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.
Endpoint definition
↥
Endpoint definition steps |
FinnGen |
|---|---|
Phenotype data |
520210 |
1. Apply sex-specific ruleNone |
520210 |
2. Check conditionsNone |
520210 |
3. Check pre-conditions, main-only, mode, registry filtersRegistry filters: 2 out of 7 registries used, show all original rules. |
150 |
4. Check minimum number of eventsNone |
150 |
5. Include endpointsNone |
150 |
6. Filter based on genotype QC (FinnGen only) |
150 |
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
Similar endpoints
↥List of similar endpoints to
Adult-onset Still disease
based on the number of shared cases.
Similar with more cases:
- Other (seronegative) rheumatoid arthritis, wide
- Rheumatoid arthritis
- Rheumatoid arthritis, including avohilmo
- Polyarthropathies
- Autoimmune diseases related-to ILD
Similar with less cases:
None
Case counts by codes
↥
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 | 150 | 92 | 58 |
| Unadjusted period prevalence (%) | 0.03 | 0.03 | 0.03 |
| Median age at first event (years) | 45.82 | 44.32 | 48.21 |
-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
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
- 142
- Matched controls
- 1422
Code
Vocabulary
Description
Odds Ratio
-log10(p)
N matched cases
N matched controls
M06.1
ICD-10 Finland
Adult-onset Still disease
+∞
205.2
142
*
202
Kela drug reimbursment
Connective tissue diseases, rheumatoid arthritis and comparable disease
73.1
86.0
99
43
L04AX03
ATC
methotrexate; systemic (immunosuppressants)
41.3
62.8
81
44
L01BA01
ATC
methotrexate; systemic (folic acid analog.)
74.1
52.4
58
13
P01BA02
ATC
hydroxychloroquine; oral
28.1
44.7
63
39
H02AB06
ATC
prednisolone; systemic
17.4
42.4
125
422
313
Kela drug reimbursment
Abatacept, adalimumab, anakinra, certolizumab pegol, etanercept, golimumab, infliximab, ixekizumab, sarilumab, secukinumab, tocilizumab and ustekinumab (rheumatoid diseases)
67.0
40.9
46
10
TNX10
NOMESCO Finland
Arthrocentesis, unspecified region, unspecified region
27.3
39.1
55
32
R50.9
ICD-10 Finland
Fever, unspecified
14.0
37.1
72
97
281
Kela drug reimbursment
Abatacept, adalimumab, anakinra, certolizumab pegol, etanercept, golimumab, infliximab, ixekizumab, sarilumab, secukinumab and tocilizumab
71.2
36.8
41
8
M06.0
ICD-10 Finland
Seronegative rheumatoid arthritis
43.2
36.7
45
15
A12AX
ATC
Calcium, combinations with vitamin D and/or other drugs
10.6
36.0
96
234
A07EC01
ATC
sulfasalazine; oral, rectal
22.7
33.7
50
33
NK4XE
NOMESCO Finland
Joint ultrasound examination not elsewhere classified
19.0
30.4
48
37
M05.8
ICD-10 Finland
Other seropositive rheumatoid arthritis
21.6
28.2
42
27
M05BA04
ATC
alendronic acid; oral
16.3
26.4
44
38
GD1AA
NOMESCO Finland
Thorax X-ray examination
6.6
24.3
93
318
H02AB07
ATC
prednisone; oral
9.2
21.9
50
79
M08.2
ICD-10 Finland
Juvenile arthritis with systemic onset
+∞
21.4
20
*
L04AC03
ATC
anakinra; parenteral
+∞
20.3
19
*
M13.9
ICD-10 Finland
Arthritis, unspecified
13.0
19.9
36
36
M06.9
ICD-10 Finland
Rheumatoid arthritis, unspecified
26.2
19.0
26
12
L04AX01
ATC
azathioprine; systemic
27.2
18.5
25
11
NK6QA
NOMESCO Finland
Measurement of bone density from tow or more locations with X-ray
12.7
16.1
29
28
L04AA13
ATC
leflunomide; oral
25.7
16.1
22
10
L04AA01
ATC
[U] ciclosporin; systemic
32.9
15.7
20
7
L88
ICPC
Rheumatoid/seropositive arthritis
17.8
15.5
24
16
A49.9
ICD-10 Finland
Bacterial infection, unspecified
7.2
13.2
33
57
JN3AE
NOMESCO Finland
Abdominal ultrasound examination
5.7
12.6
39
89
L04AB04
ATC
adalimumab; parenteral
51.4
12.1
14
*
L04AD01
ATC
ciclosporin; systemic
18.5
12.0
18
11
M08.0
ICD-10 Finland
Juvenile rheumatoid arthritis
130.1
11.6
12
*
NH3DA
NOMESCO Finland
Bilateral foot X-ray examination
22.3
11.4
16
8
ND2DA
NOMESCO Finland
Bilateral hand X-ray examination
27.7
11.4
15
6
7143A
ICD-9 Finland
Rheumatoid arthritis and other inflammatory polyarthropathies, Juvenile chronic polyarthritis[MB STILL]
+∞
10.5
10
*
ND2AA
NOMESCO Finland
Hand and fingers X-ray examination
5.6
10.0
30
65
NF1AA
NOMESCO Finland
Hip X-ray examination
7.2
9.6
23
37
252
Kela drug reimbursment
NA
+∞
9.5
9
*
3053
Kela drug reimbursment
NA
+∞
9.5
9
*
M01CB01
ATC
sodium aurothiomalate; parenteral
17.7
8.7
13
8
A02BC02
ATC
pantoprazole; systemic
3.1
8.7
103
657
NH3BA
NOMESCO Finland
Foot and toes extensive X-ray examination
8.8
8.7
18
23
ZX120
NOMESCO Finland
Intravenous
5.9
8.7
24
47
NF1AE
NOMESCO Finland
Hip joint ultrasound examination
29.6
8.6
11
*
M08.3
ICD-10 Finland
Juvenile polyarthritis (seronegative)
+∞
8.4
8
*
NDG30
NOMESCO Finland
Fusion of wrist, total
47.6
7.8
9
*
L04AC07
ATC
tocilizumab; parenteral
47.6
7.8
9
*
JN6DR
NOMESCO Finland
PET-CT of whole body metabolism
47.6
7.8
9
*
L04AA11
ATC
[U] etanercept; parenteral
84.3
7.5
8
*
NA1EA
NOMESCO Finland
Cervical spine X-ray examination with flexion
84.3
7.5
8
*
M06.8
ICD-10 Finland
Other specified rheumatoid arthritis
84.3
7.5
8
*
L04AB06
ATC
golimumab; parenteral
84.3
7.5
8
*
NH3AA
NOMESCO Finland
Foot and toes X-ray examination
5.3
7.4
22
47
L04AA14
ATC
[U] anakinra; parenteral
+∞
7.3
7
*
L04AB01
ATC
etanercept; parenteral
21.3
7.2
10
5
NB1AA
NOMESCO Finland
Shoulder X-ray examination without contrast
6.3
7.0
18
32
ND2BA
NOMESCO Finland
Hand and fingers extensive X-ray examination
11.8
6.9
12
11
UJD10
NOMESCO Finland
Esophagoscopy, gastroscopy and duodenoscopy
3.0
6.9
46
196
R70.0
ICD-10 Finland
Elevated erythrocyte sedimentation rate
17.8
6.8
10
6
GD1DD
NOMESCO Finland
High resolution CT examination of lungs
9.4
6.7
13
15
K51.9
ICD-10 Finland
Ulcerative colitis, unspecified
73.0
6.5
7
*
NG1AE
NOMESCO Finland
Knee joint ultrasound exmination
73.0
6.5
7
*
M06.4
ICD-10 Finland
Inflammatory polyarthropathy
73.0
6.5
7
*
NG1AA
NOMESCO Finland
Knee X-ray examination without contrast
4.4
6.5
23
60
NG1DA
NOMESCO Finland
X-ray of knees
28.0
6.3
8
*
L04AA17
ATC
[U] adalimumab; parenteral
+∞
6.3
6
*
M46.1
ICD-10 Finland
Sacroiliitis, not elsewhere classified
+∞
6.3
6
*
NK5AT
NOMESCO Finland
Joint injection with ultrasound guidance not elsewhere classified
+∞
6.3
6
*
208
Kela drug reimbursment
Ulcerative colitis and Crohn's disease
+∞
6.3
6
*
O99.1
ICD-10 Finland
Other diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism complicating pregnancy, childbirth and the puerperium
+∞
6.3
6
*
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: 1
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 data