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ORIGINAL RESEARCH article

Front. Immunol.
Sec. Inflammation
Volume 15 - 2024 | doi: 10.3389/fimmu.2024.1502324
This article is part of the Research Topic Study on Immune Mechanism and Immune Intervention in Connective Tissue Diseases View all 6 articles

BMP-4 and fetuin A in systemic sclerosis patients with or without calcinosis

Provisionally accepted
Francesco Demetrio Lofaro Francesco Demetrio Lofaro 1Dilia Giuggioli Dilia Giuggioli 2*Susanna Bonacorsi Susanna Bonacorsi 1Martina Orlandi Martina Orlandi 2Amelia Spinella Amelia Spinella 2Marco De Pinto Marco De Pinto 2Ottavio Secchi Ottavio Secchi 2Clodoveo Ferri Clodoveo Ferri 3Federica Boraldi Federica Boraldi 1*
  • 1 Department of Life Sciences, University of Modena and Reggio Emilia, Modena, Emilia-Romagna, Italy
  • 2 Department of Medical and Surgical, Maternal-Infantile and Adult Sciences, University of Modena and Reggio Emilia, Modena, Lombardy, Italy
  • 3 Rheumatology Clinic ‘Madonna dello Scoglio’ Cotronei, Crotone, Italy, Department of Medical and Surgical, Maternal-Infantile and Adult Sciences, University of Modena and Reggio Emilia, Modena, Lombardy, Italy

The final, formatted version of the article will be published soon.

    Systemic sclerosis (SSc) is a connective tissue disease at the interface between inflammation and autoimmunity progressively leading to diffuse microvascular and fibrotic involvement of the skin and of multiple internal organs. Approximately, 20-40% of SSc patients suffer from cutaneous calcinosis, a debilitating manifestation due to calcium salt deposition in soft connective tissues, causing pain, ulceration, infection, and deformities, responsible of severe functional limitations. Pathomechanisms are poorly understood as well as markers/molecules capable to predict the risk of patients to develop calcinosis. An observational study was performed in 51 female patients, 25 with and 26 without calcinosis to compare clinical and laboratory parameters and to evaluate pro-and anti-calcifying circulating markers and the in vitro serum calcification potential (T50). Moreover, calcinosis samples were analyzed to characterize their mineral composition. Data demonstrate statistically significant differences in the prevalence of clinical manifestations and ACA and Scl70 autoantibodies in SSc patient with calcinosis compared to those without calcinosis. In SSc patients with calcinosis, serum levels of BMP-4 are higher, fetuin A might be regarded as a potential circulating prognostic marker and a negative correlation was observed between T50 and the global score of clinical manifestations, suggesting a potential predictive role of pro-and anti-calcifying molecules in SSc patients. Furthermore, calcinosis samples were characterized by the co-existence of phosphate and carbonate minerals with different stability and solubility. Further investigations on circulating markers in larger patient cohorts, especially at the early stages and throughout the natural course of the disease, may clarify their pathogenetic role in the SSc-related cutaneous calcinosis.

    Keywords: Bone morphogenic protein, calcification, Fetuin A, scleroderma, Serum calcification propensity

    Received: 26 Sep 2024; Accepted: 14 Nov 2024.

    Copyright: © 2024 Lofaro, Giuggioli, Bonacorsi, Orlandi, Spinella, De Pinto, Secchi, Ferri and Boraldi. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence:
    Dilia Giuggioli, Department of Medical and Surgical, Maternal-Infantile and Adult Sciences, University of Modena and Reggio Emilia, Modena, 41121, Lombardy, Italy
    Federica Boraldi, Department of Life Sciences, University of Modena and Reggio Emilia, Modena, Emilia-Romagna, Italy

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