Churg-Strauss syndrome: Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a systemic vasculitis of small-to medium vessels, characterized by asthma, transient pulmonary infiltrates, and hypereosinophilia.
Polyarteritis with lung involvement [Churg-Strauss]/EGPA
DOID EFO Endpoint Browser PheWeb
M13_CHURGSTRAUSS
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. |
140 |
4. Check minimum number of eventsNone |
140 |
5. Include endpointsNone |
140 |
6. Filter based on genotype QC (FinnGen only) |
140 |
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
- M30
- Name in latin
- Polyarteritis nodosa cum manifestationibus pulmonalibus
Similar endpoints
↥List of similar endpoints to
Polyarteritis with lung involvement [Churg-Strauss]/EGPA
based on the number of shared cases.
Similar with more cases:
- Polyarteritis nodosa and related conditions
- Systemic connective tissue disorders
- Autoimmune diseases related-to ILD
- Autoimmune diseases
- Used for control exclusion in common control thyroid runs
Similar with less cases:
None
Case counts by codes
↥
Summary Statistics
↥-FinRegistry-
Key figures
| All | Female | Male | |
|---|---|---|---|
| Number of individuals | |||
| Whole population | 674 | 428 | 243 |
| Only index persons | 564 | 368 | 196 |
| 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 | 59.17 | 58.51 | 60.28 |
| Only index persons | 57.40 | 56.70 | 58.70 |
-FinnGen-
Key figures
| All | Female | Male | |
|---|---|---|---|
| Number of individuals | 140 | 93 | 47 |
| Unadjusted period prevalence (%) | 0.03 | 0.03 | 0.02 |
| Median age at first event (years) | 53.71 | 52.24 | 56.63 |
-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
- 124
- Matched controls
- 1239
Code
Vocabulary
Description
Odds Ratio
-log10(p)
N matched cases
N matched controls
M30.1
ICD-10 Finland
Polyarteritis with lung involvement [Churg-Strauss]
+∞
175.9
123
*
H02AB06
ATC
prednisolone; systemic
28.3
40.1
116
420
D72.1
ICD-10 Finland
Eosinophilia
467.5
35.9
34
*
203
Kela drug reimbursment
Chronic asthma and similar chronic obstructive pulmonary diseases
11.8
33.2
77
151
L04AX01
ATC
azathioprine; systemic
33.3
31.4
41
18
J45.9
ICD-10 Finland
Asthma, unspecified
10.0
28.7
71
146
202
Kela drug reimbursment
Connective tissue diseases, rheumatoid arthritis and comparable disease
13.2
28.1
54
68
NK6QA
NOMESCO Finland
Measurement of bone density from tow or more locations with X-ray
18.1
26.2
42
34
J45.1
ICD-10 Finland
Nonallergic asthma
15.6
25.6
44
42
R03DC03
ATC
montelukast; oral
9.3
25.4
63
124
A12AX
ATC
Calcium, combinations with vitamin D and/or other drugs
7.9
25.1
85
268
R03AC02
ATC
salbutamol; inhalant
6.7
19.2
100
476
J45.0
ICD-10 Finland
Predominantly allergic asthma
9.7
18.4
40
58
DMB20
NOMESCO Finland
Functional endoscopic opening of maxillary antrum
12.1
17.6
33
36
R03AK06
ATC
salmeterol and fluticasone; inhalant
7.1
17.3
48
101
J32.4
ICD-10 Finland
Chronic pansinusitis
33.0
17.2
22
8
DM1AA
NOMESCO Finland
Paranasal sinuses X-ray examination without contrast
18.3
15.7
24
16
R01AD08
ATC
fluticasone; nasal
5.0
15.6
68
242
J32.0
ICD-10 Finland
Chronic maxillary sinusitis
9.2
15.5
34
49
R03AK08
ATC
formoterol and beclometasone; inhalant
15.3
15.2
25
20
L04AX03
ATC
methotrexate; systemic (immunosuppressants)
8.4
15.2
35
55
DNB20
NOMESCO Finland
Endoscopic ethmoidectomy
18.5
15.1
23
15
GD1AA
NOMESCO Finland
Thorax X-ray examination
4.7
15.0
83
372
R03AC12
ATC
salmeterol; inhalant
12.5
14.9
27
27
J82
ICD-10 Finland
Pulmonary eosinophilia, not elsewhere classified
+∞
14.9
14
*
M05BA04
ATC
alendronic acid; oral
8.8
14.4
32
47
J01MA14
ATC
moxifloxacin; systemic
8.4
14.0
32
49
R03AK07
ATC
formoterol and budesonide; inhalant
5.2
13.6
50
143
UDH02
NOMESCO Finland
Rhinopharyngoscopy
8.4
13.6
31
47
UGC12
NOMESCO Finland
Flexible bronchoscopy
9.2
13.1
28
38
R03BA02
ATC
budesonide; inhalant
5.2
12.8
46
127
R50.9
ICD-10 Finland
Fever, unspecified
6.0
12.7
38
85
J33.0
ICD-10 Finland
Polyp of nasal cavity
13.1
12.6
22
20
GD1DD
NOMESCO Finland
High resolution CT examination of lungs
11.0
12.1
23
25
XG474
NOMESCO Finland
Exhalation nntrogen oxide
13.0
12.1
21
19
N30.1
ICD-10 Finland
Interstitial cystitis (chronic)
33.8
12.0
15
5
R03BB04
ATC
tiotropium bromide; inhalant
6.9
11.9
31
57
M31.1
ICD-10 Finland
Thrombotic microangiopathy
+∞
11.6
11
*
J45.8
ICD-10 Finland
Mixed asthma
13.0
11.5
20
18
J20.9
ICD-10 Finland
Acute bronchitis, unspecified
4.7
11.5
45
135
R03BA05
ATC
fluticasone; inhalant
4.5
11.2
45
138
TDM10
NOMESCO Finland
Puncture of maxillary antrum
10.0
11.1
22
26
N02AX02
ATC
tramadol; systemic, rectal
3.7
11.1
74
351
J18.9
ICD-10 Finland
Pneumonia, unspecified
4.3
10.8
46
148
R03BA08
ATC
ciclesonide; inhalant
6.4
10.7
29
56
M31.3
ICD-10 Finland
Wegener granulomatosis
+∞
10.6
10
*
J33.8
ICD-10 Finland
Other polyp of sinus
14.9
10.5
17
13
NK4XE
NOMESCO Finland
Joint ultrasound examination not elsewhere classified
9.5
10.3
21
26
H02AB07
ATC
prednisone; oral
4.6
10.3
40
117
WZC30
NOMESCO Finland
Teaching
5.2
10.3
34
84
XG412
NOMESCO Finland
Flow-volume spirometry with bronchospasmolysis
7.2
10.2
25
42
DHB20
NOMESCO Finland
Polypectomy of internal nose
22.3
10.1
14
7
D69.0
ICD-10 Finland
Allergic purpura
107.9
9.6
10
*
M31.9
ICD-10 Finland
Necrotizing vasculopathy, unspecified
+∞
9.5
9
*
M30.0
ICD-10 Finland
Polyarteritis nodosa
+∞
9.5
9
*
R03BA01
ATC
beclometasone; inhalant
4.6
9.5
35
97
TGC00
NOMESCO Finland
Bronchial lavation
14.0
9.1
15
12
UDM02
NOMESCO Finland
Sinoscopy
14.0
9.1
15
12
H02AB04
ATC
methylprednisolone; systemic
4.2
8.8
37
115
R03DA04
ATC
theophylline; systemic, rectal
7.7
8.8
20
30
L04AA06
ATC
mycophenolic acid; systemic
21.9
8.7
12
6
J45
ICD-10 Finland
Asthma
5.1
8.6
28
67
L95.9
ICD-10 Finland
Vasculitis limited to skin, unspecified
96.3
8.5
9
*
R01AD01
ATC
beclometasone; nasal
4.2
8.4
34
102
A02BC02
ATC
pantoprazole; systemic
3.4
8.3
97
637
R03AC13
ATC
formoterol; inhalant
7.0
8.3
20
33
L01AA01
ATC
cyclophosphamide; systemic
35.9
8.2
10
*
DHA10
NOMESCO Finland
Biopsy of cavity of nose
23.9
8.2
11
5
ZX120
NOMESCO Finland
Intravenous
4.5
8.2
30
82
GD1ED
NOMESCO Finland
Extensive high resolution CT examination of lungs
10.5
8.0
15
16
J01MA12
ATC
levofloxacin; systemic
3.8
8.0
37
125
DM1QA
NOMESCO Finland
Paranasal sinuses X-ray examination in one projection
6.6
7.9
20
35
XG410
NOMESCO Finland
Flow-volume spirometry
7.6
7.9
18
27
DM1AI
NOMESCO Finland
Cone beam CT of paranasal sinus
13.0
7.8
13
11
Z01.8
ICD-10 Finland
Other specified special examinations
3.3
7.8
45
181
DPA30
NOMESCO Finland
Sphenotomy
26.9
7.7
10
*
J01FA06
ATC
roxithromycin; oral
2.9
7.6
68
365
M31.0
ICD-10 Finland
Hypersensitivity angiitis
+∞
7.4
7
*
M30.8
ICD-10 Finland
Other conditions related to polyarteritis nodosa
+∞
7.4
7
*
M35.9
ICD-10 Finland
Systemic involvement of connective tissue, unspecified
+∞
7.4
7
*
J01EE01
ATC
sulfamethoxazole and trimethoprim; systemic
5.9
7.3
20
39
R01AD09
ATC
mometasone; nasal
2.8
7.3
71
397
TQX10
NOMESCO Finland
Biopsy of skin and subcutaneous tissue
4.5
7.3
26
69
KCV04
NOMESCO Finland
Distension of bladder with fluid
21.6
7.3
10
5
GX1L5
NOMESCO Finland
Exhaled nitric oxide analysis
32.1
7.3
9
*
A01AB09
ATC
miconazole; local oral
5.2
7.2
22
49
ZY020
NOMESCO Finland
Telephone, letter or other consultation without direct patient contact
5.8
7.2
20
40
A02BC01
ATC
omeprazole; systemic
3.0
7.2
50
227
FM1EE
NOMESCO Finland
Extensive structural and functional ultrasound examination of heart
3.3
7.0
41
163
R91
ICD-10 Finland
Abnormal findings on diagnostic imaging of lung
4.6
7.0
24
61
R03AK11
ATC
formoterol and fluticasone; inhalant
11.9
7.0
12
11
R03DX09
ATC
mepolizumab; parenteral
42.4
6.8
8
*
297
Kela drug reimbursment
NA
42.4
6.8
8
*
3028
Kela drug reimbursment
Mepolizumab
42.4
6.8
8
*
J15.9
ICD-10 Finland
Bacterial pneumonia, unspecified
5.6
6.7
19
39
J46
ICD-10 Finland
Status asthmaticus
10.9
6.7
12
12
DM1AD
NOMESCO Finland
CT of paranasal sinuses and face area
8.2
6.6
14
19
J01FF01
ATC
clindamycin; systemic
3.0
6.5
46
206
R03DC01
ATC
zafirlukast; oral
11.9
6.4
11
10
B01AA03
ATC
warfarin; systemic
3.4
6.4
32
114
4939X
ICD-9 Finland
Asthma, unspecified[ASTHMA BRONCHIALE NUD]
19.2
6.4
9
5
P01BA02
ATC
hydroxychloroquine; oral
5.5
6.4
18
37
J01CR02
ATC
amoxicillin and beta-lactamase inhibitor; systemic
2.7
6.4
78
483
M80.4
ICD-10 Finland
Drug-induced osteoporosis with pathological fracture
+∞
6.3
6
*
I77.6
ICD-10 Finland
Arteritis, unspecified
+∞
6.3
6
*
R06AX22
ATC
ebastine; oral
3.3
6.1
33
123
R03AC03
ATC
terbutaline; inhalant
3.7
6.1
27
87
ZXA10
NOMESCO Finland
Bilateral
3.2
6.1
34
131
Mortality – FinRegistry
↥Association
Association between endpoint M13_CHURGSTRAUSS and mortality.
Females
| Parameter | HR [95% CI] | p-value |
|---|---|---|
| M13_CHURGSTRAUSS | 1.776 [1.31, 2.4] | < 0.001 |
| Birth year | 0.997 [0.99, 1.01] | 0.509 |
During the follow-up period (1.1.1998 — 31.12.2019), 95 out of 338 females with M13_CHURGSTRAUSS died.
Males
| Parameter | HR [95% CI] | p-value |
|---|---|---|
| M13_CHURGSTRAUSS | 1.771 [1.23, 2.56] | 0.002 |
| Birth year | 0.988 [0.98, 1.0] | 0.009 |
During the follow-up period (1.1.1998 — 31.12.2019), 54 out of 192 males with M13_CHURGSTRAUSS died.
Mortality risk
Mortality risk for people of age
years, who have M13_CHURGSTRAUSS.| N-year risk | Females | Males |
|---|---|---|
| 1 | 0.195% | 0.339% |
| 5 | 1.128% | 2.094% |
| 10 | 2.675% | 5.255% |
| 15 | 5.195% | 9.979% |
| 20 | 9.422% | 16.502% |
Relationships between endpoints
↥Index endpoint: M13_CHURGSTRAUSS – Polyarteritis with lung involvement [Churg-Strauss]/EGPA
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
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