scleroderma: Scleroderma is a rare autoimmune connective tissue disorder characterized by abnormal hardening of the skin and, sometimes, other organs. It is classified into two main forms: localized scleroderma and systemic sclerosis (SSc), the latter comprising three subsets; diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc) and limited SSc (lSSc) (see these terms).
Endpoint definition
↥
						Endpoint definition steps | 
					
						FinnGen | 
				
|---|---|
						Phenotype data | 
					429209 | 
								1. Apply sex-specific ruleNone  | 
							429209 | 
								2. Check conditions
  | 
							688 | 
								3. Check pre-conditions, main-only, mode, registry filtersRegistry filters: 
 1 out of 7 registries used, show all original rules.  | 
							
								 251  | 
						
								4. Check minimum number of eventsNone  | 
							251 | 
								5. Include endpointsNone  | 
							251 | 
										6. Filter based on genotype QC (FinnGen only) | 
									244 | 
Control definitions (FinnGen only)
					Control conditions
					not SYSTSCLE_ICD10
				
			Extra metadata
					First used in FinnGen datafreeze
					DF3
				
			Similar endpoints
↥List of similar endpoints to
		
Systemic sclerosis,strict definition
	based on the number of shared cases.
		
		Broader endpoints:
	
- Systemic sclerosis, icd10
 - Systemic sclerosis (FG)
 - Systemic sclerosis
 - Systemic connective tissue disorders
 - KELA_REIMBURSEMENT_202
 
		
		Narrower endpoints:
	
None
Case counts by codes
↥Summary Statistics
↥-FinRegistry-
Key figures
| All | Female | Male | |
|---|---|---|---|
| Number of individuals | |||
| Whole population | 1154 | 971 | 174 | 
| Only index persons | 1108 | 946 | 162 | 
| Unadjusted period prevalence (%) | |||
| Whole population | 0.02 | 0.03 | 0.00 | 
| Only index persons | 0.03 | 0.03 | 0.01 | 
| Median age at first event (years) | |||
| Whole population | 56.01 | 56.13 | 55.70 | 
| Only index persons | 55.73 | 55.79 | 55.39 | 
-FinnGen-
Key figures
| All | Female | Male | |
|---|---|---|---|
| Number of individuals | 244 | 211 | 33 | 
| Unadjusted period prevalence (%) | 0.08 | 0.09 | 0.02 | 
| Median age at first event (years) | 54.51 | 54.37 | 55.42 | 
-FinRegistry-
Age distribution of first events
-FinnGen-
Age distribution of first events
-FinRegistry-
Year distribution of first events
-FinnGen-
Year distribution of first events
-FinRegistry-
Cumulative Incidence Function
-FinnGen-
Cumulative Incidence Function
Mortality – FinRegistry
↥Relationships between endpoints
↥Index endpoint: SYSTSCLE_STRICT – Systemic sclerosis,strict definition
GWS hits: 1
                
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