What is scleroderma?
Scleroderma is a chronic, rare autoimmune disease in which normal tissue is replaced by dense, thick fibrous tissue. Normally the immune system helps protect the body from disease and infection. In patients with scleroderma, the immune system triggers other cells to produce too much inflammation. Extra collagen is deposited in the skin and organs, which causes them to harden and thicken (similar to the scarring process).
Scleroderma can affect many different parts of the body, including the skin, gastrointestinal tract, lungs, kidneys, heart, blood vessels, muscles and joints. In its most severe forms, scleroderma can be life-threatening.
Explanation of medical terms and concepts Scleroderma
Scleroderma (sklair-oh-DUR-muh), conjointly referred to as general pathology, could be a cluster of rare diseases that involve the hardening and adjustment of the skin. it's going to conjointly cause issues within the blood vessels, internal organs and canal.
Scleroderma is usually categorized as "limited" or "diffuse," that refers solely to the degree of skin involvement. Each variety will involve any of the opposite vascular or organ issues. Localized scleroderma, conjointly referred to as dermatosclerosis, affects solely the skin.
While there's no cure for scleroderma, treatments will ease symptoms, slow progression and improve quality of life.
or CREST Syndrome Systemic sclerosis (also known as scleroderma) is a rare disease resulting in hardening of the skin and connective tissues Since the 1970s there has been confusion about the terminology for this disorder with terms including systemic sclerosis and CREST syndrome (calcinosis Raynaud's phenomenon esophageal dysfunction/dysmotility sclerodactyly [hardening of the hands] and telangiectasias) Although most people with systemic sclerosis have diffuse thickening and tightening of their skin over many areas of their bodies not everyone experiences this symptom In.
Scleroderma which means hard skin is a chronic rheumatic disease that affects the connective tissue Although not all patients develop the thickening of skin and scarring that are characteristic of scleroderma most patients experience systemic involvement in the form of inflammation and progressive damage to internal organs This damage can be severe enough to lead to death from heart failure or lung disease Scleroderma is also known as CREST syndrome because it includes calcinosis (calcium deposits) Raynaud's phenomenon (cold intolerance leading to color changes in fingers toes and ears) esophageal motility disorders.
Types
Forms of scleroderma
Scleroderma can be classified into two main types: localized and systemic. Localized scleroderma is broken down into two main types: diffuse and limited. Systemic scleroderma can be divided into two subtypes: diffuse and limited.
Localized scleroderma
Scleroderma is a more common form of the disease that affects just one's skin. It often appears as 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 scleroderma
Scleroderma is a condition that affects many parts of the body, including the skin and internal organs. It can hinder digestive and respiratory functions and lead to kidney failure. Scleroderma can sometimes become life-threatening.
Limited scleroderma
Crest syndrome is a group of symptoms that are associated with the disease: Each letter in CREST syndrome stands for a feature of the disease: C - central nervous system problems E - epilepsy R - rash S - seizures T - tiredness E - eyesight problems
CAlcinosis is a condition in which calcium deposits are found in the skin.
RAynaud's phenomenon is a medical condition that causes symptoms.
ESophageal dysmotility means that it is difficult for someone to swallow.
SSkin tightening on the fingers is called sclerodactyly.
Telangiectasias (red spots on the skin)
Patients with limited scleroderma do not experience kidney problems, as the skin thickening is restricted to the fingers, hands, and forearms. Digestive involvement is confined mostly to the esophagus. Later complications include pulmonary hypertension (high blood pressure in the lungs), which can develop. Pulmonary hypertension can be potentially serious in about 20% to 30% of cases. Early signs of the condition include shortness of breath, fatigue, and a rapid breathing rate. These symptoms can be caused by a variety of conditions, including shortness of breath, chest pain, and fatigue.
How common is scleroderma?
250 people out of every million American adults are affected by scleroderma. It usually develops between the ages of 35 and 55, but a form that affects children also occurs. Scleroderma is four times more common in women than it is in men.
Symptoms and Causes Scleroderma
What causes scleroderma?
Scleroderma is an illness that is unknown for the most part. Rarely, a person with scleroderma may have a family history of the disease. Scleroderma is not contagious.
What are the symptoms of scleroderma?
People with scleroderma may experience a variety of symptoms, including thickening of the skin. Other symptoms might include the following:
Swelling in the hands and feet.
Red spots on the skin (telangiectasias)
If calcium builds up in the skin, it is called calcinosis.
Joint contractures (rigidity)
Tight, mask-like facial skin
Ulcerations on the fingertips and toes
Pain and stiffness in the joints
Persistent cough
Breathlessness
Heartburn (acid reflux)
Having difficulty swallowing.
Digestive and gastrointestinal problems
Constipation
Weight loss
Fatigue
Hair loss
Some symptoms of scleroderma also include Raynaud's phenomenon and Sjögren's syndrome. Approximately 85% to 95% of scleroderma patients experience one or more of these conditions. Primary Raynaud's phenomenon is common, but there are other cases where the condition becomes less severe over time. Raynaud's phenomenon is often the result of an underlying connective tissue disorder. However, only 10% of patients with Raynaud's will develop Scleroderma.
Sjögren's syndrome is caused by a lack of tears and saliva. This dryness is due to immune damage and destruction of the glands that produce moisture. The condition is named after the Swedish doctor Henrik Sjögren, who first described it in the early 1900s. It is described in this passage. Scleroderma is a condition that affects approximately 20% of patients.
Diagnosis Scleroderma
How is scleroderma diagnosed?
Scleroderma is a condition that can affect other parts of the body, such as the joints. It may be initially mistaken for rheumatoid arthritis or lupus.
Your doctor will perform a physical examination based on your personal family medical history. During the examination, he or she may notice any of the symptoms mentioned above, such as thickening or hardening of the skin around your fingers and toes or discoloration of the skin. If it is suspected that you have scleroderma, tests will be ordered to confirm the diagnosis and to determine the severity of the disease. These tests may include:
Blood tests Scleroderma is a condition in which high levels of immune factors called antinuclear antibodies are found in 95% of patients. These antibodies are also present in people with lupus, so testing for them can help to identify potential scleroderma patients. To diagnose a problem, you need to know what to look for.
Pulmonary function tests These tests are used to measure how well the lungs are functioning. If scleroderma is suspected or has been confirmed, it is important to check to see if it has spread to the lungs where it can cause scar tissue formation. An X-ray or CT scan may be used to look for this. Cancer.
ElectrocardiogramScleroderma can cause scarring of the heart tissue, which may lead to congestive heart failure and defective electrical activity of the heart. This test is performed to see whether the disease has affected the heart.
Echocardiogram(An ultrasound of the heart): This is recommended every 6 to 12 months to check for complications like pulmonary hypertension and/or congestive heart failure.
Gastrointestinal tests Scleroderma can affect the muscles of the esophagus and intestine. This can cause difficulty with breathing, heartburn, and swallowing problems. Scleroderma can also impede the absorption of nutrients and movement of food through the intestine. An endoscopy (the insertion of a small tube with a camera) can help diagnose and treat scleroderma. Endoscopy (sometimes called a "stomach scope") is used to view the esophagus and intestines.A test called manometry can measure the strength of the muscles in the esophagus.
Kidney function Scleroderma can affect the kidneys, resulting in an increase in blood pressure and leakage of protein into the urine. In its most serious form, called a scleroderma renal crisis, a rapid increase in blood pressure may occur leading to kidney failure. Kidney function can be impaired. Medical tests are used to assess a person's health.
Treatment Scleroderma
How is scleroderma treated?
At this time, there is no cure for scleroderma. Treatment focuses on managing and controlling the symptoms. Because scleroderma can have many symptoms, a variety of approaches must be taken to treat it effectively.
Skin treatments Some topical medications for scleroderma are beneficial. Moisturizers help to prevent the skin from drying out and to treat hardened skin. To improve blood flow so that sores in the fingers can heal, nitrates such as nitroglycerin may be prescribed. Nitrates work by relaxing the blood vessels. Smooth muscles cause the arteries to widen. Smooth muscles are those that make up the support blood vessels and some internal organs. Nitrates can have side effects, such as dizziness, nausea, rapid heartbeat, and blurred vision. It is important to talk with your doctor about whether or not you should take nitrates before surgery. It's possible that these plants are not right for you.
Digestive remedies: Medicine may be prescribed to help patients with heartburn and other digestive difficulties. These include over-the-counter and prescription antacids, proton pump inhibitors (like Prevacid Protonix or Nexium), and H 2 receptor blockers (like Zantac or Pepcid). Proton pump inhibitors and H 2 receptor blockers work by preventing the stomach's proton or acid pump from working. This stops the secretion of stomach acid.
Treatment of lung disease A study recently found that a drug called Cyclophosphamide can be helpful for patients with Scleroderma who are having rapid worsening pulmonary fibrosis. Decoupling from the disease process can improve lung function and quality of life in scleroderma patients with interstitial lung disease.
For pulmonary hypertension, the most successful treatment is continuous intravenous infusion of epoprostenol (Flolan®), a prostaglandin. A similar prostaglandin, treprostinil (Remodulin®), can also be administered through a pump. Therapy that is currently approved by the FDA for pulmonary hypertension includes oral medications such as bosentan (Tracleer) and sildenafil (Revatio) and inhaled medications such as iloprost. These substances have the ability to relax smooth muscle and widen blood vessels. This is a trademark name for a type of medicine.
Lung transplant is an option for people with severe interstitial lung disease and pulmonary hypertension.
Joint difficultiesScleroderma patients may be prescribed anti-inflammatory drugs to reduce pain and swelling. Physical therapy to prevent joints from locking can sometimes be helpful.
Raynaud's phenomenon Some effective medications include vasodilators like calcium channel blockers (Procardia or Norvasc), nitroglycerine patches/ointments, alpha blockers, and sildenafil. Oral agents such as sildenafil (Viagra) or amlodipine (Norvasc) may be added to treat ischemic digital ulcers. Aspirin is often prescribed to help prevent heart attacks. If you have severe ulceration or are about to go into gangrene, you might need to take Bosentan (Tracleer®) or Epoprostenol (Flolan®) intravenously. If an infected ulcer needs local wound care and a prolonged course of antibiotics, that's appropriate. Antibiotics are medications used to treat infections.
Sjögren's syndrome Dry eyes can be treated with artificial tears and cyclosporine eye drops (Restasis®). Dry mouth can be alleviated by sipping liquids or chewing gum. If the dry mouth is more severe, drugs that stimulate the production of saliva may be prescribed. Doctors may prescribe Evoxac® or Salagen®.
Kidney problemsIf kidney problems are related to scleroderma, treatment can include medications (especially ACE inhibitors) and dialysis.
Management of scleroderma
There are many things a person with scleroderma can do to better manage the disease. Some of these include:
Exercise
Regular exercise will make you physically and spiritually healthier, and it will help keep your joints flexible. Talk to your doctor or physical therapist about the appropriate exercises for you.
Joint protection
If you have joint pain, avoid heavy lifting or activities that may cause further injury. A physical therapist can help you learn new ways to do your daily activities without placing too much strain on your joints.
Skin protection
Taking proper precautions and care of your skin can be beneficial in treating symptoms of Raynaud's phenomenon as well as the dry, thick patches of skin that are a result of localized scleroderma. There are many ways to accomplish this, including:
When it's cold outside, dress for the weather. Wearing boots, a hat, gloves, and a scarf will help keep your body warm and your blood flowing.
Wear several thin layers. These will keep you warmer than wearing one thick layer.
Wear comfortable shoes or boots so that the blood flow to your feet is unimpeded.
Keep your house humid by placing a humidifier in it.
Use soap and cream that are specifically designed for dry skin.
Diet
To get the proper amounts of vitamins and nutrients, it is important to eat foods that are not likely to irritate your stomach. Some ways to do this include:
Avoiding foods that cause heartburn.
Adding water or another liquid to food will soften it further.
Eating high fiber foods will help reduce constipation.
Eating smaller meals allows the body to digest the food more easily. Wait at least four hours after eating before lying down.
To prevent stomach acid from entering the esophagus while you are sleeping, raise your bed about six inches by placing blocks or bricks underneath it.
Dental care
For patients with scleroderma, proper dental care is essential due to the increased risk of developing cavities and tooth decay.
Stress management
Reducing stress can have many benefits, such as improving your emotions and health. To do this, follow these steps:
Getting proper sleep and rest.
Avoiding stressful situations is possible.
Eating a healthy diet.
How to control anxieties and fears.
Exercising.
Scleroderma is not a cure, but it is often slowly progressive, and people with the disease can lead healthy and productive lives. By understanding scleroderma and connecting with local support groups, people can manage the condition effectively. Treatment with decoupage may reduce the risk of further complications.
Coping and support
As is true with other chronic diseases, living with scleroderma can place you on a roller coaster of emotions. Here are some suggestions to help you even out the ups and downs:
Maintain normal daily activities as best you can.
Pace yourself and be sure to get the rest that you need.
Stay connected with friends and family.
Continue to pursue hobbies that you enjoy and are able to do.
Keep in mind that your physical health will have an immediate impact on your mental state. Denial, anger and frustration square measure common with chronic diseases.
At times, you would like further tools to influence your emotions. Professionals, like therapists or behavior psychologists, are also able to assist you place things in perspective. they'll conjointly assist you develop header skills, together with relaxation techniques.
Joining a support cluster, wherever you'll share experiences and feelings with people, is commonly an honest approach. raise your doctor what support teams square measure on the market in your community.
Preparing for your appointment
You'll probably initially bring your symptoms to the eye of your GP, UN agency could refer you to a doctor UN agency makes a specialty of the treatment of inflammatory disease and different diseases of the joints, muscles and bone (rheumatologist). As a result of scleroderma will have an effect on several organ systems, you will have to be compelled to see a range of medical specialists.
What you can do
Time with your doctors may be brief. To make the best use of the limited time, plan ahead and write lists of important information, including:
Detailed descriptions of all your symptoms
A list of all your medications and dosages, including nonprescription drugs and supplements
Questions for the doctor, such as what tests or treatments he or she may recommend
What to expect from your doctor
Your doctor may ask some of the following questions:
Do your fingers change colors when you get cold?
Do you regularly experience heartburn or swallowing problems?
Have you noticed any skin tightening or skin thickening?
General summary
Like all autoimmune diseases treatment for scleroderma focuses on controlling symptoms and preventing the progression of the disease As there is no cure yet simply controlling scleroderma is often a challenge Medications used to treat patients include immunosuppressants and non-steroidal anti-inflammatory drugs (NSAIDS) Blood pressure medication may also be required in some cases.
What is the latest treatment for scleroderma?
The autoimmune disease known as scleroderma can be difficult to treat but recent advancements have made it much easier to manage As new drugs and therapies continue to be developed and improved upon by scientists people with this chronic condition will gain more options for treatment in the future.
Can scleroderma go away?
Scleroderma is a progressive autoimmune disorder that affects the skin connective tissue and internal organs While there is presently no cure symptoms may be reduced or controlled In addition to traditional treatments such as medications and physical therapy complementary integrative medicine approaches may help reduce pain and inflammation decrease the chances of organ damage delay disability progression and improve quality of life Examples include managing stress with acupuncture or exploring mind-body therapies such as yoga.
Can scleroderma be treated naturally?
Treatments that have been shown to be highly effective include vitamin B12 a natural compound called Fumaric acid and garlic Vitamin B12 has actually been approved by the FDA as a treatment for scleroderma Other herbs including licorice root and dandelion root have also been shown in clinical trials to be highly beneficial in reducing symptoms of scleroderma.
What should I avoid with scleroderma?
Scleroderma causes the body's immune system to mistakenly attack the collagen a protein that gives connective tissue strength and elasticity The following can increase signs of scleroderma or put you at risk for complications: Nonsteroidal anti-inflammatory drugs (NSAIDs) Scleroderma is sometimes triggered by overusing NSAIDs such as ibuprofen (Advil Motrin IB others) and naproxen sodium (Aleve) which are used to relieve pain and reduce fever and inflammation If you have scleroderma talk with your doctor before taking these medications Steroids Cort.
What are the stages of scleroderma?
You may have heard that scleroderma is a rare disease But the truth is that most people don't know much about it Here are some quick facts to help you get educated and understand more about this condition that affects the skin blood vessels and internal organs 1 in 100,000 Americans have scleroderma Only 1 in 5 people with scleroderma survive past 20 years of diagnosis There is no known cure for Scleroderma More than 90 percent of people diagnosed live longer than 10 years after their symptoms first appear.
Is scleroderma serious?
Scleroderma is a rare disorder that affects the skin connective tissues (such as tendons and ligaments) and internal organs A hardened layer of skin called "sclerosis" forms over the affected areas Early signs of scleroderma include thickening and hardening of the skin on the hands or fingers As it progresses this condition can cause damage to parts of the body such as muscles joints heart lungs and kidneys Most people with scleroderma have Raynaud's phenomenon which causes changing coloration in fingers when exposed to cold.