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.

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

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

98

4. Check minimum number of events

None

98

5. Include endpoints

None

98

6. Filter based on genotype QC (FinnGen only)

96

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 96 60 36
Unadjusted period prevalence (%) 0.02 0.02 0.02
Median age at first event (years) 61.68 59.52 65.30

-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 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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