Adult-onset Still disease

STILL_ADULT

adult-onset Still's disease: adult-onset Still disease (AOSD) is a rare inflammatory multisystem disorder characterized clinically by fever of unknown origin, arthralgia or arthritis, hyperleucocytosis, and typical skin rash.

Suggest a new description

Endpoint definition

Endpoint definition steps

FinnGen

Phenotype data

473681

1. Apply sex-specific rule

None

473681

2. Check conditions

None

473681

3. Check pre-conditions, main-only, mode, registry filters

Registry filters:

  • Hospital discharge: ICD-10 M06.1
  • Cause of death: ICD-10 M06.1

2 out of 7 registries used, show all original rules.

142

4. Check minimum number of events

None

142

5. Include endpoints

None

142

6. Filter based on genotype QC (FinnGen only)

134

Control definitions (FinnGen only)

Control exclude ILD_SYST_AUTO_DISEASES

Extra metadata

First used in FinnGen datafreeze DF5
Parent code in ICD-10 M06
Name in latin Arthritis rheumatoides seronegativa

Case counts by codes

FinnGen case counts by registry codes:

generic upset plot

Upset plot

Full data table

Summary Statistics

-FinRegistry-

Key figures

All Female Male
Number of individuals
Whole population 695 385 303
Only index persons 637 353 284
Unadjusted period prevalence (%)
Whole population 0.01 0.01 0.01
Only index persons 0.01 0.01 0.01
Median age at first event (years)
Whole population 49.30 49.81 48.68
Only index persons 47.00 47.43 46.47

-FinnGen-

Key figures

All Female Male
Number of individuals 134 83 51
Unadjusted period prevalence (%) 0.03 0.03 0.03
Median age at first event (years) 46.09 43.78 49.85

-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

No data

Mortality – FinRegistry

Association

Association between endpoint STILL_ADULT and mortality.

Females

Parameter HR [95% CI] p-value
STILL_ADULT 1.988 [1.4, 2.83] < 0.001
Birth year 0.999 [0.99, 1.01] 0.737

During the follow-up period (1.1.1998 — 31.12.2019), 53 out of 328 females with STILL_ADULT died.

Males

Parameter HR [95% CI] p-value
STILL_ADULT 1.642 [1.18, 2.28] 0.003
Birth year 0.988 [0.98, 1.0] 0.009

During the follow-up period (1.1.1998 — 31.12.2019), 57 out of 253 males with STILL_ADULT died.

Mortality risk

Mortality risk for people of age

years, who have STILL_ADULT.

N-year risk Females Males
1 0.161% 0.335%
5 1.301% 1.57%
10 3.268% 4.225%
15 6.061% 8.388%
20 10.759% 14.54%

Relationships between endpoints

Index endpoint: STILL_ADULT – Adult-onset Still disease

GWS hits: -

Endpoint
Case Overlap
Survival Analysis
Genetic Correlations
Genetic Signals
N (Jaccard index)
HR [CI]
Extremity
rg [CI]
Extremity
Hits
Coloc Hits
FinRegistry
FinnGen
FinRegistry
FinnGen
FinnGen
loading spinner Loading data