A Comprehensive Review of Systemic Sclerosis: Diagnosis, Clinical Features and Management Strategies
Introduction Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis of the skin and internal organs, along with vasculopathy. It is associated with significant morbidity and mortality, particularly due to pulmonary and renal complications. Early diagnosis is challenging...
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Kazimierz Wielki University
2025-02-01
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| Series: | Journal of Education, Health and Sport |
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| Online Access: | https://apcz.umk.pl/JEHS/article/view/57679 |
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| author | Agnieszka Borowiec Kinga Borowiec Paulina Kwaśniewska Julia Biernikiewicz Milena Biernikiewicz Anna Wilewska Bartosz Pomirski Agata Pomirska Konstanty Alabrudziński Miszela Kałachurska |
| author_facet | Agnieszka Borowiec Kinga Borowiec Paulina Kwaśniewska Julia Biernikiewicz Milena Biernikiewicz Anna Wilewska Bartosz Pomirski Agata Pomirska Konstanty Alabrudziński Miszela Kałachurska |
| author_sort | Agnieszka Borowiec |
| collection | DOAJ |
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Introduction
Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis of the skin and internal organs, along with vasculopathy. It is associated with significant morbidity and mortality, particularly due to pulmonary and renal complications. Early diagnosis is challenging, but crucial, as severe internal organ involvement can occur within three years of disease onset. SSc is categorized into subsets, such as diffuse and limited cutaneous forms with varying degrees of skin and organ involvement. Specific autoantibodies are critical for diagnosing and predicting disease severity and understanding these serological profiles aids in personalized treatment decisions. Management focuses on addressing specific manifestations like Raynaud’s phenomenon, skin fibrosis, pulmonary involvement, and gastrointestinal issues with treatment strategies tailored to the disease’s stage and organ involvement. Despite advances in treatment, SSc remains a challenging disease requiring ongoing vigilance for early detection and intervention.
Purpose
The purpose of this article is to provide a comprehensive overview of systemic sclerosis, focusing on its diagnostic criteria, clinical features and management strategies. It aimsto highlight the importance of early detection, particularly in the context of skin and organ involvement and the role of serological markers in guiding diagnosis and treatment. Ultimately, the goal is to improve awareness and understanding of SSc among healthcare professionals, particularly dermatologists, to enhance early diagnosis and optimize patient outcomes.
Materials and Methods
Our review is based on an analysis of material collected in 'Pubmed', 'Google Scholar' and other scientific articles using the keywords: systemic sclerosis, antinuclear antibodies, Raynaud’s phenomenon, pulmonary arterial hypertension.
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| format | Article |
| id | doaj-art-e53f61cc788a4f14af23dc068d9ba1e6 |
| institution | DOAJ |
| issn | 2391-8306 |
| language | English |
| publishDate | 2025-02-01 |
| publisher | Kazimierz Wielki University |
| record_format | Article |
| series | Journal of Education, Health and Sport |
| spelling | doaj-art-e53f61cc788a4f14af23dc068d9ba1e62025-08-20T03:11:47ZengKazimierz Wielki UniversityJournal of Education, Health and Sport2391-83062025-02-017810.12775/JEHS.2025.78.57679A Comprehensive Review of Systemic Sclerosis: Diagnosis, Clinical Features and Management StrategiesAgnieszka Borowiec0https://orcid.org/0000-0002-1428-170XKinga Borowiec1https://orcid.org/0009-0000-5546-9787Paulina Kwaśniewska2https://orcid.org/0009-0009-4677-3387Julia Biernikiewicz3https://orcid.org/0009-0004-1192-9365Milena Biernikiewicz4https://orcid.org/0009-0006-7288-6965Anna Wilewska5https://orcid.org/0009-0001-5136-4598Bartosz Pomirski6https://orcid.org/0009-0004-4868-0073Agata Pomirska7https://orcid.org/0009-0009-5367-7123Konstanty Alabrudziński8https://orcid.org/0009-0008-4729-0937Miszela Kałachurska9https://orcid.org/0009-0006-0687-638XThe Regional Specialist Hospital in Biala Podlaska, Terebelska 57-65 21-500 Biala Podlaska, PolandFaculty of Medicine, Medical University of Warsaw Żwirki i Wigury 61, 02-091 Warsaw, PolandFaculty of Medicine, Medical University of Warsaw Żwirki i Wigury 61, 02-091 Warsaw, PolandFaculty of Medicine, Medical University of Warsaw Żwirki i Wigury 61, 02-091 Warsaw, PolandFaculty of Medicine, Wroclaw Medical University Wybrzeże Ludwika Pasteura 1, 50-367 Wroclaw, PolandFaculty of Medicine, Medical University of Warsaw Żwirki i Wigury 61, 02-091 Warsaw, PolandFaculty of Medicine, Medical University of Warsaw Żwirki i Wigury 61, 02-091 Warsaw, PolandFaculty of Medicine, Medical University of Warsaw, Żwirki i Wigury 61, 02-091 Warsaw, PolandSchool of Medicine Collegium Medicum University of Warmia and Mazury in Olsztyn, Oczapowskiego 2, 10-719 Olsztyn, PolandFaculty of Medicine, Pomeranian Medical University of Szczecin, Rybacka 1, 70-204 Szczecin Introduction Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis of the skin and internal organs, along with vasculopathy. It is associated with significant morbidity and mortality, particularly due to pulmonary and renal complications. Early diagnosis is challenging, but crucial, as severe internal organ involvement can occur within three years of disease onset. SSc is categorized into subsets, such as diffuse and limited cutaneous forms with varying degrees of skin and organ involvement. Specific autoantibodies are critical for diagnosing and predicting disease severity and understanding these serological profiles aids in personalized treatment decisions. Management focuses on addressing specific manifestations like Raynaud’s phenomenon, skin fibrosis, pulmonary involvement, and gastrointestinal issues with treatment strategies tailored to the disease’s stage and organ involvement. Despite advances in treatment, SSc remains a challenging disease requiring ongoing vigilance for early detection and intervention. Purpose The purpose of this article is to provide a comprehensive overview of systemic sclerosis, focusing on its diagnostic criteria, clinical features and management strategies. It aimsto highlight the importance of early detection, particularly in the context of skin and organ involvement and the role of serological markers in guiding diagnosis and treatment. Ultimately, the goal is to improve awareness and understanding of SSc among healthcare professionals, particularly dermatologists, to enhance early diagnosis and optimize patient outcomes. Materials and Methods Our review is based on an analysis of material collected in 'Pubmed', 'Google Scholar' and other scientific articles using the keywords: systemic sclerosis, antinuclear antibodies, Raynaud’s phenomenon, pulmonary arterial hypertension. https://apcz.umk.pl/JEHS/article/view/57679systemic sclerosisantinuclear antibodiesRaynaud’s phenomenonpulmonary arterial hypertension |
| spellingShingle | Agnieszka Borowiec Kinga Borowiec Paulina Kwaśniewska Julia Biernikiewicz Milena Biernikiewicz Anna Wilewska Bartosz Pomirski Agata Pomirska Konstanty Alabrudziński Miszela Kałachurska A Comprehensive Review of Systemic Sclerosis: Diagnosis, Clinical Features and Management Strategies Journal of Education, Health and Sport systemic sclerosis antinuclear antibodies Raynaud’s phenomenon pulmonary arterial hypertension |
| title | A Comprehensive Review of Systemic Sclerosis: Diagnosis, Clinical Features and Management Strategies |
| title_full | A Comprehensive Review of Systemic Sclerosis: Diagnosis, Clinical Features and Management Strategies |
| title_fullStr | A Comprehensive Review of Systemic Sclerosis: Diagnosis, Clinical Features and Management Strategies |
| title_full_unstemmed | A Comprehensive Review of Systemic Sclerosis: Diagnosis, Clinical Features and Management Strategies |
| title_short | A Comprehensive Review of Systemic Sclerosis: Diagnosis, Clinical Features and Management Strategies |
| title_sort | comprehensive review of systemic sclerosis diagnosis clinical features and management strategies |
| topic | systemic sclerosis antinuclear antibodies Raynaud’s phenomenon pulmonary arterial hypertension |
| url | https://apcz.umk.pl/JEHS/article/view/57679 |
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