Fibrosis Research in Systemic Sclerosis (SSc)

Deadline for manuscript submissions: 30 April 2025.

Guest Editors (2)

Carol M.  Artlett
Prof. Carol M. Artlett 
Department of Microbiology & Immunology, College of Medicine, Drexel University, Philadelphia, 19129, PA, USA
Interests: Inflammasome Signaling; IL-1 Mediated Fibrosis; Collagen Export from the Endoplasmic Reticulum; miR-155; Small Molecule Therapeutic Development
Clara  Dees
Dr. Clara Dees 
Friedrich-Alexander-University (FAU) Erlangen-Nürnberg, Department of Internal Medicine 3—Rheumatology and Immunology, Universitätsklinikum Erlangen, Erlangen, Germany
Interests: Cell Signaling; Signal Transduction; Extracellular Matrix Biology; Cell Culture; Rheumatology; Fibrosis; Fibroblast; Systemic Sclerosis

Special Issue Information

Systemic sclerosis (SSc, scleroderma), is an autoimmune disease characterized by inflammation with vascular abnormalities. A major hallmark of SSc is the excessive accumulation of extracellular matrix (ECM) in the skin and visceral organs, leading to morbidity and mortality. The ECM products are released from fibroblasts activated by cytokines, growth factors, and/or epigenetic changes in the genome. Currently, there are no truly effective treatment options for fibrosis.

In this special issue of Fibrosis, we invite you to contribute original research articles, reviews, case reports, or expert perspectives/opinions on all aspects related to "Fibrosis Research in Systemic Sclerosis (SSc)".

Relevant topics related to SSc fibrosis might be:
  • Cellular and molecular mechanisms governing fibroblast activation
  • Recent advances in experimental models of fibrosis (in vitro or in vivo models)
  • SSc-related organ fibrosis (e.g. lung, gastrointestinal, cardiac, renal, skin)
  • Genetics/epigenetics mechanisms driving fibrosis in SSc
  • Translational studies or therapies targeting fibrogenesis

Published Papers (1 Papers)

Open Access

Review

22 May 2025

NLRP3 Inflammasome and IL-11 in Systemic Sclerosis Pulmonary Fibroblasts

Systemic sclerosis (SSc) is an autoimmune disease characterized by widespread fibrosis affecting multiple organ systems. There is clinical heterogeneity among patients with SSc in terms of the organs affected. However, the pathophysiology of the disease remains elusive. The NLRP3 inflammasome is upregulated in SSc and exerts its fibrotic effects through activation of caspase-1, which in turn activates a fibrotic signaling cascade, resulting in increased collagen deposition and myofibroblast transition. Recently, IL-11 has been shown to be elevated in disease and has been shown to participate in downstream signaling via the NLRP3 inflammasome. A significant number of patients with SSc will develop pulmonary involvement, termed interstitial lung disease (SSc-ILD). Though this type of pulmonary involvement is distinct from other types of pulmonary fibrosis (such as idiopathic pulmonary fibrosis), it may be a valuable model to study mechanisms of fibrosis that could apply to other fibrotic diseases. Here, we discuss recent advances in understanding the mechanisms of the NLRP3 inflammasome and IL-11 in SSc pulmonary fibroblasts. We tie together some of the recent findings, such as senescence, the unfolded protein response, and reactive oxygen species, that contribute to fibrotic pathology via modulating NLRP3 activation, possibly leading to IL-11 expression.

Caya M.McFalls*
Carol M.Artlett
Fibrosis
2025,
3
(2), 10006; 
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