Preventing Scleroderma Development

Introduction:

Systemic sclerosis is also known as scleroderma. It is a rare disease that causes the skin to harden and tighten. It could also harm the digestive system, internal organs, and blood vessels. Scleroderma is divided as ‘limited’ or ‘diffuse,’ depending upon the extent to which skin is affected. Morphea is the other name for localized scleroderma affecting the skin.

Treatments for Scleroderma can alleviate symptoms, slow the disease's progression, and improve quality of life, but there is no possible cure for this disease.

What Is Scleroderma?

Scleroderma is a chronic autoimmune condition where normal tissue is replaced and dense, thick fibrous tissues occur. Regularly, the invulnerable framework shields the body against illness and contamination. In scleroderma, the immune system causes other cells to produce excessive collagen (a protein). The skin and organs harden and thicken due to this extra collagen deposited there, similar to how scars form. Scleroderma most frequently affects the skin but can also affect the gastrointestinal tract, lungs, kidneys, heart, blood vessels, muscles, and joints. In the most severe forms, scleroderma can be fatal.

What Are the Types of Scleroderma?

There are two types of scleroderma present:

1. Localized Scleroderma - Localized scleroderma is a more common form of Scleroderma. It only affects a person's skin, usually in a few places. It frequently manifests itself on the skin as waxy streaks or patches, and it is not uncommon for the less severe form to disappear or cease to progress without treatment.

2. Systemic Scleroderma - This form has many effects on the body. It can affect the skin and several internal organs, making it difficult to breathe and digest food and even leading to kidney failure. Systemic scleroderma can occasionally progress to life-threatening levels. Systemic scleroderma is of two types diffuse and limited.

What Is Limited Scleroderma?

There are no kidney issues in those with limited scleroderma. The skin thickening is limited to the fingers, forearms, and hands, but it can also occur on the feet and legs at times. The esophagus is the primary site of digestive involvement. Pulmonary hypertension is one of the later complications that can be potentially serious. In pulmonary hypertension, the arteries that connect the heart to the lungs narrow, putting a lot of pressure on the right side of the heart, which can eventually result in right-sided heart failure. Pulmonary hypertension often begins with fatigue, chest pain, and shortness of breath. Limited Scleroderma is also called CREST syndrome. Where CREST denotes:

• Calcinosis (calcium salts depositing in skin and tissues).

• Raynaud's phenomenon (decreased blood flow in extremities due to muscle spasms).

• Esophageal dysmotility.

• Sclerodactyly (thickening and tightening).

• Telangiectasias (widening blood vessels on the skin).

What Are the Causes of Scleroderma?

The overproduction and accumulation of collagen in the body's tissues lead to scleroderma. Collagen forms the body's skin and other connective tissues, a fibrous protein.

The body's immune system plays an important role in this process, even though doctors are unsure of the exact cause. The most common factors that cause Scleroderma are issues with the immune system, genetics, and environmental problems.

What Are the Symptoms of Scleroderma?

1. Skin-Related Signs and Symptoms -

• Almost all people with scleroderma experience skin tightening and hardening. Fingers, hands, feet, and the face are typically the first areas of the body to be affected. The skin can thicken on the forearms, upper arms, chest, abdomen, lower legs, and thighs in some people as well. Swelling and itching may be the first signs. Due to the tightness, the affected skin may become lighter or darker and appear shiny.
• Telangiectasia, or small red spots, can also appear on the hands and faces of some people. Calcium deposits can form under the skin, particularly at the fingertips, resulting in X-ray-visible bumps.

2. Raynaud's Phenomenon - Scleroderma sufferers frequently experience Raynaud's phenomenon, characterized by an inappropriate and exaggerated contraction of the fingers' and toes' small blood vessels in response to cold or emotional distress. The digits may turn white, blue, or red, making them feel painful or numb. People without Scleroderma can also experience Raynaud's phenomenon.

3. Digestive Problems - From the esophagus to the rectum, any part of the digestive system can be affected by Scleroderma. Signs and symptoms can include the following, depending on which parts of the digestive system are affected:

4. Incontinence Feces - difficulty swallowing, bloating, diarrhea, and constipation.

5. Heart and Lung Problems - Scleroderma that affects the heart or lungs can cause dizziness, decreased exercise tolerance, and shortness of breath. Scleroderma may cause long-term shortness of breath. Some medicines might slow the damage to the lungs down.

Additionally, Scleroderma can elevate blood pressure in the circulation that connects the heart and lungs. Pulmonary hypertension is the name for this. Pulmonary hypertension can cause shortness of breath and excessive fluid in the legs, feet, and occasionally around the heart.

Heartbeats may become irregular if Scleroderma affects the heart. Some people may also develop heart failure.

How to Diagnose Scleroderma?

Scleroderma diagnosis is difficult. Scleroderma can be misdiagnosed with rheumatoid arthritis or lupus at first because it can affect other parts of the body, like the joints.

After discussing personal and family medical history, the doctor will conduct a comprehensive physical examination. As part of this process, the doctor will look for any of the above symptoms, particularly hardening of the skin of fingers and toes or skin discoloration. Tests will be ordered to confirm the diagnosis and determine the severity of Scleroderma if suspected. These tests might include the following:

• Blood Tests - Most scleroderma patients have elevated immune factors (antinuclear antibodies). Even though these antibodies are also found in other autoimmune diseases like lupus, testing for them in people who might have Scleroderma also helps in diagnosis.

• Tests of Pulmonary Function - These tests aim to assess how well the lungs work. Checking to see if Scleroderma has spread to the lungs. An X-ray or a computed tomography (CT) scan helps diagnose damage to the lung.

• Gastrointestinal Test - Test to check gastrointestinal health.

• Kidney Function Tests - Blood and urine tests to check how the kidney’s are functioning.

What Is the Treatment of Scleroderma?

Scleroderma does not have a treatment. Instead, the goal of treatment is to control and manage the symptoms. To effectively treat and manage Scleroderma, various treatments are frequently required.

• Skin Care -Topical treatments are frequently helpful in treating localized Scleroderma. Moisturizers are used to treat hardened skin and prevent skin from drying out. Nitrates like nitroglycerin help increase blood flow and speed up the healing process for finger abrasions. Nitrates dilate the arteries by relaxing the smooth muscles. Generally, smooth muscles support some internal organs and blood vessels. Consult the doctor before taking nitrates because they can cause side effects like dizziness, nausea, rapid heartbeat, and blurred vision.

• Remedies For the Stomach: Heartburn and other digestive issues can be treated with many medications. Antacids, proton pump inhibitors, and H2 receptor blockers. Proton pump inhibitors prevent stomach acid from being secreted by inhibiting the proton or acid pump. H2 receptor blockers work by blocking histamine, a chemical in the body that makes the stomach produce more acid.

• Treatment For Lung Diseases: Cyclophosphamide, a type of chemotherapy, was helpful for scleroderma patients with rapidly worsening pulmonary fibrosis (scarring of the lung tissue). One study demonstrated that oral cyclophosphamide improved lung function and quality of life in scleroderma patients with interstitial lung disease.

• Vaccination: Scleroderma-damaged lungs may benefit from regular influenza and pneumonia vaccinations.

• Painkillers: The doctor may recommend prescription painkillers if over-the-counter pain relievers do not provide sufficient relief.

• Organ Transplantation: In the case of severely damaged organs, organ transplantation is advised.

How to Manage Scleroderma?

With all the medications and other treatments, some lifestyle changes are beneficial in managing Scleroderma. Such as -

• Regular exercises.

• Skin protection during winters.

• Joint protection by avoiding any injury.

• Avoid unhealthy diets, such as fast food, alcohol, and smoking.

• Reduce stress.

Conclusion:

Even though Scleroderma has no known cure, the condition is frequently manageable and progresses slowly, and those who suffer from it may live productive and healthy lives. As with many other conditions, education about Scleroderma and local support groups may be the most effective means of managing the condition and lowering the likelihood of recurrence.