Atherosclerosis (ATS) has been considered to be a degenerative disease affecting large and medium-sized arteries, resulting in a passive build-up of cholesterol in the artery wall. In the last decade, immune system was proved to play the key role in the pathogenesis of ATS, suggesting ATS to be more progressive and accelerated in chronic inflammatory conditions. Studies in patients with autoimmune diseases, particularly in the most prevalent ones such as rheumatoid arthritis and systemic lupus erythematosus, confirmed the significantly more serious atherosclerotic disease and increased cardiovascular (CV) risk compared to the general population, suggesting these diseases as an independent risk factor for CV diseases. There are only few studies evaluating ATS and CV risk in systemic sclerosis (SSc). Moreover, these studies present contradictory results. Furthermore, it is complicated to differentiate primary vascular affection related to the pathogenesis of SSc from the secondary vascular infliction due to ATS. Nevertheless, most of the studies to date suggest ATS and its clinical manifestations to be more prevalent in SSc. Future studies evaluating larger cohorts of patients are required to determine the relevance of ATS and CV disease and management of these comorbidities in SSc.
Part of the book: Systemic Sclerosis
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 knowledge about the renal involvement in SSc and current treatment principles and possibilities.
Part of the book: New Insights into Systemic Sclerosis
Cardiovascular diseases are the leading cause of morbidity and mortality in general population worldwide. There is an increasing significance of cardiovascular risk in the field of rheumatology, and accordingly, the evidence on the relation between immune system disorders and atherosclerosis has been substantially growing especially in last decades. Since novel immune and metabolic factors are considered to participate in pathogenesis of atherosclerosis and increased cardiovascular risk in rheumatic patients, they are getting to the forefront of the research. Since novel therapeutic approaches have improved survival of rheumatic patients, and decreased morbidity caused by rheumatic disease activity and damage, the significance of other comorbidities leading to premature mortality has arisen. Nevertheless, appropriate recommendations for the management of cardiovascular risk are still lacking. Recently, European League Against Rheumatism (EULAR) recommendations for management of the cardiovascular risk and comorbidities in patients with inflammatory arthropathies have been published. However, the cardiovascular management of these patients is still suboptimal. In addition, the situation in other orphan diseases such as idiopathic inflammatory myopathies, systemic sclerosis and others is even more uncertain and strongly requires further research. This chapter provides an overview of epidemiology, pathogenesis, clinical manifestations, screening and management of atherosclerosis in patients with rheumatic diseases.
Part of the book: Atherosclerosis, Arteriosclerosis and Arteriolosclerosis