Scleroderma is a rare autoimmune disease characterized by hardening of the skin. The main effect of this disease is the abnormal growth of connective tissue and its hardening and thickening and begins to mistakenly attackthe healthy tissues of the body.This leads to an overproduction of collagen that hardens the skin and in the process of whichthe internal organs , such as the heart, lungs, kidneys, stomach or blood vessels, can also be affected.
As with other autoimmune diseases, scleroderma is more frequent in women between thirty and fifty years of age, and affects, according to the Spanish Society of Rheumatology, one in 50,000 people.
The cause of scleroderma is not known, but there are recent studies that point to a multifactorial origin in which genetic and environmental factors are involved.As is the case with exposure to silica – for example, in mining and sandblasting activities – and to certain drugs.
What you have to keep in mind is that it is an autoimmune disease, that is, the body’s own immune system is the one that reacts against other parts of the body.
It should be noted that up to 90% of people with scleroderma may develop some level of scarring in the lungs, making it difficult for them to breathe and carry out daily activities. Thus, pulmonary fibrosis, for which there is no treatment, is one of the main causes of mortality among people with this disease.
SYMPTOMS
Symptoms of scleroderma include:
Calcium deposits in the connective tissues
Reynaud’s phenomenon, narrowing of blood vessels in the hands or feet
Inflammation of the esophagus, the tube between the throat and stomach
Thick, tight skin on the fingers
Red spots on the hands and face
SCLERODERMA CYCLE
According to the support website Knows about scleroderma, the evolution and progression of this disease in each patient is highly variable, although the process usually occurs as follows:
The immune system sends wrong messages to the fibroblasts and tells them to produce large amounts of collagen. This occurs because it believes that its own cells are a danger and tries to defend the body against itself.
So the fibroblasts produce too much collagen.
Unnecessary and excess collagen clumps together to form thick, rigid areas similar to scars. Scar (fibrotic) tissue can make the problem worse by causing the cycle of inflammation, collagen, and scarring to continue. Fibrosis and inflammation of the skin and other organs affects their function and causes the symptoms of scleroderma.
Likewise, in the general development, there is alteration at three different levels:
Vascular involvement: there is a narrowing and hardening of the blood vessels.
Inflammatory changes:Fibrosis or hardening of the body’s tissues and organs develops as a result of increased collagen production. It can also appear connective tissue disease, a disease that affects the connective tissue of the body.
Autoimmune alteration: there is presence in serum of antibodies (substances that react against the cells or proteins of the body itself).
TYPES
Broadly speaking, several types of scleroderma are identified:
Localized scleroderma: affects only the skin, although it can spread to bones, joints and muscles.
Systemic scleroderma: the disease affects internal organs and, depending on the greater or lesser extent of the affected skin, it is usually distinguished between:
-Limited systemic scleroderma (minor involvement).
-Diffuse systemic scleroderma (greater involvement).
Scleroderma without scleroderma: the disease receives this name because there is no skin involvement, although internal organs are.
TREATMENT
In some cases, the skin problems associated with scleroderma go away on their own within two to five years.The type of scleroderma that affects the internal organs usually gets worse over time.For this reason, although there is no specific cure, there are several treatments to control the symptoms and complications.
It should be noted that no medication is specifically indicated for the disease, but its most frequent effects are. Medications that have been shown to help treat other autoimmune diseases, such as rheumatoid arthritis and lupus, usually don’t work for people with scleroderma. Therefore, doctors try to use drugs for individual effects such as:
Treat or delay changes in the skin.Steroid creams or pills can help reduce swelling and joint pain, loosen tight skin, and slow the development of new skin changes.
Dilate blood vessels.Blood pressure medications that dilate blood vessels can help prevent lung and kidney problems, and can help treat Raynaud’s disease.
Suppress the immune system.Drugs that suppress the immune system, such as those taken after an organ transplant, may help reduce the symptoms of scleroderma.
Reduce digestive symptoms.Stomach acid reducer tablets can help relieve heartburn. Antibiotics and medications that help move food through the intestines can help reduce bloating, diarrhea, and constipation.
Prevent infections.Antibiotic ointment, cleansing, and cold protection can help prevent infection of fingertip ulcers caused by Raynaud’s disease. Regular flu and pneumonia shots can help protect lungs that have been damaged by scleroderma.
Ease the pain.If over-the-counter pain relievers don’t help enough, you can ask your doctor to prescribe stronger medications.
