Description

Reinhold-Keller et al developed a score to describe the extent of Wegener's granulomatosis. It is based on an expansion of the ELK (ENT, lung, kidney) classification. The authors are from the Medical University of Lubeck, University of Kiel and Rheumaklinik Bad Bramstedt in Germany.


Parameters:

(1) expanded ELK classification of organ involvement (1 to 8)

(2) constitutional, "B" symptoms (fever, weight loss, fatigue)

 

Organ Involvement

Code

upper respiratory tract (ENT)

E

lung

L

kidney

K

rheumatic symptoms

A

inflammatory eye involvement

EY

nervous system (polyneuropathy, etc.)

N

heart (pericarditis, myocarditis, etc.)

H

skin

S

 

where:

• In the ELK classification a single organ may be involved except for the kidney alone.

 

Constitutional ("B") Symptoms

Finding

Points

fever, weight loss, fatigue

absent

0

 

present

1

 

modified ELK score =

= (2 * (number of organs involved)) + (points for B symptoms)

 

Interpretation:

• minimum score: 2

• maximum score: 17

• The higher the score the more extensive the disease.

• A score <= 9 indicates less extensive disease.

 

Potential modification:

• Assign 1 point for mild organ involvement and 2 points for moderate to severe.


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