Scleroderma

• Skin Hardening and Thickening: This mainly affects the skin on the fingers, hands, face, and forearms, making it tight and thickened.
• Raynaud's Phenomenon: Fingers and toes turn white or blue in response to cold or emotional stress and then may turn red.
• Joint Pain and Swelling: Common in small joints of the hands and feet.
• Digestive System Issues: These include symptoms such as acid reflux, difficulty swallowing, bloating, and slowed bowel movements.
• Respiratory Problems: Difficulty breathing and coughing may occur due to lung fibrosis.
• Cardiac Issues: Conditions such as pericarditis and arrhythmias.
• Kidney Problems: Kidney dysfunction can lead to hypertension and kidney failure.

Causes

The exact cause of scleroderma is not fully understood, but research suggests it may be related to the following factors:

• Genetics: A higher incidence is observed in individuals with a family history of scleroderma or other rheumatic diseases.
• Autoimmune Abnormalities: The immune system mistakenly attacks its connective tissues, leading to fibrosis and hardening. Specific autoantibodies, such as anti-Scl-70 and anti-centromere antibodies, can be found in the patient’s serum.
• Vascular Abnormalities: Most scleroderma patients exhibit Raynaud’s phenomenon, which can be associated with the thickening of small arteries or microvessel endothelium, lumen narrowing, or occlusion.
• Fibroblast Dysfunction: Continuous stimulation of fibroblasts produces excessive collagen fibres, accumulating under the skin and in internal organs, causing skin hardening and organ damage.
• Environmental Exposure: Exposure to organic solvents and silica dust may be contributing factors.
   

Risk Factors

• Gender: Women are more likely to develop scleroderma than men.
• Age: The condition commonly occurs between the ages of 30 and 50.
• Family History: A history of scleroderma or other autoimmune diseases increases the risk.

Diagnosing scleroderma early can be challenging, especially in the absence of typical symptoms and when skin thickening occurs rapidly. Doctors must thoroughly review the patient’s medical history and conduct a comprehensive physical examination. Common tests include:

• Blood and Immunological Tests
• Chest X-ray and Imaging
• Pulmonary Function Tests
• Electrocardiogram (ECG)
• Echocardiogram
• Nailfold Capillaroscopy

According to the classification criteria from the American College of Rheumatology and the European League Against Rheumatism in 2013, a systemic sclerosis (scleroderma) diagnosis can be made if the total score from clinical features and immunological tests reaches 9 or more points.

⚠️ Scleroderma can affect multiple organ systems, and a diagnosis requires professional medical judgment. Accurate diagnosis depends on a comprehensive assessment of the patient’s test results and clinical features.

Scleroderma can affect multiple organ systems, and treatment plans need to be tailored based on the patient's clinical manifestations, affected organs, and disease severity. The following are standard treatment methods:

1. Vasodilators

• Calcium Channel Blockers: Increase blood flow and relieve Raynaud's phenomenon.
• Prostacyclin Analogue and Phosphodiesterase Type 5 Inhibitors: Such as Sildenafil, used to manage ischemic ulcers and gangrene.
• Endothelin Receptor Antagonists: Effectively reduce pulmonary hypertension.

2. Corticosteroids

• They are used for interstitial pneumonia and pulmonary fibrosis and monitoring blood pressure, blood glucose, and kidney function.

3. Immunosuppressants

• Includes Azathioprine, Mycophenolate, Cyclosporin, and Cyclophosphamide.
• Methotrexate can slow down skin fibrosis but should be used cautiously to prevent lung side effects.
• When using immunosuppressants, attention should be given to personal hygiene and infection prevention measures.

4. Proton Pump Inhibitors

• They are used to treat gastroesophageal reflux disease, oesophageal ulcers, and reflux esophagitis and to improve gastrointestinal motility.