Scleroderma is connective tissue disease changes in the skin, blood vessels, skeletal muscles, and internal organs. It’s also known as systemic sclerosis. This disease affects approximately 300,000 people in the US, and the rates keep increasing 2-20 % yearly. Found more common in women than men. Genes are also playing in leading to the development of scleroderma. A connective tissue disease is one that affects tissues such as skin, tendons, and cartilage. Scleroderma can be classified in terms of the degree and location of the skin involvement.
Symptoms of Scleroderma
- Heartburn: is a result of stomach acid back flowing up into the esophagus causes heart burn.
- Diffuse form of scleroderma: symmetric thickening of skin of the extremities, face, trunk (chest, back, abdomen, or flanks).
- Stomach cramps and diarrhea.
- Difficulty swallowing and/or localized pain in the central chest.
- Shorted breath, especially with physical exertion.
- Elevated blood pressure is potentially serious and can lead to kidney damage
- Headache, fatigue, and in severe cases, stroke.
- Elevated pressure in the arteries to the lungs (pulmonary hypertension) can also cause shortness of breath and difficulty getting an adequate breath with activity.
Causes of Scleroderma
Scleroderma causes inflammation and thickening of the skin. In these disorders, a person’s antibodies are directed against his or her own tissues.
- Exposure to industrial solvents or an environmental agent may play a role in predisposing to scleroderma.
- Plaquenil drug has relatively few side effects, and it’s also effective for the arthritis that can be associated with scleroderma. This medication to affect the way immune cells work.
Scleroderma has been categorized into two major groups, diffuse and limited:
- Diffuse form of scleroderma: can rapidly progress to hardening after an early inflammatory phase. Organ disease can occur early on and be serious. Organs affected include the esophagus, bowels, and lungs with scarring (fibrosis), heart, and kidneys.
- Limited scleroderma: typically has gradual onset and is restricted to certain areas of the skin. Reynaud’s phenomenon is due to vasoconstriction of the small arteries of exposed peripheries - particularly the hands and feet - in the cold temperature. The skin changes and other features of disease tend to occur more slowly than in the diffuse form. Because a characteristic clinical pattern can occur in patients with the limited form of scleroderma, this form has taken another name which is composed of the first initials of the common components. Telangiectasia is a condition caused by the swelling of tiny blood vessels, in which small red spots appear on the hands and face. While not painful, these red spots can create cosmetic problems. Genetic predisposition appears to be limited: genetic concordance is small; still, there often is a familial predisposition for autoimmune disease. Polymorphisms in COL1A and TGF-ß1 may influence severity and development of the disease. There is limited evidence implicating cytomegalovirus (CMV) as the original epitope of the immune reaction, and organic solvents and other chemical agents have been linked with scleroderma.
Treatments of Scleroderma
- Medications that help with blood circulation such as, angiotensin-converting enzyme (ACE) inhibitors and low-dose enteric-coated aspirin.
- Tracleer is an oral medication that has been approved for pulmonary hypertension in people with scleroderma.
- Prilosec, Nexium, or Prevacid will help to relieve esophagus irritation and heartburn.
- Antacids can also be helpful for heartburn as well.
- Elevating the head of the bed can reduce backflow of acid into the esophagus that causes inflammation and heartburn.
- Avoiding caffeine and cigarette smoking also helps for Scleroderma patient.
- People with scleroderma-related lung disease found cyclophosphamide modestly improved lung function and quality of life. Cyclophosphamide (Cytoxan) is sometimes used to treat pulmonary fibrosis.