Scleroderma - Symptoms, Causes, Types, Diagnosis, and Treatment

What Is Scleroderma?

A long-lasting rheumatic disease that affects the skin, connective tissues, and internal organs are scleroderma, which is often characterized as limited or diffuse, depending on the degree of skin involvement. Autoimmune diseases tend to be caused due to the immune system attacking the body cells and tissues, which weakens and destroys organs in the body.

What Are the Causes of Scleroderma?

The immune system causes the body to produce large amounts of the protein collagen, which is an important part of the skin. This causes the skin to get thick and tight, and scars form on the lungs and kidneys.Most likely, scleroderma is caused by a combination of factors, including immune system problems, genetics, and environmental triggers. Research suggests that scleroderma symptoms may be triggered due to exposure to certain viruses, medications, and drugs.

What Are the Types of Scleroderma?

1. Localized Scleroderma - This type of scleroderma affects only the skin and not the major organs. It consists of three types:

• Circumscribed Morphea: Circumscribed morphea is characterized by either a single or a few discolored patches. These patches appear pale yellow in color with a red border and are of varying sizes. Active or symptomatic lesions are typically enlarged and become flat when treated and asymptomatic.

• Generalized Morphea: Generalized morphea is characterized by several discolored patches across different anatomical areas. These patches are not distinct from each other. They may blend into each other and appear as tight and thick lesions.

• Linear Scleroderma: Linear scleroderma is characterized by the appearance of a single, tight, thick band in a single body area that may be interspersed with morphea patches. It may even affect the muscles and bones and cause disability of the limbs when it appears in the extremities.

2. Systemic Scleroderma - This type of scleroderma affects the skin and may affect the tissues underlying it, including the blood vessels and major organs, like the gastrointestinal tract, kidneys, heart, and lungs. It consists of the following three types:

• Limited Cutaneous Scleroderma: The commonest type, limited scleroderma causes the tightening and hardening of the fingers and occasionally the forearms, hands, or face. These patients are able to lead normal lives, although some may develop concurrent issues like heartburn, musculoskeletal pain, and Raynaud’s phenomenon.

• Diffuse Cutaneous Scleroderma: Diffuse scleroderma affects large areas of the body and is characterized by the thickening of the skin (accompanied by an itching sensation and dryness) over these areas because of excess collagen production. Damage to organs like the heart, kidneys, gastrointestinal tract, and lungs may occur in this type of sclerosis. The condition can be pretty severe and life-threatening.

• Sine Sclerosis: Sine sclerosis demonstrates systemic symptoms and illnesses seen in limited or diffuse scleroderma-like Raynaud’s phenomenon, but the skin symptoms are absent.

What Are the Signs and Symptoms of Scleroderma?

The following are the manifestations of scleroderma:

• Red spots on the skin (telangiectasia).

• Rashes.

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• Swollen blood vessels in the skin or ulcers.

• Acid reflux.

• Shortness of breath.

• Tight and hardened fingers.

• Weight loss.

• Fatigue.

• Headache.

• Chronic cough.

• Difficulty swallowing.

• Joint stiffness.

• Calcinosis (formation of calcium deposits in any soft tissue).

• Raynaud’s phenomenon and Sjogren’s syndrome are seen in patients with scleroderma. About 85 % to 95 % of scleroderma patients experience Raynaud’s phenomenon.

Who Is at an Increased Risk of Getting Scleroderma?

Although anyone can get scleroderma, some groups are at a higher risk of getting affected. They include:

• Sex - Scleroderma is more common in women than men.

• Age - It occurs most commonly between the ages of 30 and 50 and is more common in adults than children.

• Race - Scleroderma can affect people of all races and ethnic groups, but African- Americans are more severely affected.

How Is Scleroderma Diagnosed?

In addition to the patient's symptoms, medical history, and thorough physical examination, various tests are conducted by the doctor to aid in the diagnosis of scleroderma. These include -

1. Antinuclear Antibody Testing: Elevated levels of immune factors, known as antinuclear antibodies found in 95% of scleroderma patients. It checks the presence of autoantibodies (blood proteins) that are present in the blood.

2. Pulmonary Function Test: Rules out the functioning of the lungs.

3. Electrocardiogram : The ECG test is carried out to check whether the disease has affected the heart. Detects the activities of the heart and checks the abnormal rhythm of the heart.

4. Echocardiogram: To evaluate complications like hypertension and congestive heart failure.

5. Gastrointestinal Tests:

• Manometry can measure the strength of the esophageal muscles.

• An endoscopy can be performed to view the esophagus.

6. Kidney Function Tests: These are done to evaluate the function of the kidneys and whether the disease has affected the kidneys, which can result in an increase in blood pressure as well as the leakage of protein into the urine. The serious form is called scleroderma renal crisis.

What Are the Complications of Scleroderma?

• Digestive disorders.

• Pulmonary hypertension.

• Wound infections.

• Renal failure.

• Cardiovascular disorders.

• Muscle pain and joint pain.

• Inability to open mouth due to tightening of facial skin and narrowing of mouth.

• Lungs disease.

• Fingertips ulceration.

What Is the Treatment of Scleroderma?

Managing the symptoms is the aim of treatment, and a combination of approaches is often needed to treat and manage the disease effectively.

• Pain relief by non-steroidal anti-inflammatory drugs or corticosteroids.

• Maintaining muscle strength through physical exercise and therapies.

• Controlling blood pressure and increasing blood flow with medications.

• Improving emotional state through counseling, motivation, and other measures.

• Skin Treatments: For localized scleroderma, topical medications are beneficial, and moisturizers prevent the drying of the skin. Nitrates help to relax the smooth muscles, causing arteries to dilate.

• Digestive Medications: A variety of medications can be used to prevent digestive problems and heartburn, such as Antacids and Proton pump inhibitors.

• Treatment of Lung Disease: In patients with scleroderma who have rapidly worsening pulmonary fibrosis, the drug Cyclophosphamide (Cytoxan), a form of chemotherapy, has been proven useful in recent research.

• Surgery- If the complications cannot be resolved with minimally invasive therapies. Ulcers lead to gangrene, and amputation of the parts might be done.

Conclusion:

Although there is no cure for scleroderma, the disease is often slowly progressive and manageable, and people can lead a healthy life. A multidisciplinary approach is most important for scleroderma patients because of the complications of the disease. Make sure to pursue regular activities, stay well-rested, seek the help of a therapist for coping and relaxation techniques, and reach out to support groups and trusted friends and family members. These simple acts can help to deal with the condition and stay positive.