Rheumatic fever

Metadata

Name	Rheumatic fever (rh_fever)
Category	Infections
Sex
Adult	Yes
UUID	b5a06ac0-19ac-49dd-a229-18c7b1fb095a
Definition	A febrile disease occurring as a delayed sequela of infections with streptococcus pyogenes. It is characterized by multiple focal inflammatory lesions of the connective tissue structures, such as the heart, blood vessels, joints, and brain, and by the presence of Aschoff bodies in the myocardium and skin.

Ontologies

Disease Ontology (DOID)	DOID_1586
GWAS Catalogue	EFO_1001160
MeSH	D012213
SNOMED-CT	58718002

Key Summary Statistics

Cases from any source

Total	4029
Female (%)	2230 (55%)
Male (%)	1799 (45%)

Year first recorded

Cases linked in all UK Biobank sources

Cross-source concordance

Vertical bars correspond to number of cases per source/intersection; horizontal bars to left correspond to total number of cases per source.

Incidence & prevalence

Baseline prevalence stratified by sex

Female	61.53 (56.27,66.79)
Male	44.24 (39.37,49.1)

Incidence stratified by sex

Female	1.45 (1.14,1.76)
Male	1.8 (1.41,2.18)

Incidence stratified by age

Ages 40-44	0.7 (0.18,1.23)
Ages 45-49	0.59 (0.18,1.01)
Ages 50-54	0.76 (0.33,1.19)
Ages 55-59	1.31 (0.79,1.82)
Ages 60-64	1.82 (1.3,2.33)
Ages 65-69	3.45 (2.63,4.27)

Period prevalence

Methods

Comparison with general population

Sex-standardised prevalence from CALIBER study (Kuan et al. 2019)

Population	Ages 40-49	Ages 50-59	Ages 60-69
UK Biobank	12 (9,16)	31 (27,35)	61 (56,67)
CALIBER	13 (12,14)	33 (31,34)	71 (69,74)

Stratified by age and Townsend deprivation status

Deprivation status	Ages 40-49	Ages 50-59	Ages 60-69
Least deprived	9(4,19)	22(14,31)	54(45,66)
Low	2(0,9)	25(17,36)	60(49,71)
Medium	12(6,24)	28(20,39)	57(47,69)
High	15(8,26)	32(24,43)	57(47,70)
Most deprived	17(10,27)	45(35,58)	83(69,98)

Stratified by age and country

Country	Ages 40-49	Ages 50-59	Ages 60-69
England	12(9,16)	30(26,36)	59(54,65)
Scotland	8(2,21)	41(29,57)	76(60,94)
Wales	16(5,37)	15(6,29)	62(45,83)

Risk factors analysis

‘Healthy’ BMI, smoking status ‘never’, or no hypertension (absence of hypertension records) were considered as reference values.

Hazard Ratio (HR) values with P < 0.0002 were considered to show significant association with disease onset after correction for multiple-testing using the Bonferroni method

N=153969, Events=129

Factor	Level	Hazard Ratio	P value
BMI	overweight	1.18 (0.74, 1.86)	0.489
BMI	obese	1.91 (1.2, 3.06)	0.00685
BMI	underweight	0 (0, Inf)	0.995
smoking	previous	1.29 (0.88, 1.87)	0.189
smoking	current	1.41 (0.81, 2.45)	0.23
hypertension	yes	1.93 (1.34, 2.77)	0.000403

Definition

UK Biobank field id	Description	Code	Value
20002	Non-cancer illness code, self-reported	1479	rheumatic fever
41202	Diagnoses - main ICD10	I00	Rheumatic fever without mention of heart involvement
41202	Diagnoses - main ICD10	I01	Rheumatic fever with heart involvement
41202	Diagnoses - main ICD10	I02	Rheumatic chorea
41202	Diagnoses - main ICD10	I05	Rheumatic mitral valve diseases
41202	Diagnoses - main ICD10	I06	Rheumatic aortic valve diseases
41202	Diagnoses - main ICD10	I07	Rheumatic tricuspid valve diseases
41202	Diagnoses - main ICD10	I09	Other rheumatic heart diseases
41204	Diagnoses - secondary ICD10	I00	Rheumatic fever without mention of heart involvement
41204	Diagnoses - secondary ICD10	I01	Rheumatic fever with heart involvement
41204	Diagnoses - secondary ICD10	I02	Rheumatic chorea
41204	Diagnoses - secondary ICD10	I05	Rheumatic mitral valve diseases
41204	Diagnoses - secondary ICD10	I06	Rheumatic aortic valve diseases
41204	Diagnoses - secondary ICD10	I07	Rheumatic tricuspid valve diseases
41204	Diagnoses - secondary ICD10	I09	Other rheumatic heart diseases
40001	Underlying (primary) cause of death: ICD10	I00	Rheumatic fever without mention of heart involvement
40001	Underlying (primary) cause of death: ICD10	I01	Rheumatic fever with heart involvement
40001	Underlying (primary) cause of death: ICD10	I02	Rheumatic chorea
40001	Underlying (primary) cause of death: ICD10	I05	Rheumatic mitral valve diseases
40001	Underlying (primary) cause of death: ICD10	I06	Rheumatic aortic valve diseases
40001	Underlying (primary) cause of death: ICD10	I07	Rheumatic tricuspid valve diseases
40001	Underlying (primary) cause of death: ICD10	I09	Other rheumatic heart diseases
40002	Contributory (secondary) causes of death: ICD10	I00	Rheumatic fever without mention of heart involvement
40002	Contributory (secondary) causes of death: ICD10	I01	Rheumatic fever with heart involvement
40002	Contributory (secondary) causes of death: ICD10	I02	Rheumatic chorea
40002	Contributory (secondary) causes of death: ICD10	I05	Rheumatic mitral valve diseases
40002	Contributory (secondary) causes of death: ICD10	I06	Rheumatic aortic valve diseases
40002	Contributory (secondary) causes of death: ICD10	I07	Rheumatic tricuspid valve diseases
40002	Contributory (secondary) causes of death: ICD10	I09	Other rheumatic heart diseases
42040	GP clinical event records	14A1.00	H/O: rheumatic fever
42040	GP clinical event records	G00..00	Rheumatic fever without heart involvement
42040	GP clinical event records	G010.00	Acute rheumatic pericarditis
42040	GP clinical event records	G011.00	Acute rheumatic endocarditis
42040	GP clinical event records	G012.00	Acute rheumatic myocarditis
42040	GP clinical event records	G01..00	Rheumatic fever with heart involvement
42040	GP clinical event records	G01y000	Acute rheumatic pancarditis
42040	GP clinical event records	G01y.00	Other acute rheumatic heart disease
42040	GP clinical event records	G01yz00	Other acute rheumatic heart disease NOS
42040	GP clinical event records	G01z.00	Acute rheumatic heart disease NOS
42040	GP clinical event records	G020.00	Rheumatic chorea with heart involvement
42040	GP clinical event records	G021.00	Rheumatic chorea without mention of heart involvement
42040	GP clinical event records	G02..00	Rheumatic chorea
42040	GP clinical event records	G02..11	Sydenham’s chorea
42040	GP clinical event records	G02z.00	Rheumatic chorea NOS
42040	GP clinical event records	G0…00	Acute rheumatic fever
42040	GP clinical event records	G0y..00	Other specified acute rheumatic fever
42040	GP clinical event records	G0z..00	Acute rheumatic fever NOS
42040	GP clinical event records	G100.00	Adherent rheumatic pericardium
42040	GP clinical event records	G102.00	Chronic rheumatic myopericarditis
42040	GP clinical event records	G10..00	Chronic rheumatic pericarditis
42040	GP clinical event records	G110.11	Rheumatic mitral stenosis
42040	GP clinical event records	G111.00	Rheumatic mitral insufficiency
42040	GP clinical event records	G111.11	Mitral incompetence - rheumatic
42040	GP clinical event records	G111.12	Mitral regurgitation - rheumatic
42040	GP clinical event records	G11..11	Rheumatic mitral valve disease
42040	GP clinical event records	G120.00	Rheumatic aortic stenosis
42040	GP clinical event records	G121.00	Rheumatic aortic insufficiency
42040	GP clinical event records	G121.11	Aortic incompetence - rheumatic
42040	GP clinical event records	G121.12	Aortic regurgitation - rheumatic
42040	GP clinical event records	G122.00	Rheumatic aortic stenosis with insufficiency
42040	GP clinical event records	G12..00	Rheumatic aortic valve disease
42040	GP clinical event records	G12z.00	Rheumatic aortic valve disease NOS
42040	GP clinical event records	G140000	Rheumatic tricuspid stenosis
42040	GP clinical event records	G140100	Rheumatic tricuspid insufficiency
42040	GP clinical event records	G140111	Tricuspid regurgitation - rheumatic
42040	GP clinical event records	G140112	Tricuspid incompetence - rheumatic
42040	GP clinical event records	G140200	Rheumatic tricuspid stenosis and insufficiency
42040	GP clinical event records	G14021X	Rheumatic tricuspid stenosis and regurgitation
42040	GP clinical event records	G14021Y	Rheumatic tricuspid stenosis and incompetence
42040	GP clinical event records	G140z00	Rheumatic tricuspid valve disease NOS
42040	GP clinical event records	G141000	Rheumatic pulmonary stenosis
42040	GP clinical event records	G141100	Rheumatic pulmonary insufficiency
42040	GP clinical event records	G141200	Rheumatic pulmonary stenosis and insufficiency
42040	GP clinical event records	G141.00	Rheumatic pulmonary valve disease
42040	GP clinical event records	G141z00	Rheumatic pulmonary valve disease NOS
42040	GP clinical event records	G14..00	Other chronic rheumatic endocardial disease
42040	GP clinical event records	G14z.00	Rheumatic endocarditis NOS
42040	GP clinical event records	G14z.11	Rheumatic valvulitis, chronic NOS
42040	GP clinical event records	G1…00	Chronic rheumatic heart disease
42040	GP clinical event records	G1y0.00	Rheumatic myocarditis
42040	GP clinical event records	G1y..00	Other specified chronic rheumatic heart disease
42040	GP clinical event records	G1yz000	Rheumatic heart disease unspecified
42040	GP clinical event records	G1yz100	Rheumatic left ventricular failure
42040	GP clinical event records	G1yz.00	Other and unspecified rheumatic heart disease
42040	GP clinical event records	G1yzz00	Other rheumatic heart disease NOS
42040	GP clinical event records	G1z..00	Chronic rheumatic heart disease NOS
42040	GP clinical event records	GA0..00	Carditis due to rheumatic fever
42040	GP clinical event records	GA…00	Rheumatic heart disease
42040	GP clinical event records	Gyu0000	[X]Other acute rheumatic heart disease
42040	GP clinical event records	Gyu0.00	[X]Acute rheumatic fever
42040	GP clinical event records	Gyu1100	[X]Other rheumatic aortic valve diseases
42040	GP clinical event records	H571.00	Rheumatic pneumonia
42040	GP clinical event records	M15y600	Erythema marginatum in acute rheumatic fever
42040	GP clinical event records	XaDyG	Rheumatic heart disease
42040	GP clinical event records	Xaagc	Carditis due to rheumatic fever
42040	GP clinical event records	XE0Ua	Rheumatic endocarditis NOS
42040	GP clinical event records	XE0UZ	Mitral stenosis