Although there is currently no cure for scleroderma, intensive research efforts have resulted in significant advances in disease management. Treatments are focused on providing symptomatic relief and in minimizing the long term damage caused by collagen overproduction. Due to the highly variable nature of the disease, treatment is often simultaneously managed by several specialists. Typically, primary disease management is provided by rheumatologists that specialize in the treatment of rheumatic diseases and musculoskeletal disorders.
Depending on disease progression, other specialists may become involved such as nephrologists (for kidneys), cardiologists (for the heart), gastroenterologists (for the digestive tract) or pulmonologists (for the lungs). Treatment plans are designed to target the specific problems unique to each scleroderma patient; as a result treatment can vary extensively over the course of the disease and from one individual to the next.
Additional professionals such as physical or occupational therapists, psychologists, nurse practitioners, patient educators, and social workers may also provide essential care. Dentists, orthodontists, and speech therapists can treat oral complications that arise from thickening of tissues in and around the mouth and face. Personalized treatment plans are best provided by health care professionals experienced in the care of people living with scleroderma.
In most cases, localized scleroderma carries a good prognosis and patients have a normal life span. Most patients with limited CREST scleroderma can also expect a favourable outlook. Many internal organs may become affected over time, however disease progression tends to be slow.
Disease severity varies widely in systemic scleroderma, making it is difficult to predict future outcomes. Progression can be rapid and acute, quickly developing into a life threatening condition, or it can progress very slowly thereby offering better long term outcomes. The highest risk of disease progression is during the initial years of disease onset. During this critical period, patients run the highest risk of rapid progression and the development of serious complications, particularly if the lungs, heart, or kidneys are affected. Complete spontaneous improvement is very rare, however symptoms can be improved with medication and life style changes. Some medications are aimed at specific symptoms, while others are aimed at decreasing the activity of the immune system. Patient survival has improved a great deal over the past years due to advances in treatment.
Although at present there is no cure for scleroderma, several effective treatment options exist to help alleviate symptoms and slow down disease progression. Current medications are designed to treat scleroderma by targeting four key factors: inflammation, autoimmunity, vascular disease and tissue fibrosis. Patients must develop a keen understanding of prescribed medications and should understand the mechanism of action and how the drugs work to control disease symptoms from progressing. Patient knowledge is a powerful tool in reducing anxiety and increasing compliance, thereby resulting in a higher percentage of successful treatments.
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