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Lupus and Scleroderma: New Aspects and Considerations
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Lupus and Scleroderma: New Aspects and Considerations

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

Deadline for manuscript submissions: closed (30 May 2023) | Viewed by 14978

Special Issue Editors

Dr. Isabel Castrejon

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Guest Editor
Rheumatology Department, Hospital General Universitario Gregorio Marañón. Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Universidad Complutense de Madrid. Dr. Esquerdo 46, 28007 Madrid, Spain
Interests: epidemiology; disease activity measures; lupus; scleroderma; rheumatoid arthritis
Prof. Dr. David A. Isenberg

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Guest Editor
Department of Rheumatology, Division of Medicine, University College London, London, UK.
Interests: lupus; autoimmune rheumatic diseases
Dr. Francesca Ingegnoli

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Guest Editor
Clinical Rheumatology Unit, ASST Pini-CTO, Department of Clinical Science and Community Health, Università degli Studi di Milano, Milan, Italy
Interests: systemic sclerosis; rheumatoid athritis; nutrition; patient reported outcomes; capillaroscopy
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Both systemic lupus erythematous (SLE) and systemic sclerosis (scleroderma) (SSc) are autoimmune diseases that can result in substantial morbidity and mortality. Diagnosis and treatment for both are clinical challenges because patient presentation and response to therapy are heterogeneous. SLE management has improved over the years; however, many patients will have irreversible organ damage by the time they are diagnosed. A greater understanding of the preclinical SLE, genetic risk, and environmental factors can help to identify and treat patients earlier. Tremendous progress has been made over the last few decades with the identification of novel biomarkers and with epidemiologist and genetic studies. Despite the progress in treating organ involvement in SSc, however, effective treatments for cutaneous fibrosis remain elusive. None of the disease-modifying antirheumatic drugs have shown proven efficacy for SSc skin fibrosis, and it is challenging to carry out clinical trials because of SSc low prevalence, diverse clinical manifestations, and variable and fluctuating course. In this Special Issue, we will focus on emerging diagnostic tools or biomarkers, novel therapeutics and non-pharmacologic interventions, comorbidity management, and other challenging aspects of lupus and scleroderma.

Dr. Isabel Castrejon
Prof. Dr. David A. Isenberg
Dr. Francesca Ingegnoli
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. 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

  • lupus
  • scleroderma
  • autoimmune diseases
  • interstitial lung disease
  • biologics
  • treat to target
  • remission

Published Papers (5 papers)

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Research

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10 pages, 6045 KiB  
Article
Characteristics of Hearing Loss in Patients with Systemic Lupus Erythematosus
by Huixian Chen, Fan Wang, Ye Yang, Bingzhu Hua, Hong Wang, Jie Chen and Xuebing Feng
J. Clin. Med. 2022, 11(24), 7527; https://doi.org/10.3390/jcm11247527 - 19 Dec 2022
Viewed by 1756
Abstract
Objective: To investigate the clinical characteristics of hearing loss (HL) in patients with systemic lupus erythematosus (SLE) and its related factors. Methods: Ninety-one hospitalized SLE patients and thirty healthy controls were enrolled. All subjects completed pure tone audiometry (PTA), extended high frequency audiometry [...] Read more.
Objective: To investigate the clinical characteristics of hearing loss (HL) in patients with systemic lupus erythematosus (SLE) and its related factors. Methods: Ninety-one hospitalized SLE patients and thirty healthy controls were enrolled. All subjects completed pure tone audiometry (PTA), extended high frequency audiometry (EHFA) and distortion product otoacoustic emission (DPOAE) to assess hearing function. SLE patients were divided into two groups according to the presence or absence of HL, and the risk factors of HL were determined by multivariate logistic regression. Results: The incidence of HL was 27.47% in SLE patients, significantly higher than in the control group (3.3%) and most cases were mild-to-moderate, bilateral and predominantly sensorineural. Compared with the control group, the hearing thresholds of SLE patients increased significantly in the middle and high frequencies starting from 2000 Hz. Even though the PTA test results were normal, the EHFA test results showed significant differences in hearing impairment between SLE patients and normal controls. For patients with abnormal PTA results, the signal-to-noise ratio (SNR) in DPOAE was markedly reduced, and the pass rate was also decreased. The Systemic Lupus International Collaborating Clinics Damage Index (SDI, OR 9.13) and secondary Sjögren’s syndrome (sSS, OR 8.20) were identified as independent associated factors for HL, and there was no difference in PTA and EHFA at all frequencies between hydroxychloroquine users and non-users. Conclusions: HL is not rare in SLE patients, and EHFA can help identify early hearing impairment. Having a high SDI score and secondary Sjögren’s syndrome may predict the presence of HL in SLE. Full article
(This article belongs to the Special Issue Lupus and Scleroderma: New Aspects and Considerations)
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Review

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12 pages, 625 KiB  
Review
The Impact of Progressive Pulmonary Fibrosis in Systemic Sclerosis–Associated Interstitial Lung Disease
by María Martín-López and Patricia E. Carreira
J. Clin. Med. 2023, 12(20), 6680; https://doi.org/10.3390/jcm12206680 - 23 Oct 2023
Cited by 1 | Viewed by 1496
Abstract
Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by immune dysregulation and progressive fibrosis, typically affecting the skin, with variable internal organ involvement. Interstitial lung disease (ILD), with a prevalence between 35 and 75%, is the leading cause of death in [...] Read more.
Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by immune dysregulation and progressive fibrosis, typically affecting the skin, with variable internal organ involvement. Interstitial lung disease (ILD), with a prevalence between 35 and 75%, is the leading cause of death in patients with SSc, indicating that all newly diagnosed patients should be screened for this complication. Some patients with SSc-ILD experience a progressive phenotype, which is characterized by worsening fibrosis on high-resolution computed tomography (HRCT), a decline in lung function, and premature mortality. To assess progression and guide therapeutic decisions, regular monitoring is essential and should include pulmonary function testing (PFT), symptom assessment, and repeat HRCT imaging when indicated. Multidisciplinary discussion allows a comprehensive evaluation of the available information and its consequences for management. There has been a shift in the approach to managing SSc-ILD, which includes the addition of targeted biologic and antifibrotic therapies to standard immunosuppressive therapy (particularly mycophenolate mofetil or cyclophosphamide), with autologous hematopoietic stem-cell transplantation and lung transplantation reserved for refractory cases. Full article
(This article belongs to the Special Issue Lupus and Scleroderma: New Aspects and Considerations)
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9 pages, 269 KiB  
Review
Steroids in Lupus: Enemies or Allies
by Eugenia Enríquez-Merayo and Maria J. Cuadrado
J. Clin. Med. 2023, 12(11), 3639; https://doi.org/10.3390/jcm12113639 - 24 May 2023
Viewed by 2177
Abstract
Glucocorticoids are the gold standard treatment for reducing immune activation and inflammation in a wide range of inflammatory and systemic autoimmune diseases. Glucocorticoids have potent and fast actions that quickly relieve some symptoms and lower mortality in some life-threatening conditions, but they also [...] Read more.
Glucocorticoids are the gold standard treatment for reducing immune activation and inflammation in a wide range of inflammatory and systemic autoimmune diseases. Glucocorticoids have potent and fast actions that quickly relieve some symptoms and lower mortality in some life-threatening conditions, but they also have side effects that limit the duration of treatment and the dose used. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by the involvement of numerous organs and systems and the production of autoantibodies. Most current treatments include the use of corticosteroids and immunosuppressive medications. Glucocorticoids in SLE have been classically used not only to induce remission or treat an acute situation but also as maintenance therapy. During the last decades, new approaches to managing SLE have emerged, but corticosteroids continue to be part of all therapeutic regimes. There is more and more evidence about the side effects related to the use (or abuse) of steroids and their relationship with the accrual damage. In this manuscript, we try to make a critical review of the published literature about the benefit and side effects/damage that can be attributed to the use of glucocorticoids. Full article
(This article belongs to the Special Issue Lupus and Scleroderma: New Aspects and Considerations)
12 pages, 275 KiB  
Review
Treat-to-Target in Systemic Lupus Erythematosus: Reality or Pipe Dream
by Dina Zucchi, Chiara Cardelli, Elena Elefante, Chiara Tani and Marta Mosca
J. Clin. Med. 2023, 12(9), 3348; https://doi.org/10.3390/jcm12093348 - 08 May 2023
Cited by 3 | Viewed by 1986
Abstract
Treat-to-target is a therapeutic approach based on adjustments to treatment at set intervals in order to achieve well-defined, clinically relevant targets. This approach has been successfully applied to many chronic conditions, and in rheumatology promising results have emerged for rheumatoid arthritis. For systemic [...] Read more.
Treat-to-target is a therapeutic approach based on adjustments to treatment at set intervals in order to achieve well-defined, clinically relevant targets. This approach has been successfully applied to many chronic conditions, and in rheumatology promising results have emerged for rheumatoid arthritis. For systemic lupus erythematosus (SLE), defining the most meaningful treatment targets has been challenging, due to disease complexity and heterogeneity. Control of disease activity, the reduction of damage accrual and the patient’s quality of life should be considered as the main targets in SLE, and several new drugs are emerging to achieve these targets. This review is focused on describing the target to achieve in SLE and the methods to do so, and it is also aimed at discussing if treat-to-target could be a promising approach also for this complex disease. Full article
(This article belongs to the Special Issue Lupus and Scleroderma: New Aspects and Considerations)
31 pages, 7029 KiB  
Review
Targeted Therapy for SLE—What Works, What Doesn’t, What’s Next
by Veronica Venturelli and David Alan Isenberg
J. Clin. Med. 2023, 12(9), 3198; https://doi.org/10.3390/jcm12093198 - 29 Apr 2023
Viewed by 6987
Abstract
For many years, the failure of randomized controlled trials (RCTs) has prevented patients with systemic lupus erythematosus (SLE) from benefiting from biological drugs that have proved to be effective in other rheumatological diseases. Only two biologics are approved for SLE, however they can [...] Read more.
For many years, the failure of randomized controlled trials (RCTs) has prevented patients with systemic lupus erythematosus (SLE) from benefiting from biological drugs that have proved to be effective in other rheumatological diseases. Only two biologics are approved for SLE, however they can only be administered to a restricted proportion of patients. Recently, several phase II RCTs have evaluated the efficacy and safety of new biologics in extra-renal SLE and lupus nephritis. Six drug trials have reported encouraging results, with an improvement in multiple clinical and serological outcome measures. The possibility of combining B-cell depletion and anti-BLyS treatment has also been successfully explored. Full article
(This article belongs to the Special Issue Lupus and Scleroderma: New Aspects and Considerations)
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