OverviewWhat is scleroderma?Scleroderma is a chronic, although rare, autoimmune disease in which normal tissue is replaced with dense, thick fibrous tissue. Normally, the immune system helps defend the body against disease and infection. In patients with scleroderma, the immune system triggers other cells to produce too much collagen (a protein). This extra collagen is deposited in the skin and organs, which causes hardening and thickening (similar to the scarring process). Show
Although it most often affects the skin, scleroderma also can affect many other parts of the body including the gastrointestinal tract, lungs, kidneys, heart, blood vessels, muscles and joints. Scleroderma in its most severe forms can be life-threatening. Forms of sclerodermaThere are 2 major forms of scleroderma, localized and systemic. Systemic scleroderma can be broken down into two main types: diffuse and limited. Localized sclerodermaThe more common form of the disease, localized scleroderma, affects only a person's skin, usually in just a few places. It often appears in the form of waxy patches or streaks on the skin, and it is not uncommon for this less severe form to go away or stop progressing without treatment. Diffuse sclerodermaAs its name implies, this form affects many parts of the body. Not only can it affect the skin, but it also can affect many internal organs, hindering digestive and respiratory functions, and causing kidney failure. Systemic scleroderma can sometimes become serious and life-threatening. Limited sclerodermaAlso known as CREST syndrome, each letter stands for a feature of the disease:
Patients with limited scleroderma do not experience kidney problems. The skin thickening is restricted to the fingers, hands and forearms, and also sometimes the feet and legs. Digestive involvement is confined mostly to the esophagus. Among later complications, pulmonary hypertension, which can develop in 20% to 30% of cases, can be potentially serious. In pulmonary hypertension, the arteries from the heart to the lungs narrow down and generate high pressure on the right side of the heart, which can ultimately lead to right sided heart failure. Early symptoms of pulmonary hypertension include shortness of breath, chest pain, and fatigue. How common is scleroderma?Approximately 250 persons per million American adults are affected by scleroderma. It usually develops between the ages of 35 and 55, although a pediatric form also occurs. Scleroderma is four times more common in women than it is in men. Symptoms and CausesWhat causes scleroderma?The exact cause of scleroderma is unknown. Although rarely, scleroderma can run in families. Most cases do not show any family history of the disease. Scleroderma is not contagious. What are the symptoms of scleroderma?In addition to the thickening of skin, the following other symptoms may occur in a person with scleroderma:
In addition to these symptoms, 2 other conditions—Raynaud's phenomenon and Sjögren's syndrome—also affect patients with scleroderma. Approximately 85% to 95% of scleroderma patients experience Raynaud's phenomenon. However, primary Raynaud's phenomenon is common and often occurs by itself without any underlying connective tissue disorder. Only 10% of patients with Raynaud's phenomenon will develop scleroderma. Sjögren's syndrome is manifested by dry eyes and mouth. This dryness is due to lack of secretion of tears and saliva as a result of immune damage and destruction of the moisture-producing glands of the body. This condition is named after the Swedish eye doctor, Henrik Sjögren, who first described it. It is seen in approximately 20% of patients with scleroderma. Diagnosis and TestsHow is scleroderma diagnosed?The diagnosis of scleroderma is not always easy. Because it can affect other parts of the body — such as the joints — scleroderma may be initially mistaken for rheumatoid arthritis or lupus. After discussing your personal family medical history, your doctor will perform a thorough physical examination. In doing so, he or she will be looking for any of the symptoms mentioned above, especially thickening or hardening of the skin around the fingers and toes or discoloration of the skin. If scleroderma is suspected, tests will be ordered to confirm the diagnosis, as well as to determine the severity of the disease. These tests may include:
Management and TreatmentHow is scleroderma treated?Currently, there is no cure for scleroderma. Instead, treatment is directed at controlling and managing the symptoms. Because scleroderma can have many symptoms, a combination of approaches is often needed to treat and manage the disease effectively.
For pulmonary hypertension, the most successful treatment is continuous intravenous infusion of epoprostenol (Flolan®), a prostaglandin, through a pump. Subcutaneous infusion of treprostinil (Remodulin®) – a related prostaglandin – is an acceptable alternative. Prostaglandins are hormone-like substances found in the body that, among other things, help relax the smooth muscle, and hence dilate blood vessels. Other forms of therapy that are currently FDA approved for pulmonary hypertension include oral bosentan (Tracleer ®), sildenafil (Revatio ®) and inhaled iloprost (Ventavis®). Lung transplant is a viable option for both severe (drug refractory) interstitial lung disease and pulmonary hypertension.
Management of sclerodermaIn addition to taking prescribed medications correctly and regularly, there are many steps a person with scleroderma can take to better manage the disease. These include: ExerciseRegular exercise will not only help improve your overall physical and spiritual well-being, but it will also help keep your joints flexible and improve circulation. Consult your doctor or physical therapist for appropriate exercises. Joint protectionWhen your joints hurt, avoid lifting heavy objects or performing chores that may place a strain on them, thus risking further injury. A physical therapist can help you learn new ways to perform daily activities without placing undue strain on your joints. Skin protectionTaking proper precautions and care of your skin can be beneficial not only for symptoms of Raynaud's phenomenon, but also in taking care of the dry, thick patches of skin that result from localized scleroderma. There are many ways to accomplish this, including:
DietAside from eating healthy foods to get the proper amounts of vitamins and nutrients, it is important to eat foods that do not aggravate existing stomach problems. Ways to do this include:
Dental careFor those patients with scleroderma who also have Sjögren's syndrome, proper dental care is essential. Sjögren's syndrome increases the risk of developing cavities and tooth decay. Stress managementBecause the effects of stress can play a part in reducing your blood flow, as well as affect many other aspects of your emotions and health, it is important to learn to manage or reduce stress. This can be done by taking the following steps:
Although no cure has been found for scleroderma, the disease is very often slowly progressive and manageable, and people who have it may lead healthy and productive lives. Like many other conditions, education about scleroderma and local support groups can be the greatest tools for managing the disease and reducing the risk of further complications. Which action would the nurse take when providing care for a client suspected of having the Ebola virus?If assessment indicates possible Ebola virus infection, take action. Isolate the patient in a private room with a private bathroom or covered, bedside commode and close the door. Wear appropriate personal protective equipment (PPE). Limit the healthcare personnel who enter the room.
Which complication is a result of Clostridium Welchii Clostridium perfringens entering a clients wound and causing crepitus?Gas gangrene is a highly lethal infection of soft tissue, caused by Clostridium species, with Clostridium perfringens being the most common.
Which clinical manifestations support a client's diagnosis of cryptosporidiosis?Clinical manifestations. fever.. cough.. fatigue.. nausea.. eye pain.. vomiting.. dizziness.. joint pain.. Which diagnostic test determines the specific cause of a client's allergic reaction?Allergen-specific immunoglobulin E (IgE) testing is the most common blood allergy test. IgE is a type of protein known as an antibody that is associated with allergic reactions. Certain types of IgE are related to specific allergens, and elevated levels can reflect a possible hypersensitivity.
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