Description

The European Vasculitis Study Group (EVSG) classifies the disease severity of an ANCA-associated vasculitis based on clinical and laboratory findings. The apparent clinical severity of the vasculitis can help guide immunosuppressive therapy.


 

Patient selection: ANCA-associated vasculitis

 

Parameters:

(1) systemic, constitutional symptoms (fever, malaise, etc.)

(2) threatened or actual failure in one or more vital organs

(3) serum ANCA status

(4) serum creatinine

 

Vital organs that may be affected:

(1) renal

(2) pulmonary (diffuse alveolar hemorrhage, other)

(3) cardiac

(4) gastrointestinal

 

Systemic Symptoms

Organ Failure

Serum ANCA

Serum Creatinine

Category

absent

absent

negative (see note)

< 120 µmol/L

localized (or in remission)

present

absent

negative or positive

< 120 µmol/L

early systemic

present

present

positive

< 500 µmol/L

generalized

present

present

positive

>= 500 µmol/L

generalized with severe renal disease

present

present

negative or positive

NA

refractory (if being treated)

 

where:

• In theory a localized reaction could have a positive ANCA.

 


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