Overview

Scleroderma, also known as systemic sclerosis, is a rare autoimmune disease characterized by hardening and tightening of the skin and connective tissues. It can affect not only the skin but also internal organs such as the lungs, heart, kidneys, and digestive system. Our scleroderma services provide comprehensive diagnosis, treatment, and long-term management to control symptoms, improve quality of life, and minimize damage to vital organs.

Diagnostic Procedures

1. Clinical Evaluation:
   • A detailed medical history and physical examination to assess symptoms such as skin thickening, joint pain, and organ involvement.
2. Laboratory Tests:
   • Antinuclear Antibody (ANA) Test: Common in autoimmune diseases, a positive ANA is often seen in scleroderma.
   • Anti-Scl-70 Antibody (Topoisomerase): A specific marker for diffuse cutaneous systemic sclerosis.
   • Anticentromere Antibody: Typically associated with limited cutaneous scleroderma (CREST syndrome).
   • Complete Blood Count (CBC): To assess anemia or other blood abnormalities.
3. Imaging Studies:
   • Chest X-rays and High-resolution CT Scans: To assess lung involvement, such as interstitial lung disease.
   • Echocardiograms: To evaluate heart function and check for pulmonary hypertension.
   • Esophageal Manometry: To assess the motility of the esophagus if scleroderma affects the digestive system.
4. Pulmonary Function Tests (PFTs):
   • To measure lung capacity and function, especially in patients with suspected lung involvement.
5. Skin Biopsy:
   • In some cases, a small skin sample may be taken for histological examination to confirm the diagnosis of scleroderma.

Symptoms of Scleroderma

• Skin Changes: Hardening and tightening of the skin, often beginning with the fingers, hands, and face, and progressing to other parts of the body.
• Raynaud’s Phenomenon: Cold sensitivity in the fingers and toes, causing them to turn white or blue in response to cold temperatures or stress.
• Joint Pain and Stiffness: Arthritis-like symptoms, often affecting the hands and wrists.
• Digestive Issues: Heartburn, difficulty swallowing, and slow digestion due to esophageal and intestinal involvement.
• Lung Involvement: Shortness of breath and coughing, often caused by interstitial lung disease or pulmonary hypertension.
• Heart and Kidney Involvement: In more severe cases, scleroderma can lead to heart failure or kidney crisis.

Possible Treatments

1. Medications:
   • Immunosuppressants: Such as methotrexate, mycophenolate mofetil, or cyclophosphamide to reduce the autoimmune response and slow the progression of the disease.
   • Vasodilators: For improving blood flow in patients with Raynaud’s phenomenon and reducing the risk of ulcers and digital ischemia.
   • Proton Pump Inhibitors (PPIs): For managing acid reflux and esophageal problems.
   • Calcium Channel Blockers: To treat Raynaud’s and control blood pressure.
   • Endothelin Receptor Antagonists or Phosphodiesterase Inhibitors: For managing pulmonary hypertension.
   • Anti-fibrotic Medications: Nintedanib or pirfenidone to slow the progression of lung fibrosis in patients with interstitial lung disease.
2. Physical Therapy:
   • Customized exercise programs to maintain joint mobility, prevent contractures, and improve flexibility, especially in the hands and feet.
   • Occupational Therapy: To help patients maintain their independence in daily activities despite skin tightening and joint stiffness.
3. Skin Care:
   • Use of moisturizers and emollients to manage dry, tight skin, and reduce discomfort.
   • Topical Treatments: For localized scleroderma (morphea) to soften and improve the appearance of the affected skin.
4. Lifestyle Modifications:
   • Temperature Management: Patients are advised to dress warmly and avoid exposure to cold, which can trigger Raynaud’s attacks.
   • Dietary Adjustments: To manage digestive symptoms, patients may need to eat smaller, more frequent meals, avoid acidic foods, and elevate the head of the bed to reduce acid reflux.
5. Pain Management:
   • NSAIDs and Analgesics: To relieve joint pain and reduce inflammation.
   • Alternative Therapies: Techniques such as acupuncture or biofeedback to help with pain management and improve overall well-being.
6. Specialized Care for Organ Involvement:
   • Pulmonary Rehabilitation: For patients with lung involvement, improving breathing and exercise capacity.
   • Kidney Monitoring: Regular blood pressure checks and kidney function monitoring to prevent or detect scleroderma renal crisis early.

Final Results

With ongoing and individualized care, patients with scleroderma can experience:

• Symptom Relief: Improved skin condition, reduced joint stiffness, and fewer Raynaud’s attacks.
• Organ Protection: Prevention or management of damage to the lungs, heart, kidneys, and digestive system.
• Improved Mobility and Function: Physical therapy and medication help maintain joint mobility and prevent complications like contractures.
• Enhanced Quality of Life: Proper management allows patients to continue leading active, fulfilling lives despite their diagnosis.

Our multidisciplinary approach involves rheumatologists, pulmonologists, cardiologists, and other specialists to deliver personalized care for each patient. By tailoring treatment to the specific needs and progression of scleroderma, we ensure the best possible outcomes, focusing on symptom control and long-term disease management.