Behçet disease

M13_BEHCET

Behcet's syndrome: Bechet disease (BD) is a chronic, relapsing, multisystemic vasculitis characterized by mucocutaneous lesions, as well as articular, vascular, ocular and central nervous system manifestations.

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Endpoint definition

Endpoint definition steps

FinnGen

Phenotype data

520210

1. Apply sex-specific rule

None

520210

2. Check conditions

None

520210

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

Registry filters:

  • Hospital discharge: ICD-10 M35.2
  • Hospital discharge: ICD-9 1361
  • Cause of death: ICD-10 M35.2
  • Cause of death: ICD-9 1361

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

109

4. Check minimum number of events

None

109

5. Include endpoints

None

109

6. Filter based on genotype QC (FinnGen only)

109

Control definitions (FinnGen only)

Control exclude
M13_SYSTCONNECT

Extra metadata

Level in the ICD hierarchy
4
First used in FinnGen datafreeze
DF2
Parent code in ICD-10
M35
Name in latin
Morbus Behçet

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 612 415 187
Only index persons 535 378 157
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 53.29 53.02 54.96
Only index persons 51.23 50.44 53.11

-FinnGen-

Key figures

All Female Male
Number of individuals 109 82 27
Unadjusted period prevalence (%) 0.02 0.03 0.01
Median age at first event (years) 46.74 44.78 52.72

-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
103
Matched controls
1027
Code
Vocabulary
Description
Odds Ratio
-log10(p)
N matched cases
N matched controls
M35.2
ICD-10 Finland
Behçet disease
+∞
137.8
99
*
L04AX01
ATC
azathioprine; systemic
43.2
34.1
42
16
202
Kela drug reimbursment
Connective tissue diseases, rheumatoid arthritis and comparable disease
17.1
27.6
47
48
H02AB06
ATC
prednisolone; systemic
11.0
24.8
85
309
L04AX03
ATC
methotrexate; systemic (immunosuppressants)
17.2
24.7
41
38
K12.00
ICD-10 Finland
Recurrent oral aphthae
52.0
18.1
21
5
A12AX
ATC
Calcium, combinations with vitamin D and/or other drugs
6.3
17.0
62
198
M35.3
ICD-10 Finland
Polymyalgia rheumatica
35.8
14.4
18
6
N77.8*M35.2
ICD-10 Finland
Ulceration of vulva in Behçet's disease
+∞
13.9
13
*
H02AB07
ATC
prednisone; oral
6.6
12.5
35
74
NK4XE
NOMESCO Finland
Joint ultrasound examination not elsewhere classified
11.8
12.0
22
23
TQX10
NOMESCO Finland
Biopsy of skin and subcutaneous tissue
9.7
11.7
24
31
M35.0
ICD-10 Finland
Sicca syndrome [Sjögren]
+∞
11.7
11
*
P01BA02
ATC
hydroxychloroquine; oral
7.9
10.0
23
36
N76.6
ICD-10 Finland
Ulceration of vulva
33.6
9.6
12
*
M35.9
ICD-10 Finland
Systemic involvement of connective tissue, unspecified
+∞
9.5
9
*
L01BA01
ATC
methotrexate; systemic (folic acid analog.)
12.4
9.2
16
15
K12.10
ICD-10 Finland
Stomatitis NOS
54.8
8.8
10
*
L04AA01
ATC
[U] ciclosporin; systemic
54.8
8.8
10
*
L04AD01
ATC
ciclosporin; systemic
30.4
8.7
11
*
M32.9
ICD-10 Finland
Systemic lupus erythematosus, unspecified
+∞
8.4
8
*
TNX10
NOMESCO Finland
Arthrocentesis, unspecified region, unspecified region
8.1
8.2
18
26
GD1AA
NOMESCO Finland
Thorax X-ray examination
3.4
8.2
56
265
M05BA04
ATC
alendronic acid; oral
6.5
8.1
21
39
UJD10
NOMESCO Finland
Esophagoscopy, gastroscopy and duodenoscopy
3.7
7.9
41
157
H20.1
ICD-10 Finland
Chronic iridocyclitis
48.8
7.8
9
*
M13.9
ICD-10 Finland
Arthritis, unspecified
7.6
7.8
18
28
GD1ED
NOMESCO Finland
Extensive high resolution CT examination of lungs
20.3
7.8
11
6
ZX120
NOMESCO Finland
Intravenous
5.9
7.5
21
43
R50.9
ICD-10 Finland
Fever, unspecified
4.2
7.4
30
92
M35.1
ICD-10 Finland
Other overlap syndromes
+∞
7.4
7
*
DM1AA
NOMESCO Finland
Paranasal sinuses X-ray examination without contrast
12.2
7.0
12
11
UJF30
NOMESCO Finland
Ileoscopy through colon
5.3
7.0
21
47
H04.10
ICD-10 Finland
Dry eye syndrome
5.1
7.0
22
52
R1250
NOMESCO Finland
Evaluation of functional capability
18.2
6.9
10
6
N02AX02
ATC
tramadol; systemic, rectal
3.1
6.9
53
265
J05AB11
ATC
valaciclovir; oral
4.5
6.8
25
69
UJF92
NOMESCO Finland
Capsular endoscopy
24.4
6.8
9
*
A07EC01
ATC
sulfasalazine; oral, rectal
5.7
6.8
19
39
R29.8
ICD-10 Finland
Other and unspecified symptoms and signs involving the nervous and musculoskeletal systems
6.3
6.7
17
31
M35.7
ICD-10 Finland
Hypermobility syndrome
74.3
6.5
7
*
Z04.8
ICD-10 Finland
Examination and observation for other specified reasons
4.9
6.5
21
51
M05BA07
ATC
risedronic acid; oral
10.3
6.5
12
13
L88
ICPC
Rheumatoid/seropositive arthritis
11.0
6.2
11
11
J01FF01
ATC
clindamycin; systemic
3.1
6.1
39
169

Mortality – FinRegistry

Association

Association between endpoint M13_BEHCET and mortality.

Females

Parameter HR [95% CI] p-value
M13_BEHCET 1.717 [1.24, 2.37] 0.001
Birth year 0.994 [0.99, 1.0] 0.166

During the follow-up period (1.1.1998 — 31.12.2019), 98 out of 370 females with M13_BEHCET died.

Males

No data

Mortality risk

Mortality risk for people of age

years, who have M13_BEHCET.

N-year risk Females Males
1 0.128% No data
5 0.803% No data
10 2.061% No data
15 4.356% No data
20 7.527% No data

Relationships between endpoints

Index endpoint: M13_BEHCET – Behçet 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
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