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 |
520210 |
1. Apply sex-specific ruleNone |
520210 |
2. Check conditions
|
867 |
3. Check pre-conditions, main-only, mode, registry filtersRegistry filters:
1 out of 7 registries used, show all original rules. |
323 |
4. Check minimum number of eventsNone |
323 |
5. Include endpointsNone |
323 |
6. Filter based on genotype QC (FinnGen only) |
323 |
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.
Similar with more cases:
- Systemic sclerosis, icd10
- Systemic sclerosis (FG)
- Systemic sclerosis
- Systemic connective tissue disorders
- Reimbursement for rheumatic diseases
Similar with less cases:
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 | 323 | 280 | 43 |
Unadjusted period prevalence (%) | 0.09 | 0.10 | 0.02 |
Median age at first event (years) | 54.65 | 54.38 | 56.42 |
-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
CodeWAS (R11)
↥CodeWAS is a tool for exploring the associations between an endpoint and all of the medical codes and drug codes.
This is a new tool, please reach out using the contact form for feedback and improvement ideas.
First, a cohort is built by matching controls to the endpoint cases using year of birth and sex. Then, a Fisher test is done for all the medical codes and drug codes between the cases and controls of this cohort. Codes are reported in the table below if they have −log10(p-value) ≥ 6.
Matched cohort
- Matched cases
- 270
- Matched controls
- 2700
LabWAS
↥Mortality – FinRegistry
↥Association
Association between endpoint SYSTSCLE_STRICT and mortality.
Females
Parameter | HR [95% CI] | p-value |
---|---|---|
SYSTSCLE_STRICT | 2.104 [1.69, 2.63] | < 0.001 |
Birth year | 0.998 [0.99, 1.01] | 0.582 |
During the follow-up period (1.1.1998 — 31.12.2019), 108 out of 823 females with SYSTSCLE_STRICT died.
Males
No dataMortality risk
Mortality risk for people of age
years, who have SYSTSCLE_STRICT.N-year risk | Females | Males |
---|---|---|
1 | 0.201% | No data |
5 | 1.155% | No data |
10 | 3.086% | No data |
15 | 6.121% | No data |
20 | 10.651% | No data |
Relationships between endpoints
↥Index endpoint: SYSTSCLE_STRICT – Systemic sclerosis,strict definition
GWS hits: -