ICD-11 classes
04 Diseases of the immune system
Nonorgan specific systemic autoimmune disorders
4A42 — Systemic sclerosis
ICD-11 4A42 — Systemic sclerosis
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. (Arthritis Rheum 1980; 23:581-590)
It includes 1 item.
- Systemic scleroderma
It excludes 1 item.
- Circumscribed scleroderma (EB61.0)
Diagnosis with code 4A42 contains 4 clarifying diagnoses:
- 4A42.0 — Paediatric onset systemic sclerosis
- 4A42.1 — Diffuse systemic sclerosis
- 4A42.2 — Limited systemic sclerosis
- 4A42.Z — Systemic sclerosis, unspecified
The diagnosis is coded elsewhere: