Advances in Systemic Sclerosis Research in Japan

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

Deadline for manuscript submissions: 10 May 2026 | Viewed by 602

Special Issue Editor


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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
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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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Published Papers (1 paper)

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Review

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
Viewed by 458
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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