Scleroderma : Types,Symptoms & Treatment
Scleroderma: Scleroderma is an uncommon condition that is a group of autoimmune diseases results in hard, thickened areas of skin, blood vessels, muscles, and internal organs. Scleroderma is induced by the immune system mistakenly attacking the connective tissue under the skin and around internal organs and blood vessels. Scleroderma symptoms may comprise areas of thickened skin, stiffness, feeling tired, and inadequate blood flow to the fingers or toes with cold exposure. One form of the condition, identified as CREST syndrome, classically results in calcium deposits, thickening of the skin of the fingers and toes, Raynaud’s syndrome(a circulation problem where fingers and toes turn white in the cold), and areas of small dilated blood vessels. The cause is unknown; however, it may be due to an abnormal immune response. Risk factors combine family history, certain genetic factors, and exposure to silica. While there is no cure, treatments may promote symptoms. Outcomes depend on the extent of the condition. Those with the localized condition usually have a normal life expectancy. In those with systemic disease average life expectancy is approximately 11 years from onset. Death is usually due to the lung, heart or gastrointestinal, complications.
About 3 out of 100,000 people per year develop the systemic form of scleroderma. Scleroderma, which affects about 300,000 Americans. About one-third of those people have the systemic form of scleroderma. The condition usually begins in middle age. Women are more frequently affected than men. Scleroderma was first described in 1753 by Carlo Curzio and next well documented in 1842. The term is of the Greek “sclerosis” meaning “hardness” and “derma” indicating “skin”.
Scleroderma Types and Typical Scleroderma Symptoms
There are two main types of scleroderma-
Localised scleroderma –that only affects the skin.
Systemic sclerosis – that may affect blood circulation and internal organs as well as the skin.
Localised scleroderma is the mildest form of the condition. It usually affects children but can happen at any age. Localized scleroderma can transpire in any race, but is more prevalent in Caucasians. Most patients who acquire scleroderma are womanlike. The disease is not contagious. The disease is not carried on directly from parent to child by any one gene, though specific genes may make a child more prone to develop localized scleroderma.
Localized scleroderma is a rare condition, and the accurate number of patients with this disease is not understood. The best estimate is that 50 children out of each 100,000 will acquire localized scleroderma.
The localized scleroderma simply affects the skin, causing one or more hard patches to develop. Internal organs aren't affected.
Precisely how the skin is touched depends on the type of localized scleroderma. There are two types, named morphea and linear.
Discolored oval patches on the skin.
Patches may be hairless and shiny.
Can appear anywhere on the body.
May improve after a few years and treatment may not be needed.
Thickened skin occurs in lines along the face, scalp, legs or arms.
Infrequently attacks underlying bone and muscle.
May better after a few years, although can induce permanent growth problems, such as shortened limbs.
Systemic scleroderma is an autoimmune disorder of the connective tissue. It is marked by thickening of the skin caused by the accumulation of collagen, and by injuries to small arteries. There are two sorts of systemic sclerosis, known as limited cutaneous systemic sclerosis and diffuse systemic sclerosis.
Limited cutaneous systemic sclerosis: The limited cutaneous systemic sclerosis affects the skin of only the face, hands, and feet, although a milder form that, can ultimately affect the lungs and digestive system too.
Often starts as Raynaud's phenomenon other typical symptoms include thickening of the skin over the hands, feet, and face, red spots on the skin, hard lumps under the skin, heartburn, and problems swallowing.
Other limited cutaneous systemic sclerosis symptoms can include weight loss, fatigue, and joint pain and stiffness.
Tends to get progressively worse over time, although it's usually less severe than diffuse systemic sclerosis and can often be controlled with treatment. Patients with limited cutaneous scleroderma become a 10-year survival rate of 75%; fewer than 10% develop pulmonary arterial hypertension following 10 to 20 years.
Diffuse Systemic Sclerosis: The systemic diffuse form involves those and, in addition, may progress to visceral organs, including the kidneys, heart, lungs, and gastrointestinal tract.
Is more likely to affect internal organs.
Skin changes can affect the whole body.
Symptoms come on suddenly and get worse quickly over the first few years, but then the condition normally settles and the skin may gradually improve.
Prognosis is determined by the form of the disease and the degree of visceral involvement. Patients with diffuse cutaneous scleroderma become a 10-year survival rate of 55%. Death is most usually caused by lung, heart, and kidney involvement. There is also a slight rise in the chance of cancer.
Survival rates have considerably increased with effective treatment for kidney failure. Therapies combine immunosuppressive drugs and, in some cases, glucocorticoids. In systemic sclerosis, often affects women and normally develops between 30 and 50 years of age. Children are rarely affected.
Usually, the body's immune system attacks off any germs that infect the body. The scleroderma cause is unknown. Though, it is believed to be an autoimmune disease, meaning it happens when the body’s immune system wrongly attacks its own cells and tissues.
The scleroderma occurs because part of the immune system has enhanced overactive and out of control. People with scleroderma overproduce collagen, a key element of connective tissue, causing scarring and thickening of the tissue and may cause internal organs to function abnormally.
It's not clear why this befalls. Certain genes are thought to be connected, and having a close family member with the condition may raise your chance of causes of scleroderma.
Scleroderma is difficult to diagnose as it overlaps many other conditions. Depending on your particular symptoms, a scleroderma diagnosis may be made by a dermatologist, an orthopaedist, a respirologist or a rheumatologist.
Typical scleroderma symptoms are skin thickening, with about 70% of cases also presenting with Raynaud's phenomenon and nail-fold capillary changes. Affected individuals may or may not endure systemic organ involvement.
After a careful physical exam, your physician may suggest blood tests to check for elevated blood levels of certain antibodies generated by the immune system. He or she may remove a small tissue specimen (biopsy) of your affected skin so that it can be analyzed in the laboratory for abnormalities.
Laboratory testing can show anti-topoisomerase antibodies, like anti-scl70 that causing a diffuse systemic form, or anticentromere antibodies that causing a limited systemic form and the CREST syndrome. Other autoantibodies can be examined, such as anti-U3 or anti-RNA polymerase.
Your physician may also suggest pulmonary function tests, a CT scan of your lungs and an echocardiogram of your heart.
Currently, there is no scleroderma treatment that checks or stops the overproduction of collagen that induces all forms of scleroderma. Thus, scleroderma treatment focus on alleviating symptoms and limiting damage. Your treatment will depend on the appropriate problems you are possessing.
Because scleroderma can attack many different organs and organ systems, you may have several different specialists involved in your care. Typically, care will be guided by a rheumatologist (musculoskeletal disorders and rheumatic diseases). Your rheumatologist may suggest you to other specialists, depending on the specific problems you are holding. For instance, you may see a nephrologist for kidney difficulties, a cardiologist for the heart problem, a gastroenterologist for difficulties of the digestive tract, and a pulmonary specialist for lung involvement.
In addition to physicians, professionals such as physical or occupational therapists, nurse practitioners, psychologists, patient educators, and social workers may perform a role in your scleroderma care. Dentists, orthodontists, and speech therapists can treat oral complications that result from thickening of tissues in and around the mouth and on the face.
No drug has been developed that can stop the overproduction of collagen. Although a variety of medications can assist control your scleroderma symptoms or help to prevent complications. To fulfill this, these drugs combine:
Dilate blood vessels-Blood pressure medicines that enlarge blood vessels may further prevent lung and kidney problems and may benefit to treat Raynaud's disease.
Suppress the immune system-Drugs that suppress the immune system, such as the taken following organ transplants, may help lessen scleroderma symptoms.
Reduce stomach acid-Medications such as omeprazole can alleviate symptoms of acid reflux.
Prevent infections-Antibiotic cream, cleaning, and protection from the cold may help inhibit infection of fingertip ulcers induced by Raynaud's disease. Seasonal influenza and pneumonia vaccinations can help protect lungs that have been injured by scleroderma.
Relieve pain- If over-the-counter(OTC) pain relievers don't help complete, you can ask your physician to prescribe stronger medications.
Physical or occupational therapists can support you to:
Improve your strength and mobility
Maintain independence with daily tasks
Used as a last option, surgical options for scleroderma may include:
Amputation. If finger ulcers precipitated by severe Raynaud's syndrome have grown gangrene, amputation may be required.
Lung transplants. People who become developed high blood pressure in the arteries through their lungs (pulmonary hypertension) may be suitors for lung transplants.
Home Remedies for Scleroderma
You can practice a number of steps to help control your symptoms of scleroderma:
Exercise puts your body flexible, improves circulation and reduces stiffness. Range-of-motion exercises can keep your skin and joints flexible. Keep normal daily activities as best you can.
Continue to pursue hobbies that you enjoy and are capable to do. Stay connected with friends and family.
Nicotine induces blood vessels to contract, causing Raynaud's disease worse. Smoking can further cause permanent narrowing of your blood vessels. Quitting smoking is hard — ask your physician for help.
Avoid foods that produce you heartburn or gas. Furthermore, avoid late-night meals. Raise the head of your bed to keep stomach acid from backing up into your esophagus (reflux) as you rest or sleep. Antacids may help relieve symptoms.
Wear warm gloves for protection anytime your hands are exposed to cold. While you're outside in the cold, wrap your face and head and wear layers of warm clothing.