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Systemic sclerosis (SSc) is a rare autoimmune connective tissue disorder characterized by massive fibrosis, vascular damage, and immune imbalance. Advances in rheumatology and immunology over the past two decades have led to a redefinition of systemic sclerosis, shifting from its initial perception as primarily a "hyperfibrotic" state towards a recognition of systemic sclerosis as an immune-mediated disease. Consequently, the search for genetic markers has transitioned from focusing on fibrotic mechanisms to exploring immune regulatory pathways. Immunogenetics, an emerging field at the intersection of immunology, molecular biology, and genetics has provided valuable insights into inherited factors that influence immunity. Data from genetic studies conducted thus far indicate that alterations in genetic messages can significantly impact disease risk and progression. While certain genetic variations may confer protective effects, others may exacerbate disease susceptibility. This paper presents a comprehensive review of the most relevant genetic changes that influence both the risk and course of systemic sclerosis. Special emphasis is placed on factors regulating the immune response, recognizing their pivotal role in the pathogenesis of the disease. [ABSTRACT FROM AUTHOR]

Peripheral neuropathy (PN) is a relatively common comorbidity of connective tissue diseases. It often represents a neurological presentation of systemic diseases, while complications of systemic treatment of systemic connective tissue diseases cause some cases. This paper presents the current division and contemporary diagnostic and therapeutic strategies focusing on the most common systemic rheumatic diseases. [ABSTRACT FROM AUTHOR]

Systemic sclerosis (SSc) is a systemic connective tissue disease characterised by disseminated microangiopathy and excessively immune-stimulated fibroblast activity, leading to fibrosis of the skin and internal organs. In the literature, the first report of the disease dates back to 1753 and is attributed to the physician Carlo Curzio of Naples, who described the case of a 17-year-old girl who developed induration of the skin all over her body. The disease is a rare condition, with an estimated 1 in 10 000 people in Poland suffering from SSC. Women predominate among patients, with a 3--4-fold advantage over men. The disease typically has its onset between the ages of 30 and 50. Early detection and treatment of organ complications are key to improving quality of life and reducing mortality in SSc patients. Given the considerable variability of the clinical course, it seems justified to take an individual approach to patients and cooperate with multiple specialists, both at the stage of diagnosis and at the stage of treatment. Treatment is based on what is known as an organ-specific strategy and consists of tailoring pharmacotherapy to the clinical presentation, disease period and organ complications. In addition to pharmacology, the treatment of patients should also include education of the patient and their family, and -- if necessary -- surgical treatment or other necessary interventions. [ABSTRACT FROM AUTHOR]

Systemic sclerosis (SSc) is a systemic connective tissue disease marked by diffuse microangiopathy and excessively immune-stimulated fibroblast activity, leading to fibrosis of the skin and internal organs. In the literature, the first report of the disease dates back to 1753 and is attributed to the physician Carlo Curzio of Naples, who described the case of a 17-year-old girl who developed sclerosis of the skin all over her body. The disease is a rare condition. It is estimated that 1 in 10 000 people in Poland suffer from SSc. Women predominate among the patients, with a 3-4-fold prevalence compared to men. Typically, the disease has its onset between 30 and 50 years of age. Early detection and treatment of organ complications are key to improving quality of life and reducing mortality in patients with SSc. Given the significant variability in the clinical course, an individualised approach to patients and multidisciplinary collaboration appear to be justified, both in the diagnostic and treatment phases. The treatment is based on the organ-specific therapeutic strategy, which involves tailoring the pharmacotherapy to the clinical presentation, disease stage, and organ complications. Treatment of patients should include, in addition to pharmacology, education of the patient and family and, if necessary, surgical treatment or other necessary interventions. [ABSTRACT FROM AUTHOR]

Systemic sclerosis (SSc, scleroderma) is a chronic systemic connective tissue disease with a complex pathogenesis that is still not fully understood, in the course of which attention is increasingly drawn to the dynamic, sequential pathogenetic mechanisms according to disease stage. An increasing understanding of the diversity of mechanisms underlying this disease, as well as the prevalence of certain pathogenetic elements that depend i.a. on disease stage, will enable more effective therapeutic interventions in the future. Systemic sclerosis can thus be seen as a complex process, where the main players are immune cells, endothelial cells and fibroblasts, and the focal point is probably impaired function and subsequent damage to endothelial cells. Systemic sclerosis is also the final stage of a certain continuum of events, starting with a state of susceptibility to the development of the disease (dependent on genetic conditions and environmental influences), followed by disruption of homeostasis and initiation of pathological processes (e.g. as a result of viral infections), progression of pathological responses (inflammation, endothelial damage, fibrosis) and consequently organ damage. According to most authors, the key event and focal point of the cascade of phenomena is endothelial cell damage, and the mechanisms that lead to this damage are related to the activation of the immune system. There is growing acceptance of the thesis of an autoimmune origin of the disease involving mechanisms of innate and acquired immunity, both cellular and humoral. [ABSTRACT FROM AUTHOR]

The authors present the clinical picture of systemic sclerosis including organ and system involvement. [ABSTRACT FROM AUTHOR]

The article discusses the establishment of the "Rheumatology Forum" as a common journal for the Central European Conference in Rheumatology (CECR). The journal aims to facilitate the exchange of ideas and independent publication among European rheumatology societies. The current issue focuses on systemic sclerosis, with papers discussing treatment strategies and the use of autologous stem cell transplantation. Other topics covered include physiotherapy for rheumatoid diseases, medical standards for treating psoriatic arthritis, physical therapy for ankylosing spondylitis, cardiovascular problems in patients with axial spondyloarthropathies, and new diagnostic procedures in rheumatology. The article also mentions the VEXAS syndrome and its clinical presentation. [Extracted from the article]

This article from the Rheumatology Forum discusses the complexities of investigations in rheumatology. It highlights the multiple comorbidities that patients with rheumatoid arthritis and autoimmune disorders often experience, particularly the increased risk of cardiovascular complications. The article also addresses the role of metabolic syndrome in atherosclerosis development and progression in patients with inflammatory arthropathies. Additionally, the article includes case reports on challenging conditions such as systemic sclerosis, pyoderma gangrenosum, eosinophilic fasciitis, and inclusion body myositis, providing valuable insights for rheumatologists. The issue also features a profile of Jan van Breemen, a pioneer in rheumatology collaboration. [Extracted from the article]

Patients with autoimmune diseases are a particularly vulnerable group, prone to developing infectious complications. At the same time, due to the immunosuppressive treatment of these patients, the use of vaccination may raise some concerns as to its effectiveness and, above all, its safety. This article presents the current recommendations of scientific societies indicating the efficacy, safety and, in particular, the necessity of immunization in all patients with inflammatory rheumatic diseases. Special emphasis in this study is placed on the efficacy and safety of vaccination against COVID-19. [ABSTRACT FROM AUTHOR]

Background: Rheumatoid arthritis (RA) represents the most frequent form of inflammatory arthritis, affecting approximately 1% of the population worldwide. The introduction of novel therapeutic strategies targeting proinflammatory cytokines (TNF-α and interleukin-6) revolutionized the treatment of RA. This kind of treatment, although effective in a substantial portion of patients, may potentially cause many side effects. Among them, cardiovascular safety is one of the main concerns., Objectives: In the present study, we investigated the impact of treatment with anti-TNF-α and anti-IL-6 agents on heart function and levels of heart function biomarkers., Methods: To measure this, we used cardiac function biomarkers, such as NT-pro Brain Natriuretic Peptide, mid regional pro-Atrial Natriuretic Peptide, Galectin-3, and Heart-Type Fatty Acid-Binding Protein and compared them to patients treated with methotrexate as well as healthy controls., Results: Patients treated with biologics were characterized by low disease activity or were in remission. The disease activity in these groups was significantly lower than in the methotrexate group. All patients recruited for the study were characterized by normal heart function measured using echocardiography (EF>50%). With the exception of MR-proANP between tocilizumab and adalimumab (median: 1.01 vs. 0.49 nmol/L, p<0.05), we failed to observe any significant differences in biomarkers levels between groups treated with biologics. Contrary to this, patients on MTX showed higher NT-proBNP levels compared to adalimumab and healthy controls (p<0.05 for both). Striking differences have been shown in regard to H-FABP. The levels of these biomarkers were elevated in all biologics and the methotrexate group compared to healthy controls., Conclusion: As this biomarker reflects potential heart injury, we suggest that heart damage proceeds in a continuous manner in RA patients despite effective treatment and attainment of remission/low disease activity. This finding, however, should be verified in a larger cohort of RA patients to ascertain if the routine assessment of H-FABP may be useful for the detection of patients with RA who are at risk of development of heart damage., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)