Scleroderma and scleroderma-like syndromes

Front Immunol. 2024 Jun 3:15:1351675. doi: 10.3389/fimmu.2024.1351675. eCollection 2024.

Abstract

Systemic sclerosis is a systemic connective tissue disease whose main pathophysiological mechanism is a progressive fibrosis of internal organs and skin leading to thickening and induration. Blood vessels may also be involved. However, systemic scleroderma is not the only disease causing cutaneous sclerosis. There is a group of diseases that mimic scleroderma in their clinical presentation - these are scleroderma-like syndromes. A distinction can be made between syndromes of inflammatory/autoimmune, genetic, metabolic, toxic, drug-induced, occupational, paraneoplastic and syndromes caused by deposition disorders. In the following paper, we have reviewed the literature on scleroderma-like syndromes. We have outlined the factors predisposing to the development of each disease, its pathogenesis, clinical presentation, diagnostic and treatment process and the differences between each syndrome and systemic scleroderma.

Keywords: genetic; morphea; scleroderma; scleroderma-like syndromes; syndromes of inflammatory/autoimmune; systemic sclerosis; toxic.

Publication types

  • Review

MeSH terms

  • Diagnosis, Differential
  • Humans
  • Scleroderma, Systemic*
  • Skin / immunology
  • Skin / pathology
  • Syndrome

Grants and funding

The author(s) declare financial support was received for the research, authorship, and/or publication of this article. “Double-blind randomised clinical trial comparing the efficacy of 52-week therapy with pirfenidone versus nintedanib versus standard therapy with mycophenolate mofetil in patients with interstitial lung disease associated with systemic connective tissue disease (CTD-ILD) and hypersensitivity disease (HP)”. 2022/ ABM/ 03/00009, nr EudraCT 2023-507056-77-00.