Description

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)

 


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