Scleroderma

Scleroderma

Scleroderma: Understanding Scleroderma in Depth

Scleroderma, also known as “scleroderma,” is an autoimmune disease in which the body’s immune system malfunctions and starts attacking its own tissues. This causes the body to produce too much collagen (a protein that helps keep skin and tissues strong). This causes the body’s tissues to harden and thicken, which can affect the skin and many internal organs, such as the lungs, heart, kidneys, and digestive system.

Pathophysiology
The disease of scleroderma is related to a process called fibrosis, or excessive connective tissue formation. In people with scleroderma, the immune system malfunctions and produces autoantibodies that attack the body’s own tissues, triggering a process called fibroblast activation, which causes fibroblasts to produce too much collagen, resulting in thickening and hardening of the skin and internal organs. This accumulation of large amounts of collagen causes the tissues in organs, such as the lungs and heart, to harden and prevent them from functioning properly, disrupting blood flow and functioning of these organs.

Classification of Scleroderma
Scleroderma can be divided into two main types:
1. Localized Scleroderma:
•This type of disease usually affects only the skin or subcutaneous tissue, without affecting internal organs.
•Morphea: It appears as hard patches on the skin of varying sizes.
•Linear Scleroderma: The skin appears as long, hard lines, usually on the arms, legs or face.
2.Systemic Scleroderma:
•This is a more severe type because it can affect both the skin and internal organs.
•Limited Cutaneous Scleroderma: The disease affects only one part of the body, such as the hands and arms.
•Diffuse Cutaneous Scleroderma: Affects both the skin and internal organs, causing breathing problems and other organ functions.

Symptoms and Symptoms
•Skin-related symptoms: Skin tightness and stiffness: On the face, hands and arms, the skin often feels tight and difficult to move, and sometimes there may be changes in skin color. Skin sores and ulcers: In some cases, patients may develop sores that look like burns or superficial wounds.
• Symptoms related to internal organs: Breathing problems: Patients may have trouble breathing because their lungs have been damaged by collagen deposits.
• Digestive problems: The digestive system may be affected, such as difficult digestion or stomach ulcers.
• Blood vessel changes: Such as Raynaud’s phenomenon, which causes the blood vessels in the fingertips and toes to temporarily constrict when exposed to cold.
• Immune system symptoms: Patients may have joint pain or allergy-like symptoms that often occur with joint inflammation.

Diagnosis of Scleroderma
Scleroderma is diagnosed using a number of methods that can indicate the nature of the disease, including:
• Physical examination: Your doctor will perform tests to look for changes in your skin and the functioning of your body systems.
• Blood tests: Tests to look for abnormal immune markers, such as antinuclear antibodies (ANA), which are common in people with scleroderma.
• Specialized tests: These include CT scans, X-rays, or ultrasounds to assess damage to your lungs or internal organs.

Treatment
Although scleroderma cannot be cured, However, treatment can help relieve symptoms and prevent the disease from spreading. The methods used include:
1.Medication: Anti-inflammatory drugs such as steroids or methotrexate that reduce inflammation.
Immunosuppressants: Immunosuppressants are used to reduce abnormal immune system activity. Drugs that reduce collagen production such as colchicine.
2.Organ-specific treatments: Lung problems: Patients with lung problems may need to use newer drugs such as bosentan or pirfenidone to reduce inflammation in the lungs. Hemodialysis: Patients with kidney problems may need to undergo hemodialysis.
3.Rehabilitation: Physical therapy: to maintain joint flexibility and reduce pain.
4.General health care: controlling risk factors such as high blood pressure, diet, and gentle exercise to promote overall health.

Summary
Scleroderma is a disease in which the immune system attacks tissues in the body, causing the skin and internal organs to harden. It is divided into two main types: Localized (just the skin) and Systemic (skin and internal organs). Treatment aims to relieve symptoms and prevent the disease from spreading.