How systemic sclerosis affects the skin, lungs, and internal organs

Systemic sclerosis, or scleroderma, is a rare and complex autoimmune disorder that targets the body’s connective tissue. It causes an abnormal buildup of collagen, leading to fibrosis (scarring) and thickening of vital tissues and organs. This progressive disease can have a profound impact on the skin, lungs, and internal organs, making early detection and effective management crucial. 

In this article, we will explore how systemic sclerosis affects these areas and discuss current treatment options that help manage the disease’s progression and improve patients' quality of life.

How does systemic sclerosis affect the skin?

Systemic sclerosis can have a profound impact on the skin, causing visible and often distressing changes. As the disease progresses, the immune system mistakenly attacks healthy skin tissue, leading to the overproduction of collagen. 

This collagen buildup gradually replaces normal skin cells, causing the skin to harden and thicken. So, how exactly does systemic sclerosis alter the skin?

1. Thickening of skin:

• Often begins in the fingers and hands (a condition known as sclerodactyly) and can spread to other parts of the body.

• The skin may become shiny, tight, and less flexible.

• Severe cases can restrict movement, particularly in the fingers and joints, making everyday tasks challenging.

2. Loss of skin elasticity:

• The skin loses its ability to stretch, contributing to discomfort and functional limitations.

• The hardened skin can create a feeling of stiffness, making normal movement difficult.

3. Changes in appearance and texture:

• As the disease progresses, deeper layers of skin may also be affected, causing changes in texture, such as depigmentation, scarring, and the formation of ulcers.

• The skin may become rigid and less responsive to external stimuli.

4. Increased susceptibility to infections:

• The skin’s compromised barrier makes it more vulnerable to infections.

• Wounds may take longer to heal, causing further complications.

5. Psychological impact:

• Patients often experience significant cosmetic changes, which can affect self-esteem.

• The visible physical changes may lead to emotional challenges, contributing to mental health struggles for many individuals.

The skin manifestations of systemic sclerosis go beyond just physical changes; they can significantly impact a person’s emotional and social well-being. Managing these effects requires a holistic approach that addresses both the physical and psychological challenges of the disease.

How does systemic sclerosis affect the lungs?

Systemic sclerosis can cause serious lung complications due to collagen buildup, leading to fibrosis and impaired lung function. This thickening of lung tissue reduces the ability to expand and contract properly. How exactly does this affect lung health?

• Pulmonary fibrosis: Collagen buildup thickens lung tissue, impairing gas exchange, leading to shortness of breath, fatigue, and a persistent cough.

• Pulmonary hypertension: Increased pressure in lung blood vessels can strain both the heart and lungs, worsening respiratory issues.

• Reduced lung function: Scarred lung tissue hampers breathing efficiency and reduces overall lung capacity.

• Risk of respiratory insufficiency: Untreated fibrosis can lead to respiratory failure, a major cause of death in systemic sclerosis patients.

• Advances in treatment: Anti-fibrotic medications can slow fibrosis progression, improving lung function and quality of life.

Timely treatment is essential to manage lung complications and reduce the risk of severe outcomes.

How Does Systemic Sclerosis Affect Internal Organs?

Systemic sclerosis can cause significant damage to internal organs like the heart, kidneys, and gastrointestinal system due to collagen buildup, which disrupts normal organ function. Let’s have a look at how this affects each organ:

1. Heart:

• The thickening of heart tissues can lead to diastolic dysfunction (difficulty relaxing and filling with blood) and arrhythmias (irregular heartbeats).

• Over time, this impairs the heart’s ability to pump blood effectively, increasing the risk of heart failure and exacerbating pulmonary hypertension.

2. Kidneys:

• Systemic sclerosis can cause scleroderma renal crisis, leading to rapid hypertension and kidney failure.

• Fibrosis in the kidneys also disrupts waste filtration and fluid balance, worsening kidney function.

3. Gastrointestinal System:

• The oesophagus may be affected by thickening, leading to GERD and difficulty swallowing (dysphagia).

• Impaired motility of the gastrointestinal tract can cause bloating, constipation, and malabsorption, resulting in nutritional deficiencies.

The impact of systemic sclerosis on internal organs requires close monitoring and timely treatment to manage complications effectively.

How Is Systemic Sclerosis Managed?

While there is no cure for systemic sclerosis, advancements in treatment have greatly improved outcomes for many patients. Let’s explore the current treatment options available:

• Anti-Fibrotic Medications: Medications like Nintib 100 capsules, containing the active ingredient Nintedanib, target the fibrotic process driving disease progression, particularly in the lungs, helping to slow down the buildup of scar tissue. Target the fibrotic process driving disease progression, particularly in the lungs, helping to slow down the buildup of scar tissue.

• Immunosuppressive Medications: Suppress the overactive immune system to reduce inflammation and prevent further damage.

• Vasodilators: Help manage pulmonary hypertension by relaxing blood vessels, easing the strain on the heart and lungs.

• Symptom Management: Therapies focus on alleviating symptoms like pain, reflux, and gastrointestinal issues, improving daily function.

• Physical Therapy: Supports mobility and function as the disease progresses, improving overall well-being.

• Regular Monitoring: Ongoing assessment of lung, kidney, and organ health to manage complications and maintain quality of life.

Wrapping up

Systemic sclerosis affects the skin, lungs, and internal organs, causing significant physical and emotional challenges. With early intervention and treatments, disease progression can be slowed. Ongoing care and a proactive approach are key to managing symptoms and improving outcomes. Advances in research offer hope for better management and quality of life.