diffuse cutaneous systemic sclerosis
GPTKB entity
Statements (49)
| Predicate | Object |
|---|---|
| gptkbp:instanceOf |
autoimmune disease
subtype of systemic sclerosis |
| gptkbp:affects |
gptkb:skin
internal organs |
| gptkbp:associatedWith |
pulmonary fibrosis
cardiac involvement gastrointestinal involvement renal crisis Raynaud phenomenon |
| gptkbp:characterizedBy |
early visceral involvement
rapid progression widespread skin thickening |
| gptkbp:commonIn |
gptkb:women
|
| gptkbp:complication |
heart failure
interstitial lung disease pulmonary arterial hypertension malabsorption scleroderma renal crisis |
| gptkbp:diagnosedBy |
clinical examination
skin biopsy organ function tests autoantibody testing |
| gptkbp:differentialDiagnosis |
gptkb:limited_cutaneous_systemic_sclerosis
eosinophilic fasciitis morphea |
| gptkbp:firstDescribed |
1940s
|
| gptkbp:hasAutoantibody |
anti-RNA polymerase III
anti-Scl-70 (anti-topoisomerase I) |
| https://www.w3.org/2000/01/rdf-schema#label |
diffuse cutaneous systemic sclerosis
|
| gptkbp:onset |
30-50 years
|
| gptkbp:prevalence |
rare
|
| gptkbp:prognosis |
variable
worse than limited cutaneous systemic sclerosis |
| gptkbp:symptom |
hypertension
joint pain difficulty swallowing muscle weakness shortness of breath digital ulcers skin tightening edema of hands |
| gptkbp:treatment |
gptkb:proton_pump_inhibitors
physical therapy immunosuppressive therapy corticosteroids ACE inhibitors calcium channel blockers |
| gptkbp:bfsParent |
gptkb:systemic_sclerosis
|
| gptkbp:bfsLayer |
7
|