Secondary systemic amyloidosis

E4_AMYL_SCNDSYST

amyloidosis: A disorder characterized by the localized or diffuse accumulation of amyloid protein in various anatomic sites. It may be primary, due to clonal plasma cell proliferations; secondary, due to long standing infections, chronic inflammatory disorders, or malignancies; or familial. It may affect the nerves, skin, tongue, joints, heart, liver, spleen, kidneys and adrenal glands.

Suggest a new description

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 E85.3
  • Cause of death: ICD-10 E85.3

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

105

4. Check minimum number of events

None

105

5. Include endpoints

None

105

6. Filter based on genotype QC (FinnGen only)

105

Control definitions (FinnGen only)

Control exclude
E4_METABOLIA

Extra metadata

Level in the ICD hierarchy
4
First used in FinnGen datafreeze
DF2
Parent code in ICD-10
E85
Name in latin
Amyloidosis systemica secundaria

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 1290 873 392
Only index persons 670 472 198
Unadjusted period prevalence (%)
Whole population 0.02 0.03 0.01
Only index persons 0.01 0.02 0.01
Median age at first event (years)
Whole population 66.38 66.66 65.34
Only index persons 64.41 64.70 63.72

-FinnGen-

Key figures

All Female Male
Number of individuals 105 63 42
Unadjusted period prevalence (%) 0.02 0.02 0.02
Median age at first event (years) 62.28 60.49 64.96

-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
98
Matched controls
979
Code
Vocabulary
Description
Odds Ratio
-log10(p)
N matched cases
N matched controls
E85.3
ICD-10 Finland
Secondary systemic amyloidosis
+∞
133.0
95
*
202
Kela drug reimbursment
Connective tissue diseases, rheumatoid arthritis and comparable disease
50.4
56.3
74
56
M05.8
ICD-10 Finland
Other seropositive rheumatoid arthritis
57.0
53.2
64
31
7140A
ICD-9 Finland
Rheumatoid arthritis and other inflammatory polyarthropathies, Rheumatoid arthritis[ARTHRITIS RHEUMATOIDES SEROPOSITIVA]
110.7
45.4
47
8
L01BA01
ATC
methotrexate; systemic (folic acid analog.)
66.2
44.2
50
15
71238
ICD-8 Finland
Rheumatoid arthritis and allied conditions, Arthritis rheumatoides alia definita
172.9
41.6
41
*
N08.4*E85.9
ICD-10 Finland
Glomerular disorders in amyloidosis
+∞
39.2
35
*
E85.9
ICD-10 Finland
Amyloidosis, unspecified
+∞
38.0
34
*
138
Kela drug reimbursment
Severe anaemia associated with chronic renal failure
69.7
29.7
33
7
A11CC03
ATC
alfacalcidol; systemic
135.2
28.4
29
*
N18.9
ICD-10 Finland
Chronic kidney disease, unspecified
27.3
27.9
39
23
71239
ICD-8 Finland
Rheumatoid arthritis and allied conditions, Arthritis rheumatoides NUD
101.4
27.5
29
*
123
Kela drug reimbursment
Chronic disorders of vitamin D metabolism
70.9
27.1
30
6
M05.9
ICD-10 Finland
Seropositive rheumatoid arthritis, unspecified
46.5
26.7
32
10
TNX10
NOMESCO Finland
Arthrocentesis, unspecified region, unspecified region
25.0
26.5
38
24
L04AX01
ATC
azathioprine; systemic
28.2
24.9
34
18
M06.9
ICD-10 Finland
Rheumatoid arthritis, unspecified
29.5
23.1
31
15
A07EC01
ATC
sulfasalazine; oral, rectal
17.3
22.5
37
33
L04AA01
ATC
[U] ciclosporin; systemic
55.0
21.7
25
6
A12AA04
ATC
calcium carbonate; oral
93.3
20.7
22
*
N08.4
ICD-10 Finland
Glomerular disorders in other endocrine, nutritional and metabolic diseases
+∞
20.5
19
*
2773B
ICD-9 Finland
Other and unspecified disorders of metabolism, Amyloidosis[AMYLOIDOSIS SECUNDARIA]
+∞
20.5
19
*
313
Kela drug reimbursment
Abatacept, adalimumab, anakinra, certolizumab pegol, etanercept, golimumab, infliximab, ixekizumab, sarilumab, secukinumab, tocilizumab and ustekinumab (rheumatoid diseases)
34.7
20.4
26
10
TK800
NOMESCO Finland
Hemodialysis
59.3
20.2
23
5
Z49.1
ICD-10 Finland
Extracorporeal dialysis
69.9
19.9
22
*
C03CA01
ATC
furosemide; systemic
7.7
19.6
66
207
PBL10
NOMESCO Finland
Construction of arteriovenous fistula from radial artery
124.2
19.4
20
*
L04AX03
ATC
methotrexate; systemic (immunosuppressants)
12.9
19.2
36
42
71200
ICD-8 Finland
Rheumatoid arthritis and allied conditions, Juvenile rheumatoid arthritis
+∞
18.3
17
*
M08.0
ICD-10 Finland
Juvenile rheumatoid arthritis
+∞
18.3
17
*
L88
ICPC
Rheumatoid/seropositive arthritis
23.9
17.1
24
13
N18.0
ICD-10 Finland
End-stage renal disease
58.2
16.7
19
*
281
Kela drug reimbursment
Abatacept, adalimumab, anakinra, certolizumab pegol, etanercept, golimumab, infliximab, ixekizumab, sarilumab, secukinumab and tocilizumab
32.9
16.4
21
8
B03XA01
ATC
erythropoietin; parenteral
188.9
16.0
16
*
M01CB01
ATC
sodium aurothiomalate; parenteral
54.4
15.7
18
*
L04AA13
ATC
leflunomide; oral
21.4
15.2
22
13
9283
FHL
Subtalar joint ossification surgery (or triple)
175.0
14.9
15
*
NK4XE
NOMESCO Finland
Joint ultrasound examination not elsewhere classified
11.1
14.3
28
34
D06BB04
ATC
podophyllotoxin; topical
20.2
14.2
21
13
TPH15
NOMESCO Finland
Insertion of central venous catheter through external or internal jugular vein
17.3
14.1
22
16
L01AA02
ATC
chlorambucil; oral
+∞
13.9
13
*
A12AX
ATC
Calcium, combinations with vitamin D and/or other drugs
5.3
13.7
65
265
N19
ICD-10 Finland
Unspecified kidney failure
23.2
13.5
19
10
9314
FHL
Total knee endoprosthesis (also includes patellar part)
37.7
13.0
16
5
M08.3
ICD-10 Finland
Juvenile polyarthritis (seronegative)
+∞
12.8
12
*
H02AB04
ATC
methylprednisolone; systemic
6.7
12.8
36
78
N02AX02
ATC
tramadol; systemic, rectal
4.9
12.5
66
290
NA1AA
NOMESCO Finland
Cervical spine X-ray examination without contrast
15.4
12.3
20
16
A49.9
ICD-10 Finland
Bacterial infection, unspecified
7.7
12.2
30
53
9272
FHL
Metatarsophalangeal joint resection
+∞
11.7
11
*
9294
FHL
Endoprosthesis replacement of the entire hip joint (eg Charnley)
40.3
11.6
14
*
ZX120
NOMESCO Finland
Intravenous
8.5
11.5
26
40
V03AE01
ATC
polystyrene sulfonate; oral
49.4
11.2
13
*
NHG76
NOMESCO Finland
Arthroplasty of other tarsometatarsal joint
49.4
11.2
13
*
NA1EA
NOMESCO Finland
Cervical spine X-ray examination with flexion
49.4
11.2
13
*
H02AB06
ATC
prednisolone; systemic
4.5
11.0
69
337
ZXE20
NOMESCO Finland
More than three and less than five hours
6.4
11.0
31
66
P01BA02
ATC
hydroxychloroquine; oral
7.1
10.9
28
52
UJD10
NOMESCO Finland
Esophagoscopy, gastroscopy and duodenoscopy
4.6
10.9
48
169
9256
FHL
Synovectomy (knee 9311)
67.6
10.9
12
*
QDB10
NOMESCO Finland
Major dressing of wound of skin of lower limb
12.8
10.9
19
18
7140B
ICD-9 Finland
Rheumatoid arthritis and other inflammatory polyarthropathies, Rheumatoid arthritis[ARTHRITIS RHEUMATOIDES SERONEGATIVA]
122.6
10.6
11
*
J01MA12
ATC
levofloxacin; systemic
5.2
10.6
38
107
M04AA01
ATC
allopurinol; systemic
6.5
10.6
29
59
H02AB07
ATC
prednisone; oral
5.8
10.5
32
75
M05BA04
ATC
alendronic acid; oral
6.2
10.4
30
65
A46
ICD-10 Finland
Erysipelas
5.6
10.4
33
81
ZXD05
NOMESCO Finland
Urgent procedure
4.4
10.3
47
169
190
Kela drug reimbursment
Lanthanum carbonate, sevelamer and sucroferric oxyhydroxide
45.0
10.2
12
*
M06.0
ICD-10 Finland
Seronegative rheumatoid arthritis
23.0
10.1
14
7
PH1AE
NOMESCO Finland
Ultrasound examination of upper extremity veins
29.6
10.1
13
5
J01FF01
ATC
clindamycin; systemic
4.3
9.9
46
167
NF1AA
NOMESCO Finland
Hip X-ray examination
6.9
9.8
25
46
PG1DE
NOMESCO Finland
Other extensive ultrasound examination of artery
12.6
9.7
17
16
B03XA02
ATC
darbepoetin alfa; parenteral
20.1
9.7
14
8
C90.0
ICD-10 Finland
Multiple myeloma
33.8
9.6
12
*
9273
FHL
Other surgery for toe deformity or pain condition, e.g., Morton, hammer toe
33.8
9.6
12
*
TPX10
NOMESCO Finland
Implantation of vascular injection port
33.8
9.6
12
*
B01AB04
ATC
dalteparin; parenteral
10.0
9.6
19
23
9503
FHL
Tendon or tendon sheath cleansing surgery (tenosynovectomy)
110.2
9.6
10
*
Z94.0
ICD-10 Finland
Kidney transplant status
110.2
9.6
10
*
L04AA11
ATC
[U] etanercept; parenteral
110.2
9.6
10
*
L04AC07
ATC
tocilizumab; parenteral
110.2
9.6
10
*
TPH20
NOMESCO Finland
Insertion of central venous catheter through subclavian or brachiocephalic vein
15.8
9.5
15
11
KAS10
NOMESCO Finland
Allogenic transplantation of kidney from cadaver donor
+∞
9.5
9
*
7143C
ICD-9 Finland
Rheumatoid arthritis and other inflammatory polyarthropathies, Juvenile chronic polyarthritis[POLYARTHRITIS JUVENILIS SERONEGATIVA]
+∞
9.5
9
*
Z49.0
ICD-10 Finland
Preparatory care for dialysis
+∞
9.5
9
*
9732/3-C42.1
ICD-O-3
Plasma cell myeloma of bone marrow
+∞
9.5
9
*
GD1AA
NOMESCO Finland
Thorax X-ray examination
3.9
9.5
61
292
JN3AE
NOMESCO Finland
Abdominal ultrasound examination
6.0
9.4
27
58
L04AB04
ATC
adalimumab; parenteral
21.1
9.2
13
7
J01EE01
ATC
sulfamethoxazole and trimethoprim; systemic
9.1
9.2
19
25
GD1UA
NOMESCO Finland
Thorax X-ray examination outside radiological department
5.2
9.0
30
77
I50.9
ICD-10 Finland
Heart failure, unspecified
5.0
8.9
31
83
T84.0
ICD-10 Finland
Mechanical complication of internal joint prosthesis
10.0
8.7
17
20
H26.2
ICD-10 Finland
Complicated cataract
30.6
8.7
11
*
T84.5
ICD-10 Finland
Infection and inflammatory reaction due to internal joint prosthesis
30.6
8.7
11
*
N18.8
ICD-10 Finland
Other chronic renal failure
14.6
8.7
14
11
137
Kela drug reimbursment
Uraemia requiring dialysis
98.1
8.6
9
*
NCB20
NOMESCO Finland
Primary total prosthetic replacement of elbow joint
98.1
8.6
9
*
N17.9
ICD-10 Finland
Acute renal failure, unspecified
16.4
8.5
13
9
NC1AA
NOMESCO Finland
Elbow X-ray examination without contrast
16.4
8.5
13
9
L98.4
ICD-10 Finland
Chronic ulcer of skin, not elsewhere classified
16.4
8.5
13
9
PG5RT
NOMESCO Finland
PTA of arterio-venous shunt for dialysis
+∞
8.4
8
*
E85.0
ICD-10 Finland
Non-neuropathic heredofamilial amyloidosis
+∞
8.4
8
*
H02AB08
ATC
triamcinolone; systemic
+∞
8.4
8
*
NB1AA
NOMESCO Finland
Shoulder X-ray examination without contrast
6.9
8.4
21
37
NGB40
NOMESCO Finland
Primary total prosthetic replacement of knee joint with patellar part - sliding prosthesis
10.4
8.4
16
18
T81.4
ICD-10 Finland
Infection following a procedure, not elsewhere classified
6.4
8.3
22
42
NDG74
NOMESCO Finland
Fusion of first IP joint
36.7
8.2
10
*
127
Kela drug reimbursment
Transplant complication
36.7
8.2
10
*
ZZH31
NOMESCO Finland
Bone allograft, small pieces
36.7
8.2
10
*
NHG20
NOMESCO Finland
Fusion of talocrural joint
36.7
8.2
10
*
NH3AA
NOMESCO Finland
Foot and toes X-ray examination
7.1
8.2
20
34
L04AB01
ATC
etanercept; parenteral
24.5
8.2
11
5
ZXA05
NOMESCO Finland
Left side
3.8
8.2
44
173
NG1AA
NOMESCO Finland
Knee X-ray examination without contrast
5.1
7.9
26
65
JN3AA
NOMESCO Finland
Abdominal X-ray examination without contrast
10.2
7.9
15
17
WZA10
NOMESCO Finland
Long statement of health and health related matters
49.0
7.8
9
*
M01CB03
ATC
auranofin; oral
49.0
7.8
9
*
TPH90
NOMESCO Finland
Removal of fixed intravenous catheter
49.0
7.8
9
*
9284
FHL
Wrist ossification surgery
49.0
7.8
9
*
117
Kela drug reimbursment
Leukaemia and other malignant diseases of the blood and bone marrow, and malignant diseases of the lymphatic tissue
13.4
7.8
13
11
L04AA06
ATC
mycophenolic acid; systemic
27.5
7.7
10
*
L04AD01
ATC
ciclosporin; systemic
27.5
7.7
10
*
L01CB
ATC
Podophyllotoxin derivatives
86.3
7.5
8
*
320
Kela drug reimbursment
Lanthanum carbonate, sevelamer and sucroferric oxyhydroxide
86.3
7.5
8
*
V03AE02
ATC
sevelamer; oral
86.3
7.5
8
*
9289
FHL
Other measure in this category
86.3
7.5
8
*
PH2AE
NOMESCO Finland
Ultrasound examination of lower leg veins
5.4
7.5
23
53
GD1PA
NOMESCO Finland
X-ray examination of the lungs in one projection
6.5
7.4
19
35
9522
FHL
Synovectomy
+∞
7.4
7
*
B03XA03
ATC
methoxy polyethylene glycol-epoetin beta; parenteral
+∞
7.4
7
*
A12AA12
ATC
[U] calcium acetate anhydrous; systemic
+∞
7.4
7
*
E85.89
ICD-10 Finland
Other amyloidosis
+∞
7.4
7
*
M05BA07
ATC
risedronic acid; oral
8.1
7.4
16
23
Y83
ICD-10 Finland
Surgical operation and other surgical procedures as the cause of abnormal reaction of the patient, or of later complication, without mention of misadventure at the time of the procedure
6.0
7.3
20
40
M05
ICD-10 Finland
Seropositive rheumatoid arthritis
32.7
7.3
9
*
NHG24
NOMESCO Finland
Fusion of subtalar joint and between bones of ankle
32.7
7.3
9
*
R4180
NOMESCO Finland
Podiatric therapy
32.7
7.3
9
*
KH1AE
NOMESCO Finland
Urinary tract ultrasound examination
7.7
7.2
16
24
ND2AA
NOMESCO Finland
Hand and fingers X-ray examination
7.1
7.2
17
28
ZXE10
NOMESCO Finland
More than one and less than three hours
3.2
6.9
52
257
L01AA01
ATC
cyclophosphamide; systemic
43.2
6.9
8
*
NFC40
NOMESCO Finland
Repair of hip prosthesis by bone transplant to acetabulum
43.2
6.9
8
*
L04AA17
ATC
[U] adalimumab; parenteral
43.2
6.9
8
*
L04AX04
ATC
lenalidomide; oral
43.2
6.9
8
*
NHM20
NOMESCO Finland
Excision of synovial ganglion of ankle or foot
24.5
6.8
9
*
B01AB05
ATC
enoxaparin; parenteral
3.3
6.7
43
187
NHG80
NOMESCO Finland
Fusion of first tarsometatarsal joint
9.2
6.6
13
16
NA2AA
NOMESCO Finland
Thoracal spine X-ray examination without contrast
15.7
6.5
10
7
KA2AE
NOMESCO Finland
Ultrasound examination transplanted kidney
74.5
6.5
7
*
XX9XE
NOMESCO Finland
Other ultrasound examination
74.5
6.5
7
*
9531
FHL
Arthrodesis of the hand joint
74.5
6.5
7
*
L01BB01
ATC
NA
74.5
6.5
7
*
NBB10
NOMESCO Finland
Primary partial prosthetic replacement of humero-scapular joint not using cement, other or unspecified
74.5
6.5
7
*
L97
ICD-10 Finland
Ulcer of lower limb, not elsewhere classified
8.0
6.5
14
20
A07AA09
ATC
vancomycin; oral
19.6
6.4
9
5
NDG30
NOMESCO Finland
Fusion of wrist, total
28.7
6.3
8
*
27699
ICD-8 Finland
Amyloidosis
+∞
6.3
6
*
N084, E853
ICD-10 Finland
NA
+∞
6.3
6
*
M08.4
ICD-10 Finland
Pauciarticular juvenile arthritis
+∞
6.3
6
*
NH1AA
NOMESCO Finland
Ankle X-ray examination without contrast
6.9
6.3
15
25
NF1AE
NOMESCO Finland
Hip joint ultrasound examination
9.6
6.3
12
14
J01MA14
ATC
moxifloxacin; systemic
5.8
6.2
17
34
NDU20
NOMESCO Finland
Removal of internal fixation device from wrist or hand
13.7
6.2
10
8
A02BC03
ATC
lansoprazole; oral
3.1
6.2
41
183
R50.9
ICD-10 Finland
Fever, unspecified
4.3
6.2
23
65
J01DA01
ATC
[U] cefalexin; systemic
3.1
6.1
73
472
M01AH02
ATC
rofecoxib; oral
3.8
6.1
27
89
TNX50
NOMESCO Finland
Removal of cutom molded cast and internal stretch
8.9
6.0
12
15
TNX33
NOMESCO Finland
Custom moulded cast, unspecified region
8.9
6.0
12
15
QDB05
NOMESCO Finland
Revision of wound of skin of lower limb
16.3
6.0
9
6
WW200
NOMESCO Finland
Immunization
16.3
6.0
9
6

Mortality – FinRegistry

Association

Association between endpoint E4_AMYL_SCNDSYST and mortality.

Females

Parameter HR [95% CI] p-value
E4_AMYL_SCNDSYST 7.651 [6.33, 9.25] < 0.001
Birth year 1.0 [0.99, 1.01] 0.982

During the follow-up period (1.1.1998 — 31.12.2019), 408 out of 583 females with E4_AMYL_SCNDSYST died.

Males

Parameter HR [95% CI] p-value
E4_AMYL_SCNDSYST 6.313 [4.57, 8.71] < 0.001
Birth year 0.989 [0.98, 1.0] 0.024

During the follow-up period (1.1.1998 — 31.12.2019), 207 out of 270 males with E4_AMYL_SCNDSYST died.

Mortality risk

Mortality risk for people of age

years, who have E4_AMYL_SCNDSYST.

N-year risk Females Males
1 0.594% 1.358%
5 4.313% 7.536%
10 11.776% 18.552%
15 22.493% 31.996%
20 36.046% 49.217%

Relationships between endpoints

Index endpoint: E4_AMYL_SCNDSYST – Secondary systemic amyloidosis

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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