systems, and we discuss the data reported in the international literature. Full article

(This article belongs to the Special Issue Autoimmune and Autoinflammatory Disorders: Current Issue and Future Perspectives)

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24 pages, 19768 KiB

Open AccessReview

An Update on Familial Mediterranean Fever

by Maddalena Lancieri, Marta Bustaffa, Serena Palmeri, Ignazia Prigione, Federica Penco, Riccardo Papa, Stefano Volpi, Roberta Caorsi and Marco Gattorno

Int. J. Mol. Sci. 2023, 24(11), 9584; https://doi.org/10.3390/ijms24119584 - 31 May 2023

Cited by 12 | Viewed by 2865

Abstract

(1) Background: Familial Mediterranean Fever (FMF) is the prototypal autoinflammatory disease, characterized by recurrent bursts of neutrophilic inflammation. (2) Methods: In this study we look at the most recent literature on this condition and integrate it with novel information on treatment resistance and compliance. (3) Results: The canonical clinical presentation of FMF is in children with self-limited episodes of fever and polyserositis, associated with severe long-term complications, such as renal amyloidosis. It has been described anecdotally since ancient times, however only recently it has been characterized more accurately. We propose an updated overview on the main aspects of pathophysiology, genetics, diagnosis and treatment of this intriguing disease. (4) Conclusions: Overall, this review presents the all the main aspects, including real life outcome of the latest recommendation on treatment resistance of FMF, a disease, that not only helped understanding the pathophysiology of the auto inflammatory process but also the functioning of the innate immune system itself. Full article

(This article belongs to the Special Issue Autoimmune and Autoinflammatory Disorders: Current Issue and Future Perspectives)

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