Description

The Wegener's Granulomatosis Etanercept Trial Research Group listed criteria for limited Wegener's disease. This term "limited" refers to a patient who meets the criteria for Wegener's granulomatosis but without renal involvement.


 

Criteria - all of the following:

(1) absence of red blood cell casts in the urine

(2) one of the following:

(2a) no hematuria

(2b) hematuria AND serum creatinine <= 1.4 mg/dL AND any increase in serum creatinine from baseline is <= 25%

(3) one of the following:

(3a) no pulmonary involvement

(3b) circumscribed pulmonary involvement with PaO2 > 70 mm Hg on room air and oxygen saturation on pulse oximetry > 92% AND without pulmonary hemorrhage

(3c) circumscribed pulmonary involvement with PaO2 > 70 mm Hg on room air and oxygen saturation on pulse oximetry > 92% AND pulmonary hemorrhage which shows no evidence of progression

(4) absence of severe disease in other critical organs (gastrointestinal tract, eyes, central nervous system)

 

where:

• By definition any disease that was not limited was termed severe.

• Pulmonary hemorrhage that is progressive indicates severe disease.

• The wording for item 4 was "no disease may exist within any other critical organ....that, without the immediate institution of maximal therapy....threatens the function of that organ and/or the patient's life." I am not sure what this means. It may allow for serious disease but it reverses on maximal therapy (pulse methylprednisolone and dialy cyclophosphamide).

 


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