Is systemic sclerosis the same as scleroderma?

Systemic Sclerosis vs. Scleroderma: Understanding the Distinction

Systemic sclerosis (SSc) is the preferred medical term over scleroderma because scleroderma refers specifically to the cutaneous manifestations, while SSc encompasses the entire multisystem disease that affects both skin and internal organs. 1

Key Distinctions

• The term "scleroderma" specifically relates to the cutaneous manifestations (skin hardening/fibrosis) of the disease, while systemic sclerosis refers to the complete multisystem disorder affecting skin and internal organs 1
• Systemic sclerosis is characterized by a triad of pathogenic factors: vasculopathy, inflammation/autoimmunity, and fibrosis affecting both skin and internal organs 1
• The overall prognosis of systemic sclerosis is strongly influenced by visceral manifestations, not just skin involvement, which is why the term SSc is preferred in clinical guidelines 1

Clinical Classification of Systemic Sclerosis

• Systemic sclerosis is classified into distinct clinical subsets based on the extent of skin involvement 1, 2:
  • Limited cutaneous SSc (lcSSc): Skin fibrosis limited to areas distal to elbows/knees (may include face/neck) 1, 2
  • Diffuse cutaneous SSc (dcSSc): Skin fibrosis affecting both distal and proximal areas including the trunk 1, 2
  • SSc sine scleroderma: Absence of skin fibrosis despite having SSc-related internal organ involvement (1.5-8% of cases) 1, 2, 3

Organ Involvement in Systemic Sclerosis

• Nearly all patients (>95%) present with Raynaud phenomenon, and approximately 50% develop digital ulcers 1, 2
• Gastrointestinal involvement affects nearly 90% of SSc patients, with the esophagus most commonly affected 1
• Interstitial lung disease occurs in 40-75% of patients but is progressive in only 15-18% 1, 2
• Pulmonary arterial hypertension, cardiac involvement, and scleroderma renal crisis are serious complications that significantly impact mortality 1, 2

Diagnostic Considerations

• The modified Rodnan skin score (mRSS) measures skin thickness on a scale of 0-3 at 17 anatomical sites (score range 0-51) 1, 2
• Specific autoantibodies help predict disease subtype and organ involvement patterns 2
• Comprehensive screening for organ involvement is essential for early detection and management 1, 2

Treatment Approach

• Treatment of SSc is primarily organ-based or aimed at disease modification 1
• Recent EULAR recommendations include targeted therapies for key fibrotic manifestations of SSc 1
• Mycophenolate mofetil, nintedanib, rituximab, and tocilizumab are recommended for treating crucial disease manifestations like skin fibrosis and interstitial lung disease 1

Clinical Implications

• Understanding that systemic sclerosis is a multisystem disease rather than just a skin condition (scleroderma) is crucial for appropriate management 4, 5
• Early detection and treatment of organ involvement significantly impacts prognosis 2, 6
• Despite recent improvements in survival, SSc remains the rheumatic disease with the highest morbidity and mortality 1

In clinical practice, the distinction between these terms is important because focusing only on "scleroderma" (skin involvement) might lead to underestimating or overlooking the potentially life-threatening internal organ manifestations that define the full spectrum of systemic sclerosis 1, 6.