Terminology: Scleroderma vs. Systemic Sclerosis
The term "systemic sclerosis" (SSc) is preferred over "scleroderma" because scleroderma refers specifically to the cutaneous (skin) manifestations, while systemic sclerosis encompasses the entire multisystem disease affecting both skin and internal organs. 1, 2
Key Distinction
Scleroderma describes only the skin hardening and thickening that occurs in this disease 1, 2. The term literally means "hard skin" and focuses on the dermatologic manifestations alone 3.
Systemic sclerosis is the comprehensive term that captures the full disease spectrum, including 1:
- Skin fibrosis (scleroderma)
- Vascular manifestations (vasculopathy)
- Internal organ involvement (lungs, heart, kidneys, gastrointestinal tract)
- Autoimmune features
Why This Matters Clinically
The distinction is clinically critical because overall prognosis is strongly influenced by visceral (internal organ) manifestations, not just skin involvement. 1 The main causes of death in systemic sclerosis are interstitial lung disease and cardiac involvement, not the skin changes 1.
The European League Against Rheumatism (EULAR) explicitly recommends using "systemic sclerosis" in clinical practice and guidelines because 1, 2:
- It emphasizes the multisystem nature of the disease
- It directs clinical attention to life-threatening organ complications
- It prevents underestimation of disease severity based solely on skin findings
Clinical Subsets Within Systemic Sclerosis
Systemic sclerosis itself is classified into subsets based on the extent of skin involvement (scleroderma), but all represent systemic disease 1, 4:
- Limited cutaneous SSc (lcSSc): Skin fibrosis distal to elbows/knees only 1, 4
- Diffuse cutaneous SSc (dcSSc): Skin involvement both distal and proximal to elbows/knees, including trunk 1, 4
- SSc sine scleroderma: Major internal organ complications without definite skin involvement (1.5-8% of cases) 1, 4
Even patients without visible scleroderma can have life-threatening systemic sclerosis affecting internal organs. 1, 4
Common Pitfall to Avoid
Do not use "scleroderma" and "systemic sclerosis" interchangeably in documentation or when discussing prognosis with patients 1. Using "scleroderma" alone may inadvertently minimize the serious systemic nature of the disease and delay appropriate screening for organ involvement 1, 2. The term systemic sclerosis better reflects that nearly 90% of patients have gastrointestinal involvement, 40-75% develop interstitial lung disease, and the disease carries the highest mortality among rheumatic diseases 1, 4.