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Systemic sclerosis

DOID EFO MESH SNOMED PheWeb

M13_SYSTSLCE

systemic scleroderma: A chronic disorder, possibly autoimmune, marked by excessive production of collagen which results in hardening and thickening of body tissues. The two types of systemic scleroderma, limited cutaneous and diffuse cutaneous are classified with focus on the extent of affected skin. A relationship exists between the extent of skin area affected and degree of internal organ/system involvement. Systemic scleroderma can manifest itself in pulmonary fibrosis, Raynaud's syndrome, digestive system telangiectasias, renal hypertension and/or pulmonary hypertension.

Endpoint definition

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FinnGen phenotype data

356077 individuals

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Apply sex-specific rule None

356077

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Check conditions None

356077

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Filter registries

Hospital Discharge: ICD-10 M34
Hospital discharge: ICD-9 7101
Hospital discharge: ICD-8 7340
Cause of death: ICD-10 M34
Cause of death: ICD-9 7101
Cause of death: ICD-8 7340

633

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Check pre-conditions, main-only, mode, ICD version

Look only at ICD versions H.D: 10, 8, 9 ; C.O.D: 10, 8, 9

560

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Check minimum number of events None

560

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Include endpoints None

560

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M13_SYSTSLCE

Extra metadata

Level in the ICD hierarchy 4
First used in FinnGen datafreeze DF2
Parent code in ICD-10 M30-M36
Name in latin Sclerosis systemica

Summary Statistics

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Key figures

All Female Male
Number of individuals 560 459 101
Unadjusted prevalence (%) 0.16 0.23 0.06
Mean age at first event (years) 54.45 54.37 54.81

Mortality

Follow-up Absolute risk HR [95% CI] p N
1998–2019 0.06 3.52 [1.37, 9.09] 9.2e-3 54
15 years 0.01 1.97 [0.86, 4.54] 1.1e-1 17
5 years 0.01 8.12 [4.21, 15.66] 4.0e-10 19
1 year 0.01 22.65 [10.77, 47.60] 1.8e-16 11

Age distribution of first events

Year distribution of first events

Cumulative Incidence

Correlations

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Index endpoint: M13_SYSTSLCE – Systemic sclerosis
GWS hits: 1

Survival analyses between endpoints

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Plot

before Systemic sclerosis
after Systemic sclerosis

loading spinner Loading survival analyses plot

Drugs most likely to be purchased after Systemic sclerosis

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