Rheumatology
Systemic Sclerosis
전신경화증
Progressive skin and visceral fibrosis with vasculopathy
- How common
- 50–300 per 100,000
- Typical age
- Ages 30–50 (4:1 female)
What is it?
Endothelial injury and immune activation drive fibroblast hyperactivity and excessive collagen deposition in skin and viscera.
Commonly affected: Skin, lungs, GI, kidneys, heart
How it develops
- Endothelial injuryMicrovascular damage and ischemia
- Autoimmune activationT cells, B cells, autoantibody production
- Fibroblast activationTGF-β/CTGF drive excess collagen synthesis
- Fibrosis & organ failureSkin fibrosis, lung fibrosis, scleroderma renal crisis
Symptoms
- Skin thickeningSclerodactyly progressing proximally
- Lung fibrosis/PAHExertional dyspnea, ILD or PAH
- Raynaud phenomenonCold/stress-induced 3-color finger change
- Esophageal dysmotilityDysphagia, reflux, strictures
- Nailfold capillary changesAbnormal nailfold capillaries
How it is examined
- mRSS skin score17-site skin thickness scoring (0–3)
- Autoantibody panelANA, anti-Scl70 (diffuse), anti-centromere (limited), anti-RNA Pol III (renal crisis)
- PFT + HRCTDLCO, FVC; HRCT for ILD evaluation
Imaging
Hand X-ray shows acro-osteolysis and soft tissue calcinosis.
- Acro-osteolysis
- Soft tissue calcinosis
- Joint space narrowing
Chest HRCT shows NSIP pattern ILD; echo screens for PAH.
- NSIP pattern
- Ground-glass opacity
- Pulmonary artery dilation
Non-surgical care
- Raynaud managementWarmth, CCB (nifedipine)
- Reflux controlPPI, head-of-bed elevation
- MMF (skin/ILD)Slows skin fibrosis and ILD progression
- ACE inhibitor (renal crisis)First-line for renal crisis, start immediately
- PAH-targeted therapyBosentan, sildenafil, riociguat
Conservative options are generally tried first. Medications listed here can have side effects — discuss them with your prescriber.
When surgery is considered
End-stage organ failure or digital ulcers
Procedures that may be discussed
- Cyclophosphamide (severe ILD)
- Tocilizumab (skin/ILD)
- Nintedanib (progressive ILD)
- Autologous HSCT (selected)
- Lung transplant
Outlook
Diffuse 5-year survival 70%, limited 90%+. ILD, PAH, renal crisis drive mortality.