Scleroderma
Also known as: Systemic Sclerosis, SSc
An autoimmune condition causing skin thickening and potential internal organ fibrosis. Ranges from limited skin involvement to diffuse systemic disease requiring close monitoring.
Key Facts
- Raynaud's phenomenon is often the first symptom, appearing years before other features
- Limited cutaneous scleroderma (formerly CREST) affects skin of hands and face only
- Diffuse cutaneous scleroderma involves widespread skin and earlier organ involvement
- Interstitial lung disease and pulmonary hypertension are leading causes of mortality
- Annual screening for lung and heart involvement is essential
What is scleroderma?
Scleroderma (systemic sclerosis) is a chronic autoimmune disease characterized by thickening and tightening of the skin and connective tissue. In its systemic forms, it can affect internal organs including the lungs, heart, kidneys, and gastrointestinal tract through fibrosis (scarring).
Scleroderma exists on a spectrum:
- Limited cutaneous scleroderma (formerly CREST syndrome) — skin thickening confined to hands, face, and forearms; slower progression; associated with pulmonary hypertension risk
- Diffuse cutaneous scleroderma — widespread skin thickening with earlier and more frequent internal organ involvement
- Systemic sclerosis sine scleroderma — internal organ involvement without significant skin thickening (rare)
Symptoms
- Raynaud's phenomenon — often the earliest symptom
- Skin thickening and tightening, starting in fingers (sclerodactyly)
- Puffy, swollen fingers
- Difficulty opening mouth fully (microstomia)
- Acid reflux and swallowing difficulty (esophageal dysmotility)
- Shortness of breath (pulmonary fibrosis or pulmonary hypertension)
- Calcinosis — calcium deposits under the skin
- Telangiectasias — small, visible blood vessels on the face and hands
- Digital ulcers — painful sores on fingertips
How we diagnose it
- Clinical exam — Skin thickening assessment using the modified Rodnan skin score, Raynaud's evaluation
- Blood work — ANA, anti-centromere antibodies (limited disease), anti-Scl-70/anti-topoisomerase I (diffuse disease), anti-RNA polymerase III
- Nailfold capillaroscopy — Abnormal capillary patterns characteristic of scleroderma
- Pulmonary function tests — Screening for interstitial lung disease
- Echocardiogram — Screening for pulmonary hypertension
- CT chest — High-resolution imaging when lung involvement is suspected
How we treat it
- Raynaud's management — Calcium channel blockers, PDE-5 inhibitors
- Immunosuppression for lung disease — Mycophenolate, cyclophosphamide, or nintedanib for progressive pulmonary fibrosis
- GI management — Proton pump inhibitors, prokinetic agents for reflux and dysmotility
- Pulmonary hypertension — Referral for specialized treatment (endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogs)
- Kidney monitoring — Blood pressure monitoring; scleroderma renal crisis requires urgent management with ACE inhibitors
- Skin and joint — Physical therapy for joint contractures, skin moisturizers
At Synergy Rheumatology
Scleroderma requires ongoing surveillance across multiple organ systems. We coordinate with pulmonology, cardiology, and gastroenterology as needed, manage immunosuppressive therapy, and monitor for complications. Dr. Fellows has a keen interest in interstitial lung disease intersecting with connective tissue diseases, making our practice well-suited for this condition.
Have questions about scleroderma?
Schedule an appointment to discuss your symptoms, diagnosis, or treatment options with Dr. Fellows.