Systemic sclerosis may affect internal organs in addition to the skin. Medsger et al developed severity scales to grade severity of end-organ damage in the heart, peripheral vasculature and other organs. The authors are from multiple universities in North America and Europe.
Parameters:
(1) ECG conduction and arrhythmias
(2) left ventricular ejection fraction in percent (LVEF)
(3) congestive heart failure (CHF)
(4) digital changes secondary to vascular disease
Cardiac Severity Scale (CSS)
ECG |
LVEF |
CHF |
CSS Grade |
normal |
>= 50% |
absent |
0 (normal) |
conduction defect |
45 to 49% |
NA |
1 (mild) |
arrhythmia |
40 to 44% |
NA |
2 (moderate) |
|
< 40% |
NA |
3 (severe |
arrhythmia requiring therapy |
NA |
requiring therapy |
4 (endstage) |
Peripheral Vascular Severity Scale (PVSS)
Digital Changes |
PVSS Grade |
absent |
0 (normal) |
Raynaud's requiring vasodilators |
1 (mild) |
pitting scars |
2 (moderate) |
ulceration at tip |
3 (severe |
gangrene |
4 (endstage) |
Purpose: To grade the degree of cardiac and peripheral vascular involvement in a patient with systemic sclerosis using the scheme of Medsger et al.
Specialty: Immunology/Rheumatology
Objective: imaging studies, severity, prognosis, stage, response to therapy, disease progression
ICD-10: M34,