Pub Date : 2019-07-26DOI: 10.5772/INTECHOPEN.88286
S. Oreská, M. Tomčík
Gastrointestinal tract (GIT) involvement is the most common internal organ manifestation and is present in up to 90% of patients with systemic sclerosis (SSc). Clinical manifestations can differ according to the part of the GIT affected, disease course and symptoms. A majority of the symptoms are caused by GIT dysmotility. Up to 8% of SSc patients develop several GIT symptoms, which increase the mortality. Although GIT involvement is rarely the direct cause of death, it can lead to several comorbidities including malnutrition and negative alterations of body composition. These factors have a negative impact on quality of life and increase the mortality. To date, the treatment is rather symptomatic. The pathogenesis of GIT involvement in SSc still remains to be clarified to improve the treatment approaches including intravenous immunoglobulins and microRNA interventions.
{"title":"Gastrointestinal Involvement in Systemic Sclerosis: Overview, Neglected Aspects, Malnutrition, Body Composition and Management","authors":"S. Oreská, M. Tomčík","doi":"10.5772/INTECHOPEN.88286","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.88286","url":null,"abstract":"Gastrointestinal tract (GIT) involvement is the most common internal organ manifestation and is present in up to 90% of patients with systemic sclerosis (SSc). Clinical manifestations can differ according to the part of the GIT affected, disease course and symptoms. A majority of the symptoms are caused by GIT dysmotility. Up to 8% of SSc patients develop several GIT symptoms, which increase the mortality. Although GIT involvement is rarely the direct cause of death, it can lead to several comorbidities including malnutrition and negative alterations of body composition. These factors have a negative impact on quality of life and increase the mortality. To date, the treatment is rather symptomatic. The pathogenesis of GIT involvement in SSc still remains to be clarified to improve the treatment approaches including intravenous immunoglobulins and microRNA interventions.","PeriodicalId":127513,"journal":{"name":"New Insights into Systemic Sclerosis [Working Title]","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126120296","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-07-09DOI: 10.5772/INTECHOPEN.87187
Tomáš Soukup, Jan Toms, Sabina Oreska, Eva Honsova, Roman Safranek
Scleroderma renal crisis (SRC) is classical renal disease in systemic sclerosis (SSc). SRC is a relatively rare manifestation, approximately in 5% of patients. In terms of severity, manifestation in the form of SRC is the most common cause of acute organ failure. In SSc patients, SRC is defined as a new onset of accelerated arterial hypertension and rapidly progressive anuric or oliguric renal failure. SRC is primarily vascular injury with increased activity of the renin-angiotensin activity. These events lead to release or activation of cytokines and growth factors that result in the typical proliferative vascular lesions. Successful approach is routine use of angiotensin-converting enzyme inhibitors in the treatment of SRC (except prevention) and other advances in renal replacement therapy in SSc management. It is crucial to detect manifestations of SRC early and to manage appropriately in collaboration with intensive care medicine, cardiologists, and nephrologists. In contrast to SRC, clinical presentation of interstitial renal disease is poor, often without evidence of renal abnormality. Interestingly, other renal manifestations are glomerulonephritis and vasculitis. These manifestations are associated with overlapping mechanisms. The objective of this chapter is to focus on actual knowl-edge about the renal involvement in SSc and current treatment principles and possibilities.
{"title":"Renal Involvement in Systemic Sclerosis","authors":"Tomáš Soukup, Jan Toms, Sabina Oreska, Eva Honsova, Roman Safranek","doi":"10.5772/INTECHOPEN.87187","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.87187","url":null,"abstract":"Scleroderma renal crisis (SRC) is classical renal disease in systemic sclerosis (SSc). SRC is a relatively rare manifestation, approximately in 5% of patients. In terms of severity, manifestation in the form of SRC is the most common cause of acute organ failure. In SSc patients, SRC is defined as a new onset of accelerated arterial hypertension and rapidly progressive anuric or oliguric renal failure. SRC is primarily vascular injury with increased activity of the renin-angiotensin activity. These events lead to release or activation of cytokines and growth factors that result in the typical proliferative vascular lesions. Successful approach is routine use of angiotensin-converting enzyme inhibitors in the treatment of SRC (except prevention) and other advances in renal replacement therapy in SSc management. It is crucial to detect manifestations of SRC early and to manage appropriately in collaboration with intensive care medicine, cardiologists, and nephrologists. In contrast to SRC, clinical presentation of interstitial renal disease is poor, often without evidence of renal abnormality. Interestingly, other renal manifestations are glomerulonephritis and vasculitis. These manifestations are associated with overlapping mechanisms. The objective of this chapter is to focus on actual knowl-edge about the renal involvement in SSc and current treatment principles and possibilities.","PeriodicalId":127513,"journal":{"name":"New Insights into Systemic Sclerosis [Working Title]","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121623602","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-05-15DOI: 10.5772/INTECHOPEN.86217
J. Swisher, S. Kailash
Pulmonary hypertension is a well-known complication of systemic sclerosis. Patients with systemic sclerosis may develop a pulmonary arteriopathy characterized by vascular remodeling, increased pulmonary vascular resistance, and right ventricular failure. Pulmonary hypertension may also arise in systemic sclerosis as a consequence of interstitial lung disease or left ventricular dysfunction. Vascular remodeling is more prevalent than other forms of pulmonary hypertension in systemic sclerosis. The pathogenesis of pulmonary vascular remodeling in this disease state is not completely understood; however, there is evidence of a complex process involving genetic susceptibility, risk factors, vascular injury, and endothelial dysfunction. In those patients with pulmonary arterial hypertension, survival prognosis is extremely poor if the diagnosis is delayed or goes undetected and untreated. In recent years, a number of disease-targeted therapies have been developed that improve functional capacity, hemodynamics, and survival. Early detection and treatment with one or more targeted therapies are essential to improving survival when systemic sclerosis is complicated by pulmonary arterial hypertension.
{"title":"Advances in Management of Pulmonary Hypertension Associated with Systemic Sclerosis","authors":"J. Swisher, S. Kailash","doi":"10.5772/INTECHOPEN.86217","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.86217","url":null,"abstract":"Pulmonary hypertension is a well-known complication of systemic sclerosis. Patients with systemic sclerosis may develop a pulmonary arteriopathy characterized by vascular remodeling, increased pulmonary vascular resistance, and right ventricular failure. Pulmonary hypertension may also arise in systemic sclerosis as a consequence of interstitial lung disease or left ventricular dysfunction. Vascular remodeling is more prevalent than other forms of pulmonary hypertension in systemic sclerosis. The pathogenesis of pulmonary vascular remodeling in this disease state is not completely understood; however, there is evidence of a complex process involving genetic susceptibility, risk factors, vascular injury, and endothelial dysfunction. In those patients with pulmonary arterial hypertension, survival prognosis is extremely poor if the diagnosis is delayed or goes undetected and untreated. In recent years, a number of disease-targeted therapies have been developed that improve functional capacity, hemodynamics, and survival. Early detection and treatment with one or more targeted therapies are essential to improving survival when systemic sclerosis is complicated by pulmonary arterial hypertension.","PeriodicalId":127513,"journal":{"name":"New Insights into Systemic Sclerosis [Working Title]","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129047678","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-05-10DOI: 10.5772/INTECHOPEN.86220
M. Špiritović
Systemic sclerosis (SSc) is a rare, chronic connective tissue disease with characteristic fibrosis of the skin, musculoskeletal system, and internal organs. It is a heterogeneous and polymorphic disorder, in which fatigue, sleep disturbances, stiffness, and joint pain are among the most significant clinical symptoms. However, the presence of stiffening and thickening of the skin usually has a negative impact on the appearance of these patients, and the degree of self-dissatisfaction can fundamentally interfere with their personality. Self-consciousness and self-conception of the patient, accompanied by depression, can also be affected. Thus we cannot regard this disease and approach it only from a biomedical point of view and should not underestimate the approach to the psychosocial nature of the treatment. Biological, psychological, and social factors are interconnected, and imbalances in their complex interactions disrupt health and cause or worsen the disease. That is why it is necessary to create a so-called biopsychosocial comfort of an individual with SSc and to develop a number of activities in the sense of a complex treatment. This chapter focuses on the psychosomatic approach to health and illness and the development of the biopsychosocial model in medicine just as it should be used in patients with SSc.
{"title":"Biopsychosocial Aspects in Management of Scleroderma Patients","authors":"M. Špiritović","doi":"10.5772/INTECHOPEN.86220","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.86220","url":null,"abstract":"Systemic sclerosis (SSc) is a rare, chronic connective tissue disease with characteristic fibrosis of the skin, musculoskeletal system, and internal organs. It is a heterogeneous and polymorphic disorder, in which fatigue, sleep disturbances, stiffness, and joint pain are among the most significant clinical symptoms. However, the presence of stiffening and thickening of the skin usually has a negative impact on the appearance of these patients, and the degree of self-dissatisfaction can fundamentally interfere with their personality. Self-consciousness and self-conception of the patient, accompanied by depression, can also be affected. Thus we cannot regard this disease and approach it only from a biomedical point of view and should not underestimate the approach to the psychosocial nature of the treatment. Biological, psychological, and social factors are interconnected, and imbalances in their complex interactions disrupt health and cause or worsen the disease. That is why it is necessary to create a so-called biopsychosocial comfort of an individual with SSc and to develop a number of activities in the sense of a complex treatment. This chapter focuses on the psychosomatic approach to health and illness and the development of the biopsychosocial model in medicine just as it should be used in patients with SSc.","PeriodicalId":127513,"journal":{"name":"New Insights into Systemic Sclerosis [Working Title]","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"131860518","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-05-03DOI: 10.5772/INTECHOPEN.86219
B. Heřmánková
Systemic sclerosis (SSc) may affect all aspects of life including sexual function. Although sexual dysfunction is a neglected area of quality of life in patients with SSc, it turns out that it is an important issue for both men and women characterized by high prevalence. The etiology of sexual dysfunction in systemic sclerosis is multifactorial and includes factors associated with both physical and emotional (psychological) status. The most common physical problems in women are vaginal dryness, dyspareunia, and vaginal tightness. Erectile dysfunction is not only a frequent but also often underestimated clinical symptom in men with SSc. The incidence of erectile dysfunction in patients with SSc ranged from 12 to 81% in different studies. The main psychological factors that may affect sexuality are depression, fear, and changes in the appearance of the face and body that usually leads to impaired self-esteem. This chapter is a review about the impact of systemic sclerosis on sexual functioning.
{"title":"Sexual Dysfunction in Patients with Systemic Sclerosis","authors":"B. Heřmánková","doi":"10.5772/INTECHOPEN.86219","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.86219","url":null,"abstract":"Systemic sclerosis (SSc) may affect all aspects of life including sexual function. Although sexual dysfunction is a neglected area of quality of life in patients with SSc, it turns out that it is an important issue for both men and women characterized by high prevalence. The etiology of sexual dysfunction in systemic sclerosis is multifactorial and includes factors associated with both physical and emotional (psychological) status. The most common physical problems in women are vaginal dryness, dyspareunia, and vaginal tightness. Erectile dysfunction is not only a frequent but also often underestimated clinical symptom in men with SSc. The incidence of erectile dysfunction in patients with SSc ranged from 12 to 81% in different studies. The main psychological factors that may affect sexuality are depression, fear, and changes in the appearance of the face and body that usually leads to impaired self-esteem. This chapter is a review about the impact of systemic sclerosis on sexual functioning.","PeriodicalId":127513,"journal":{"name":"New Insights into Systemic Sclerosis [Working Title]","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-05-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122825960","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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