Systemic sclerosis

Description

systemic scleroderma: A chronic disorder, possibly autoimmune, marked by excessive production of collagen which results in hardening and thickening of body tissues. The two types of systemic scleroderma, limited cutaneous and diffuse cutaneous are classified with focus on the extent of affected skin. A relationship exists between the extent of skin area affected and degree of internal organ/system involvement. Systemic scleroderma can manifest itself in pulmonary fibrosis, Raynaud's syndrome, digestive system telangiectasias, renal hypertension and/or pulmonary hypertension.

Data source
FinnGen phenocode M13_SYSTSLCE
Hospital Discharge registry ICD-10: M34, ICD-8: 7340
Cause of Death registry ICD-10: M34, ICD-8: 7340
Level in the ICD-hierarchy 4
First defined in version DF2
Ontology
DOID 418
GWAS catalog 0000717
MESH D012595
SNOMED CT 89155008

Key figures

Sex All Female Male
Number of individuals 170 138 32
Unadjusted prevalence (%) 0.13 0.18 0.06
Mean age at first event (years) 53.95 54.3 52.45
Median number of events / individual 4 4 2

Clinical metrics

Sex All Female Male
Recurrence within 6 months (%) 69.41 72.46 56.25
Case fatality at 5-years (%) 7.65 6.52 12.5

Associations