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Scleroderma, commonly known as systemic sclerosis, is a category of uncommon disorders characterized by skin hardness and tightening. It may also create issues with blood vessels, internal organs, and the digestive tract.
Scleroderma is frequently classified as "limited" or "diffuse," which solely relates to the extent of skin involvement. Both types can be associated with any of the other vascular or organ issues. Only the skin is affected by localized scleroderma, commonly known as morphea.
There is no cure for scleroderma, however therapies can alleviate symptoms, delay progression, and improve quality of life.
WHAT ARE THE SYMPTOMS OF SCLERODERMA?
The indications and symptoms of scleroderma differ from person to person, depending on which regions of the body are affected.
Skin-related symptoms and signs
Almost everyone who has scleroderma develops skin hardening and tightness.
The fingers, hands, feet, and face are frequently the first portions of the body to be afflicted. Skin thickening can affect the forearms, upper arms, chest, belly, lower legs, and thighs in some persons. Swelling and itching are common early signs. Because of the tightness, affected skin may lighten or darken in tone and seem glossy.
Some people also have little red spots on their hands and face, known as telangiectasia. Calcium deposits can accumulate beneath the skin, particularly at the fingertips, generating visible lumps on X-rays.
Raynaud's phenomenon
Raynaud's phenomenon, which arises as a result of an incorrect and excessive constriction of the tiny blood vessels in the fingers and toes in response to cold or emotional discomfort, is common in scleroderma. When this occurs, the digits may turn white, blue, or red and become painful or numb. Raynaud's phenomenon can also affect persons who do not have scleroderma.
Digestive issues
Scleroderma can affect any region of the digestive system, including the esophagus and rectum. Signs and symptoms may include the following, depending on which areas of the digestive system are affected:
- Heartburn
- Swallowing Difficulties
- Bloating
- Diarrhea
- Constipation
- Fecal incontinence
Heart and lung issues
Scleroderma can induce shortness of breath, poor exercise tolerance, and dizziness when it affects the heart or lungs. Scleroderma can induce lung tissue scarring, which can lead to increased shortness of breath over time. Medication may assist to reduce the progression of this lung damage.
Scleroderma can also cause an increase in blood pressure in the circulation between the heart and the lungs. This is referred to as pulmonary hypertension. Pulmonary hypertension can produce extra fluid in the legs, feet, and sometimes around the heart, in addition to shortness of breath.
Heartbeats can become erratic when scleroderma affects the heart. Some persons may experience heart failure.
WHAT ARE THE CAUSES OF SCLERODERMA?
Scleroderma is caused by an excess and accumulation of collagen in human tissues. Collagen is a fibrous kind of protein found in connective tissues throughout the body, including the skin.
Doctors aren't sure what triggers this process, but the immune system appears to play a part. Scleroderma is most likely caused by a mix of causes, including immune system issues, genetics, and environmental triggers.
WHAT ARE THE RISK FACTORS OF SCLERODERMA?
Scleroderma can affect anyone, however it affects women far more frequently than men. Several factors appear to interact to influence the likelihood of getting scleroderma:
- Genetics: Scleroderma appears to be more common in people who have particular gene variants. This could explain why some scleroderma cases appear to run in families and why particular forms of scleroderma are more common in certain ethnic groups.
- Environmental triggers: Scleroderma symptoms may be induced in some people by exposure to specific infections, pills, or pharmaceuticals, according to research. Repeated exposure to some dangerous substances or chemicals, such as at work, may also raise the risk of scleroderma. Most folks do not have an environmental trigger.
- Immune system issues: Scleroderma is thought to be an autoimmune disorder. This indicates that it happens in part as a result of the body's immune system attacking the connective tissues. Scleroderma patients may also experience signs of another autoimmune disease, such as rheumatoid arthritis, lupus, or Sjogren's syndrome.
WHAT ARE THE COMPLICATIONS OF SCLERODERMA?
Scleroderma complications can range from mild to severe and can affect the following organs:
- Fingertips: Raynaud's phenomenon can become so severe in systemic sclerosis that the limited blood flow permanently destroys the tissue at the fingertips, leaving pits or skin ulcers. The tissue on the fingertips may perish in some circumstances.
- Lungs: Scarring of lung tissue might impair your breathing ability and tolerance to exercise. High blood pressure in the arteries leading to your lungs is also possible.
- Kidneys: A significant kidney consequence (scleroderma renal crisis) is characterized by an abrupt increase in blood pressure and quick kidney failure. It is critical to treat this illness as soon as possible in order to preserve renal function.
- Heart: Scarring of cardiac tissue raises the likelihood of irregular heartbeats and congestive heart failure. Scleroderma can potentially inflame the membranous sac that surrounds your heart.
- Teeth: Severe facial skin tightening can make your mouth smaller and narrower, making it difficult to wash your teeth or even have them professionally cleaned. Scleroderma patients frequently do not generate normal levels of saliva, increasing the risk of dental damage.
- Digestive system: Scleroderma-related digestive issues can cause heartburn and difficulties swallowing. It can also induce cramping, bloating, constipation, and diarrhea. Scleroderma patients may also have difficulty absorbing nutrition due to bacterial proliferation in the colon.
- Joints: Skin over joints can become so tight that it limits joint flexibility and movement, especially in the hands.
HOW IS SCLERODERMA DIAGNOSED?
Scleroderma can be difficult to diagnose since it manifests itself in so many various ways and affects so many different areas of the body.
Following a thorough physical examination, your doctor may recommend blood tests to look for high levels of particular immune system antibodies.
Other blood tests, imaging, or organ-function testing may be recommended by your doctor to help identify whether your digestive system, heart, lungs, or kidneys are impacted.
WHAT ARE THE TREATMENTS FOR SCLERODERMA?
There is no medication that can cure or stop the scleroderma-related collagen overproduction. However, a number of treatments are available to help control symptoms and avoid problems.
Medications
Because scleroderma can affect so many various sections of the body, medicine will be chosen based on the symptoms. Examples include medications that:
- Dilate blood vessels: Raynaud's phenomenon may be treated with blood pressure medicines that widen blood vessels.
- Suppress the immune system: Immunosuppressive drugs, such as those used after organ transplants, may help slow the progression of some scleroderma symptoms, such as skin thickening or worsening lung damage.
- Reduce digestive symptoms: Heartburn can be relieved by using acid-reducing pills. Antibiotics and drugs that aid in the passage of food through the intestines may aid in the reduction of bloating, diarrhea, and constipation.
- Prevent infections: Cleaning and cold protection may help avoid infection of Raynaud's disease-caused fingertip ulcers. Regular influenza and pneumonia immunizations can help protect scleroderma-damaged lungs.
- Reduce pain: If over-the-counter pain remedies are ineffective, your doctor may recommend prescription pain relievers.
Therapies
Physical and occupational therapists can assist you in increasing your strength and mobility while maintaining your independence with daily responsibilities. Hand treatment may aid in the prevention of hand contractures.
Surgical and other procedures
People with severe symptoms who have not responded to more popular treatments may be candidates for stem cell transplants. Organ transplants may be considered if the lungs or kidneys have been badly damaged.
HOME REMEDIES FOR SCLERODERMA
You can assist manage your scleroderma symptoms by doing the following:
- Stay active: Exercise maintains flexibility, promotes circulation, and relieves stiffness. Exercising your range of motion can help keep your skin and joints supple. This is always critical, but especially early in the disease's progression.
- Skin protection: Use lotion and sunscreen on a daily basis to care for dry or tight skin. Hot baths and showers, as well as exposure to harsh soaps and household chemicals, can irritate and further dry up your skin.
- Avoid smoking: Nicotine causes blood vessels to constrict, exacerbating Raynaud's illness. Smoking can also cause persistent blood vessel constriction and cause or exacerbate lung issues. Quitting smoking is difficult; seek advice from your doctor.
- Manage heartburn: Foods that cause heartburn or gas should be avoided. Avoid late-night meals as well. Elevate your bed's head to prevent stomach acid from backing up into your esophagus while you sleep. Antacids may be useful in relieving symptoms.
- Take precautions against the cold: Wear warm mittens to protect your hands from the cold, even when reaching inside a freezer. It is also critical to keep your core body temperature warm in order to avoid Raynaud's symptoms. Wear warm boots, protect your face and head, and layer your clothing when you're outside in the cold.