About scleroderma

What is scleroderma?

Scleroderma facts

  • Scleroderma is a disease of the connective tissue featuring skin thickening that can involve scarring, blood vessel problems, varying degrees of inflammation, and is associated with an overactive immune system.
  • CREST syndrome is a limited form of systemic sclerosis.
  • Patients with scleroderma can have specific antibodies (ANA, anticentromere, or antitopoisomerase) in their blood that suggest autoimmunity.
  • Treatment of scleroderma is primarily directed toward the particular individual's symptoms.

What is scleroderma?

Scleroderma is an autoimmune disease of the connective tissue characterized by skin thickening, spontaneous scarring, blood vessel disease, and varying degrees of inflammation, associated with an overactive immune system. Autoimmune diseases are illnesses that occur when the body's tissues are attacked by its own immune system. Scleroderma is characterized by the formation of scar tissue (fibrosis) in the skin and, potentially, in the organs of the body. This leads to thickness and firmness of involved skin.

Scleroderma is classified as localized or widespread (systemic sclerosis with a tendency to affect internal organs). Systemic sclerosis is further divided into limited and diffuse based upon the extent of skin involvement.

What are the symptoms for scleroderma?

Scleroderma's signs and symptoms vary, depending on which parts of your body are involved:

  • Skin. Nearly everyone who has scleroderma experiences a hardening and tightening of patches of skin. These patches may be shaped like ovals or straight lines, or cover wide areas of the trunk and limbs. The number, location and size of the patches vary by type of scleroderma. Skin can appear shiny because it's so tight, and movement of the affected area may be restricted.
  • Fingers or toes. One of the earliest signs of scleroderma is an exaggerated response to cold temperatures or emotional distress, which can cause numbness, Pain or color changes in the fingers or toes. Called Raynaud's disease, this condition also occurs in people who don't have scleroderma.
  • Digestive system. In addition to acid reflux, which can damage the section of esophagus nearest the stomach, some people with scleroderma may also have problems absorbing nutrients if their intestinal muscles aren't moving food properly through the intestines.
  • Heart, lungs or kidneys. Scleroderma can affect the function of the heart, lungs or kidneys to varying degrees. These problems, if left untreated, can become life-threatening.

What are the causes for scleroderma?

Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin.

Doctors aren't certain what prompts this abnormal collagen production, but the body's immune system appears to play a role. In some genetically susceptible people, symptoms may be triggered by exposure to certain types of pesticides, epoxy resins or solvents.

What are the treatments for scleroderma?

There is no treatment that can cure or stop the overproduction of collagen that is characteristic of scleroderma. But a variety of treatments can help control symptoms and prevent complications.

Medications

Because scleroderma can affect so many different parts of the body, the choice of medication will vary, depending on the symptoms. Examples include drugs that:

  • Dilate blood vessels. Blood pressure medications that dilate blood vessels may help treat Raynaud's phenomenon.
  • Suppress the immune system. Drugs that suppress the immune system, such as those taken after organ transplants, may help reduce progression of some scleroderma symptoms, such as the thickening of the skin or worsening of lung damage.
  • Reduce digestive symptoms. Pills to reduce stomach acid can help relieve heartburn. Antibiotics and medications that help move food through the intestines may help reduce bloating, diarrhea and constipation.
  • Prevent infections. Cleaning and protection from the cold may help prevent infection of fingertip ulcers caused by Raynaud's disease. Regular influenza and pneumonia vaccinations can help protect lungs that have been damaged by scleroderma.
  • Relieve pain. If over-the-counter pain relievers don't help enough, your doctor might suggest prescription medications to control pain.

Therapies

Physical or occupational therapists can help you improve your strength and mobility and maintain independence with daily tasks. Hand therapy may help prevent hand contractures.

Surgical and other procedures

Stem cell transplants might be an option for people who have severe symptoms that haven't responded to more-common treatments. If the lungs or kidneys have been severely damaged, organ transplants might be considered.

What are the risk factors for scleroderma?

Scleroderma occurs much more often in women than it does in men. Choctaw Native Americans and African-Americans are more likely than Americans of European descent to develop the type of scleroderma that affects internal organs.

Is there a cure/medications for scleroderma?

Systemic sclerosis, commonly referred to as scleroderma, is a group of rare disorders that cause the skin to tighten and harden. Additionally, it could affect the digestive system, internal organs, and blood vessels.

  • Scleroderma is frequently labelled as "limited" or "diffuse," which merely describes how much skin is affected. Both forms may involve any other organ or vascular issues. Skin alone is impacted by localised scleroderma, commonly known as morphea.
  • Scleroderma has no known cure, although therapies can reduce symptoms, halt progression, and enhance quality of life.


Symptomatic approach towards scleroderma medications:

  • The selection of treatment will change depending on the symptoms of scleroderma because it can impact so many various sections of the body.
  • Raynaud's phenomenon may be treated with blood vessel-dilating blood pressure medicines.
  • To reduce immune system functioning. Some scleroderma symptoms, such skin thickening or worsening lung damage, may be slowed down by immune-suppressing medications, such as those prescribed after organ transplants.
  • To ameliorate symptoms’ of digestion. Heartburn relief medications can ease the condition. Bloating, diarrhoea, and constipation may be lessened by antibiotics and drugs that facilitate the movement of food.


Symptoms
Skin tightening both in external skin and also the mucosa in the internal organ
Conditions
Raynad phenomenon of the blood vessels,Ddigestive issues,Mucosal tightening in internal organs
Drugs
Symptomatic treatment,No cure

Video related to scleroderma