Background: Scleroderma, also referred to as systemic sclerosis, is a multifaceted autoimmune condition characterized by abnormal fibrosis and impaired vascular function. Pathologically, it encompasses the persistent presence of inflammation, abnormal collagen buildup, and restructuring of blood vessels in various organs, resulting in a wide range of clinical symptoms. This review incorporates the most recent scientific literature on scleroderma, with a particular emphasis on its pathophysiology, clinical manifestations, diagnostic approaches, and treatment options.
Methodology: A comprehensive investigation was carried out on numerous databases, such as PubMed, MEDLINE, Scopus, Web of Science, and Google Scholar, to collect pertinent studies covering diverse facets of scleroderma research.
Results: Scleroderma presents with a range of systemic manifestations, such as interstitial lung disease, gastrointestinal dysmotility, Raynaud's phenomenon, pulmonary arterial hypertension, renal complications, neurological symptoms, and cardiac abnormalities. Serological markers, such as antinuclear antibodies, anti-centromere antibodies, and anti-topoisomerase antibodies, are important for classifying diseases and predicting their outcomes.
Discussion: The precise identification of scleroderma is crucial for promptly and correctly implementing effective treatment plans. Treatment approaches aim to improve symptoms, reduce complications, and slow down the progression of the disease. An integrated approach that combines pharmacological agents, including immunosuppressants, endothelin receptor antagonists, and prostanoids, with nonpharmacological interventions such as physical and occupational therapy is essential for maximizing patient care.
Conclusion: Through the clarification of existing gaps in knowledge and identification of emerging trends, our goal is to improve the accuracy of diagnosis, enhance the effectiveness of therapeutic interventions, and ultimately enhance the overall quality of life for individuals suffering from scleroderma. Ongoing cooperation and creative research are necessary to advance the field and achieve improved patient outcomes and new therapeutic discoveries.
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http://dx.doi.org/10.1002/hsr2.2072 | DOI Listing |
J Scleroderma Relat Disord
January 2025
Division of Rheumatology, Johns Hopkins University, Baltimore, MD, USA.
Autonomic dysfunction is a common and early complication among patients with systemic sclerosis, suggesting that it may play a role in the pathogenesis of the disease and be a potential target for therapeutic interventions. Although the true prevalence of autonomic dysfunction among patients with systemic sclerosis is still unclear, it is estimated that as many as 80% of patients may be affected. Autonomic dysfunction may lead to widespread multi-organ dysfunction through its effects on the cardiovascular system, gastrointestinal tract, urinary tract, sweat and salivary glands, and pupils.
View Article and Find Full Text PDFSci Rep
January 2025
Department of Dermatology, Gunma University Graduate School of Medicine, 3-39-22, Showa, Maebashi, Gunma, 371-8511, Japan.
Systemic sclerosis (SSc) is an idiopathic systemic connective tissue disorder characterized by fibrosis of the skin and internal organs, with growing interest in the imbalance between Th17 cells and regulatory T cells (Tregs) in the disease's pathogenesis. Heligmosomoides polygyrus (Hp), a natural intestinal parasite of mice, is known to induce Tregs in the host. We aimed to investigate the effects of Hp-induced Tregs on bleomycin-induced dermal fibrosis and clarify the role of the Th17/Treg balance in SSc fibrosis.
View Article and Find Full Text PDFClin Rheumatol
January 2025
Faculty of Physical Therapy and Rehabilitation, Cardiopulmonary Department, Dokuz Eylül University, Izmir, Turkey.
Purpose: To investigate the validity and reliability of the Londrina ADL Protocol in patients with systemic sclerosis (SSc).
Methods: The study included 39 individuals with SSc and 30 healthy participants aged 18-70 years. Performance-related ADL assessment was performed with the Londrina ADL Protocol which was performed twice by the same rater and energy expenditure during the test with the Dynaport Move Monitor device.
J Genomics
January 2025
Université de Lorraine, INRAE, UMR 1136 Interactions Arbres/Microorganismes, 54280, Champenoux, France.
The earthball , an ectomycorrhizal basidiomycete belonging to the Sclerodermataceae family, serves as a significant mutualistic tree symbiont globally. Originally, two genetically sequenced strains of this genus were obtained from fruiting bodies collected under chestnut trees (). These strains were utilized to establish ectomycorrhizal roots of chestnut seedlings.
View Article and Find Full Text PDFIndian J Pathol Microbiol
January 2025
Department of Dermatology, KJ Somaiya Medical College, Mumbai, Maharashtra, India.
The dermatopathological features in morphea (localized scleroderma) and their clinicopathologic correlations are not well described in the literature. To describe dermatopathological changes of different types of morphea and to investigate the association between clinical and histopathological features. A total of 18 cases of morphea who attended our tertiary care center in the last four years were evaluated.
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