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Advances in Systemic Sclerosis Research in Japan
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Advances in Systemic Sclerosis Research in Japan

A special issue of Sclerosis (ISSN 2813-3064).

Deadline for manuscript submissions: 10 November 2026 | Viewed by 3787

Special Issue Editor

Prof. Dr. Yoshihide Asano

E-Mail Website
Guest Editor
Department of Dermatology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-Machi, Aoba-Ku, Sendai 980-8574, Japan
Interests: systemic sclerosis; autoimmunity; angiogenesis; cancer-associated fibroblasts; irAE
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Systemic sclerosis (SSc) is a complex autoimmune disease characterized by fibrosis, vasculopathy and immune dysregulation. Japan has made significant contributions to elucidating the pathogenesis and developing novel therapeutic approaches for SSc, ranging from basic research to clinical and epidemiological studies. Recent advances include the identification of genetic and environmental risk factors, the discovery of key molecular pathways such as FLI1 and TGF-β signaling and the exploration of disease mechanisms involving vascular injury, fibroblast activation and immune cell dysfunction.

In addition, nationwide clinical and epidemiological studies have provided valuable insights into disease characteristics and treatment outcomes in Japanese patients. Notably, recent large-scale epidemiological research on juvenile-onset SSc has clarified its distinct clinical features and disease course compared with adult-onset SSc. These findings have deepened our understanding of disease heterogeneity and may guide tailored management strategies.

This Special Issue, “Advances in Systemic Sclerosis Research in Japan”, aims to highlight the latest progress in basic, clinical and epidemiological research conducted by Japanese investigators. By collecting original research articles and comprehensive reviews, this Issue seeks to provide an updated overview of the current understanding of SSc and to promote international collaboration toward improved patient care and precision medicine in this challenging disease.

Prof. Dr. Yoshihide Asano
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 250 words) can be sent to the Editorial Office for assessment.

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. Sclerosis is an international peer-reviewed open access quarterly 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 1000 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

  • systemic sclerosis
  • fibrosis
  • vasculopathy
  • immune dysregulation
  • patient care
  • precision medicine

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Further information on MDPI's Special Issue policies can be found here.

Published Papers (2 papers)

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Review

18 pages, 1250 KB  
Review
Targeting the IL-23p19/Th17 Axis with Guselkumab in Systemic Sclerosis: A Domain-Based Review of All Four Published Cases
by Souta Kobayashi, Yasuaki Ikuno, Masahiro Yamada, Akihiko Yamaguchi, Toshifumi Takahashi, Akiko Arakawa and Noriki Fujimoto
Sclerosis 2026, 4(2), 13; https://doi.org/10.3390/sclerosis4020013 - 7 Jun 2026
Viewed by 56
Abstract
Systemic sclerosis (SSc) is a heterogeneous autoimmune disease characterized by immune dysregulation, microvascular injury, and progressive cutaneous and internal organ fibrosis. Despite advances in the understanding of SSc pathogenesis, effective disease-modifying therapies remain limited, and there is ongoing interest in targeted approaches that [...] Read more.
Systemic sclerosis (SSc) is a heterogeneous autoimmune disease characterized by immune dysregulation, microvascular injury, and progressive cutaneous and internal organ fibrosis. Despite advances in the understanding of SSc pathogenesis, effective disease-modifying therapies remain limited, and there is ongoing interest in targeted approaches that may modulate early inflammatory-vascular mechanisms linked to subsequent fibrotic remodeling. The IL-23/Th17 axis contributes to SSc biology, providing a rationale for IL-23p19 blockade as a potential therapeutic strategy. This focused narrative review summarizes currently available clinical signals of IL-23p19 inhibition in SSc, centered on guselkumab, and interprets reported outcomes using a domain-based framework. Published evidence remains confined to uncontrolled reports totaling 4 patients (one case report of early limited cutaneous SSc and a three-patient case series of early diffuse cutaneous SSc with comorbid psoriasis). Across these reports, investigators described improvements most consistently in the cutaneous domain (modified Rodnan skin score [mRSS], modified Rodnan total skin score [mRTSS], and the American College of Rheumatology Combined Response Index in Systemic Sclerosis [CRISS]) and in microvascular manifestations, including Raynaud’s phenomenon and nailfold microangiopathy, whereas pulmonary and gastrointestinal findings remain preliminary. We discuss key limitations of the current literature, including publication and reporting bias, the heterogeneity of phenotypes and endpoints, and confounding by comorbid psoriatic disease and natural disease fluctuation. Overall, IL-23p19 inhibition offers a pathway-specific approach in SSc, but establishing efficacy and defining responsive phenotypes requires adequately powered controlled evidence. Findings pertain specifically to guselkumab and cannot be generalized to IL-23 inhibition as a class. Full article
(This article belongs to the Special Issue Advances in Systemic Sclerosis Research in Japan)
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16 pages, 963 KB  
Review
Recent Advances in Localized Scleroderma
by Toshiya Takahashi, Takehiro Takahashi and Yoshihide Asano
Sclerosis 2025, 3(4), 40; https://doi.org/10.3390/sclerosis3040040 - 2 Dec 2025
Cited by 1 | Viewed by 3179
Abstract
Localized scleroderma (LSc), or morphea, is an autoimmune connective tissue disease causing inflammation and fibrosis of the skin and underlying tissues. While distinct from systemic sclerosis, its clinical presentation is highly diverse. This review summarizes recent advances in the understanding and management of [...] Read more.
Localized scleroderma (LSc), or morphea, is an autoimmune connective tissue disease causing inflammation and fibrosis of the skin and underlying tissues. While distinct from systemic sclerosis, its clinical presentation is highly diverse. This review summarizes recent advances in the understanding and management of LSc. Pathophysiological insights have evolved significantly; the somatic mosaicism hypothesis is now supported by the observation of all six of Happle’s classic lesion patterns in LSc. Furthermore, recent single-cell RNA sequencing has elucidated key cellular mechanisms, revealing an IFN-γ-driven pro-fibrotic crosstalk between T cells, dendritic cells, and specific inflammatory fibroblast subpopulations. The discovery of a rare monogenic form of LSc caused by a STAT4 gain-of-function mutation provides a powerful human model, solidifying the critical role of the JAK-STAT pathway. Clinically, LSc is classified into subtypes such as circumscribed, linear, and generalized morphea. Extracutaneous manifestations are common, particularly in juvenile LSc, and are associated with higher disease activity and reduced quality of life, necessitating a multidisciplinary approach. Management is becoming standardized, with methotrexate as the first-line systemic therapy for severe disease. For refractory cases, targeted treatments including abatacept, tocilizumab, and JAK inhibitors are emerging as promising options. In addition, reconstructive therapies like autologous fat grafting are crucial for managing atrophic sequelae. These recent advances are paving the way for more effective, targeted therapies to improve outcomes for patients with this complex disease. Full article
(This article belongs to the Special Issue Advances in Systemic Sclerosis Research in Japan)
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