The occurrence of a major exacerbation of disease in a patient with Wegener's granulomatosis can be identified based on clinical criteria. The authors are from the Netherlands.
To be identified as having a major exacerbation of Wegener's granulomatosis,
(1) the patient should have one or more of the following criteria
(2) the finding(s) should be a new or recurrent finding
(3) the finding is not due to another cause such as infection, drug reaction, or atherosclerosis
Criteria for major exacerbation:
(1) decrease in creatinine clearance by > 30% within a period of 3 months or less in combination with erythrocyturia
(2) pulmonary infiltrates on chest X-ray in combination with dyspnea and rising C-reactive protein
(3) cerebral vasculitis
(4) motor and sensory nerve disturbances with rising C-reactive protein (CRP)
(5) cranial nerve palsy with rising C-reactive protein (CRP)
(6) orbital pseudotumor
(7) necrotizing scleritis
(8) tracheal stenosis with severe dyspnea
(9) myocardial infarction due to vasculitis
(10) acute abdomen (with intestinal perforation) or massive gastrointestinal hemorrhage due to vasculitis
(11) progressive disease despite treatment of an apparent minor relapse with prednisolone 30 mg and cyclophosphamide 75 mg for at least 2 weeks
where:
• Erythrocyturia: > 15 erythrocytes per high power field in the urine sediment
