| Name |
Systemic sclerosis (sys_sclerosis) |
| Category |
Musculoskeletal |
| Sex |
|
| Adult |
Yes |
| UUID |
25564610-ad77-440c-a8f1-f2d1e9b19d6a |
| Definition |
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. |
Ontologies
Key Summary Statistics
Cases from any source
| Total |
486 |
| Female (%) |
404 (83%) |
| Male (%) |
82 (17%) |
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 |
7.66 (5.8,9.52) |
| Male |
2.24 (1.14,3.34) |
Incidence stratified by sex
| Female |
0.86 (0.62,1.1) |
| Male |
0.21 (0.08,0.34) |
Incidence stratified by age
| Ages 40-44 |
0.3 (-0.04,0.64) |
| Ages 45-49 |
0.59 (0.18,1.0) |
| Ages 50-54 |
0.25 (0.01,0.5) |
| Ages 55-59 |
0.52 (0.2,0.84) |
| Ages 60-64 |
0.92 (0.55,1.29) |
| Ages 65-69 |
0.5 (0.19,0.81) |
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 |
5 (3,7) |
7 (5,9) |
9 (7,11) |
| CALIBER |
4 (3,4) |
7 (6,8) |
10 (9,11) |
Stratified by age and Townsend deprivation status
| Deprivation status |
Ages 40-49 |
Ages 50-59 |
Ages 60-69 |
| Least deprived |
3(0,10) |
4(1,8) |
11(7,17) |
| Low |
5(1,14) |
9(5,16) |
7(4,12) |
| Medium |
4(1,11) |
6(3,12) |
6(3,10) |
| High |
5(2,12) |
8(4,14) |
13(9,20) |
| Most deprived |
6(2,12) |
6(2,11) |
9(5,15) |
Stratified by age and country
| Country |
Ages 40-49 |
Ages 50-59 |
Ages 60-69 |
| England |
5(3,7) |
7(5,9) |
9(7,12) |
| Scotland |
3(0,12) |
5(2,12) |
10(5,18) |
| Wales |
6(1,22) |
9(3,22) |
10(4,20) |
Definition
| UK Biobank field id |
Description |
Code |
Value |
| 20002 |
Non-cancer illness code, self-reported |
1384 |
scleroderma/systemic sclerosis |
| 41202 |
Diagnoses - main ICD10 |
M34 |
Systemic sclerosis |
| 41204 |
Diagnoses - secondary ICD10 |
M34 |
Systemic sclerosis |
| 40001 |
Underlying (primary) cause of death: ICD10 |
M34 |
Systemic sclerosis |
| 40002 |
Contributory (secondary) causes of death: ICD10 |
M34 |
Systemic sclerosis |
| 42040 |
GP clinical event records |
F396600 |
Myopathy due to scleroderma |
| 42040 |
GP clinical event records |
H572.00 |
Lung disease with systemic sclerosis |
| 42040 |
GP clinical event records |
K0H..00 |
Acute scleroderma renal crisis |
| 42040 |
GP clinical event records |
K0J0.00 |
Renal involvement in scleroderma |
| 42040 |
GP clinical event records |
N001000 |
Progressive systemic sclerosis |
| 42040 |
GP clinical event records |
N001100 |
CREST syndrome |
| 42040 |
GP clinical event records |
N001200 |
Systemic sclerosis induced by drugs and chemicals |
| 42040 |
GP clinical event records |
N001.00 |
Scleroderma |
| 42040 |
GP clinical event records |
N001.11 |
Acrosclerosis |
| 42040 |
GP clinical event records |
N001.12 |
Systemic sclerosis |
| 42040 |
GP clinical event records |
Nyu4500 |
[X]Other forms of systemic sclerosis |
| 42040 |
GP clinical event records |
X30Ku |
Acute scleroderma renal crisis |
| 42040 |
GP clinical event records |
XE1DF |
Scleroderma |
| 42040 |
GP clinical event records |
X30Kt |
Renal involvement in scleroderma |
| 42040 |
GP clinical event records |
XaQbV |
Progressive systemic sclerosis |