Essay On Scleroderma

SYSTEMIC SCLERODERMA

Scholarly Paper Systemic Scleroderma
Wendy Wingate
McNeese State University
Nursing 618

SYSTEMIC SCLERODERMA

INTRODUCTION

Systemic scleroderma is an uncommon autoimmune disorder that affects the skin and internal organs. Scleroderma is derived from the Greek term skleros meaning hard and derma meaning skin. If the disease affects organs other than the skin, it is considered systemic. It is often classified as an autoimmune rheumatic disease because the body’s immune system attacks connective tissues, causing stimulation of an excess amount of collagen, eventually leading to fibrosis of the skin and internal organs. Systemic scleroderma is a very serious condition where remission is rare and cure is impossible. Treatment is limited, and focuses on minimizing or alleviating the disease effects and symptoms (Systemic Scleroderma, 2011). With no cure, it is important to provide the emotional support and necessary education when caring for the populations diagnosed with this grave disease.
INCIDENCE AND IMPACT Systemic scleroderma is a rare disease with approximately 90,000 Americans affected and an estimated annual incidence of eighteen to twenty new cases per million people. Systemic scleroderma most commonly develops in Native American or African American females between the ages of thirty-five and forty-five, whom have a strong family history that suggest concern for scleroderma. Studies have shown that African Americans and Japanese Americans have that highest morbidity and mortality rates (Scleroderma, n.d.). Improved survival contributes to higher prevalence of the disease, and the prevalence of systemic scleroderma is consistently higher in the USA and Australia than in Japan and Europe. Across the
SYSTEMIC SCLERODERMA nation, the state with the highest prevalence of systemic scleroderma is in Oklahoma, USA where the Choctaw Indian tribe resides (Nikpour, M., Stevens, W., Herrick, A., & Proudman, S., 2010). With no cure, systemic scleroderma can have a significant impact on the patient’s lives by eventually creating disability, handicap, and overall worsened quality of life. Some of the most common symptoms that affect every day living in systemic scleroderma are extreme fatigue, stiffness of hands, difficulty sleeping, and joint pain. Patients may be forced to go on prolonged sick leave depending on the severity of the disease, creating financial stress and burden on the patients and their families.
PATHOPHYSIOLOGY AND RECENT RESEARCH FINDINGS The exact etiology of systemic scleroderma remains complex and unclear, but is subject to ongoing research. To date, the most common gene associated with systemic scleroderma is the family of the human leukocyte antigen (HLA) complex. “Specific normal variations of several HLA genes seem to affect the risk of developing systemic scleroderma” (Systemic Scleroderma, 2011). “The pathological events in SSc may include impaired communication between endothelial cells, epithelial cells and fibroblasts; lymphocyte activation; autoantibody production; inflammation; and connective tissue fibrosis” (Abraham, D. J., 2009). Hypoxia has been shown as the decisive factor to regulate the inflammatory process in systemic scleroderma, causing activation of fibroblasts. Cytokines are mediators synthesized

SYSTEMIC SCLERODERMA by immune cells, and they aid in activation of fibroblasts and their differentiation into myofibroblasts, causing an increased inflammatory response (Brinckmann, 2009). An overproduction and accumulation of collagen and other extracellular matrix proteins causes fibrosis and thickening of the skin, which is the commonly noted as the first phase of scleroderma. Systemic scleroderma has two main types: limited and diffuse scleroderma. Limited scleroderma is also known as CREST syndrome. The acronym represents the most common characteristics including:

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