ICD-11

Systemic sclerosis is a systemic disorder of the connective tissue; manifested by hardening and thickening of the skin, by abnormalities involving the microvasculature and larger vessels, and by fibrotic degenerative changes in various body organs including the heart, lungs, kidneys, and gastrointestinal tract.

Systemic sclerosis arising before the age of 16. Involvement of internal organs is less common but arthritis and myositis are more common than in adults.