1. Systemic Sclerosis with Multiple Pulmonary Manifestations - PMC
Systemic sclerosis (SSc) is a chronic autoimmune multisystem disorder characterized by endothelial dysfunction and fibroblast dysfunction, which results in ...
Systemic sclerosis (SSc) is a chronic autoimmune multisystem disorder characterized by endothelial dysfunction and fibroblast dysfunction, which results in progressive fibrosis of the skin and internal organs more frequently the lungs and gastro intestinal ...
2. Systemic Sclerosis (Scleroderma) - StatPearls - NCBI Bookshelf
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Systemic sclerosis, also known as scleroderma, is a rare connective tissue disorder with an unknown and complex pathogenesis. Scleroderma can be divided into 2 primary forms—localized scleroderma (including morphea, linear scleroderma, and scleroderma en coup de sabre) and systemic sclerosis. Systemic sclerosis can be further classified as limited systemic sclerosis (formerly known as CREST syndrome, characterized by calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) or diffuse systemic sclerosis based on clinical and serological criteria. Although significant progress has been made in understanding the pathophysiology of scleroderma over the past centuries, the disease continues to pose significant morbidity and mortality in patients.[1][2]
3. Interstitial lung disease associated with systemic sclerosis (SSc-ILD)
Jan 18, 2019 · SSc is initiated by microvascular injury, inducing inflammation, an autoimmune response, and fibroblast activation and differentiation.
Systemic sclerosis (SSc) is a rare connective tissue disease with a heterogeneous clinical course. Interstitial lung disease (ILD) is a common manifestation of SSc and a leading cause of death. All patients newly diagnosed with SSc should receive a comprehensive clinical evaluation, including assessment of respiratory symptoms, a high-resolution computed tomography (HRCT) scan of the chest, and pulmonary function tests. ILD can develop in any patient with SSc, including those with pulmonary hypertension, but the risk is increased in those with diffuse (rather than limited) cutaneous SSc, those with anti-Scl-70/anti-topoisomerase I antibody, and in the absence of anti-centromere antibody. While it can occur at any time, the risk of developing ILD is greatest early in the course of SSc, so patients should be monitored closely in the first few years after diagnosis. An increased extent of lung fibrosis on HRCT and a low forced vital capacity (FVC) are predictors of early mortality. While not all patients will require treatment, current approaches to the treatment of progressive SSc-ILD focus on immunosuppressant therapies, including cyclophosphamide and mycophenolate mofetil. In patients with severe and/or rapidly progressive disease, both haematopoietic stem cell transplantation (HSCT) and lung transplantation have been successfully used. A number of medications, including the two drugs approved for the treatment of idiopathic pulmonary fibrosis (IPF), are under active investig...
4. The Molecular Mechanisms of Systemic Sclerosis-Associated ...
Feb 3, 2023 · Systemic sclerosis (SSc), or scleroderma, is an autoimmune connective tissue disease ... culminating in tissue repair and wound healing [100].
Systemic sclerosis (SSc), also known as scleroderma, is an autoimmune disorder that affects the connective tissues and has the highest mortality rate among the rheumatic diseases. One of the hallmarks of SSc is fibrosis, which may develop systemically, affecting the skin and virtually any visceral organ in the body. Fibrosis of the lungs leads to interstitial lung disease (ILD), which is currently the leading cause of death in SSc. The identification of effective treatments to stop or reverse lung fibrosis has been the main challenge in reducing SSc mortality and improving patient outcomes and quality of life. Thus, understanding the molecular mechanisms, altered pathways, and their potential interactions in SSc lung fibrosis is key to developing potential therapies. In this review, we discuss the diverse molecular mechanisms involved in SSc-related lung fibrosis to provide insights into the altered homeostasis state inherent to this fatal disease complication.
5. Fibrosis in systemic sclerosis: common and unique pathobiology
Jun 6, 2012 · Systemic sclerosis (SSc) is serious chronic fibrosing disease with high mortality without any effective therapy. Progressive fibrosis in the ...
Fibrosis in systemic sclerosis (SSc), a complex polygenic disease associated with autoimmunity and proliferative/obliterative vasculopathy, shares pathobiologic features in common with other fibrosing illnesses, but also has distinguishing characteristics. Fibroblast activation induced by transforming growth factor-β (TGF-β), Wnts and innate immune receptors, along with oxidative stress and reactive oxygen species (ROS) are implicated in pathogenesis. On the other hand, the roles of endothelial-mesenchymal differentiation and bone marrow-derived fibrocytes remain to be established. Fibrotic responses are modulated by transcriptional activators and cofactors, epigenetic factors, and microRNAs that can amplify or inhibit ligand-induced signaling. The nuclear orphan receptor PPAR-γ appears to be important in governing the duration and intensity of fibroblast activation and mesenchymal progenitor cell differentiation, and defects in PPAR-γ expression or function in SSc may underlie the uncontrolled progression of fibrosis. Identifying the perturbations in signaling pathways and cellular differentiation programs responsible for tissue damage and fibrosis in SSc allows their selective targeting using novel compounds, or by innovative uses of already-approved drugs (drug repurposing).
6. What is scleroderma? | Action for Pulmonary Fibrosis
Systemic sclerosis is an autoimmune condition. It is rare condition and more commonly affects women. It usually starts between ages 25-55 years but has been ...
Systemic sclerosis is an autoimmune condition. It is rare condition and more commonly affects women. It usually starts between ages 25-55 years but has been reported in children and older people.
7. first report from the EULAR Scleroderma Trials And
Systemic sclerosis in Europe: first report from the EULAR Scleroderma ... This was the culmina- tion of 18 months' intense activity by many people ...
8. Systemic Sclerosis: Highlighting Respiratory Complications and ...
Aug 18, 2021 · Systemic sclerosis (SSc) is an autoimmune disease that leads the patient to have a diverse clinical presentation encompassing several systems ...
Systemic sclerosis (SSc) is an autoimmune disease that leads the patient to have a diverse clinical presentation encompassing several systems and a worse prognosis, mainly when complications arise. Most SSc-related deaths are caused by pulmonary hypertension (PH) and interstitial lung disease (ILD). This article focuses on pulmonary artery hypertension (PAH) and ILD as pulmonary consequences of SSc. We examined the grave effects regarding SSc's respiratory complications, which are concealed by the disease's clinical heterogeneity. In this article, we briefly reviewed the discussion of clinical features and management and the mortality associated with the sequelae. We further addressed the benefits and significance of screening for the disease and associated respiratory complications in SSc patients in this study.
9. [PDF] TGF‐β as a driver of fibrosis: physiological roles and therapeutic ...
May 19, 2021 · Additionally, cirrhosis, the culmina- tion of fibrotic remodeling of the liver and a major risk factor for hepatocellular carcinoma, is the ...
10. [PDF] INSTITUTIONAL PROFILE FY 2010-11
... culmina- tion of a long process. A campus observer in the mid-1960s, wrote ... Sclerosis Genetics Group. • UCSF Pain Management Center. UCSF Medical ...
