Understanding Systemic Scleroderma
Systemic scleroderma, or systemic sclerosis, is an autoimmune disorder causing excessive collagen production, leading to skin hardening and organ fibrosis.
Though rare in children, when it occurs, it can affect the lungs, heart, kidneys, and digestive system in addition to the skin.
Common Signs and Symptoms
- Skin Changes
- Tight, shiny skin — especially on fingers, hands, and face
- Reduced ability to stretch or move joints
- Raynaud’s phenomenon (fingers turning white, blue, then red in cold)
- Digestive Issues
- Difficulty swallowing
- Bloating or reflux due to slowed gut movement
- Lung and Heart Involvement
- Shortness of breath
- Chest pain or palpitations
- General Symptoms
- Fatigue
- Weight loss
- Joint pain and stiffness
Red Flags
- Skin tightening over fingers or face
- Cold-sensitive color changes in fingers
- Shortness of breath or persistent cough
- Difficulty swallowing or heartburn
- Facial mask-like appearance
Diagnosis
- Blood Tests
- ANA and anti-Scl-70 or anti-centromere antibodies
- Imaging and Organ Assessment
- Chest CT scan and echocardiogram for lungs and heart
- GI studies for swallowing difficulties
- Skin and Nailfold Capillaroscopy
Assesses small vessel changes typical of scleroderma.
Treatment and Management
Medications
- Immunosuppressants (methotrexate, mycophenolate mofetil)
- Vasodilators for Raynaud’s symptoms
- Proton pump inhibitors (PPIs) for reflux
Physical Therapy
Helps maintain joint flexibility and prevent contractures.
Regular Monitoring
Because internal organs may be involved silently, routine testing is vital.
Why Early Diagnosis Matters
Prompt recognition and treatment reduce complications and improve quality of life. Early intervention prevents permanent organ damage and enhances survival.
Hope Arthritis Foundation’s Commitment
We champion early recognition and management of complex childhood rheumatic diseases like systemic scleroderma through:
- Education campaigns
- Training healthcare providers
- Connecting families to pediatric rheumatology care
Every child deserves the chance to move, smile, and grow without pain or restriction.
Written by:
Dr. Angela Migowa, MD, MMed, Paediatric Rheumatology, PhD
Pediatric Rheumatologist & Founder, Hope Arthritis Foundation