Mycophenolate Mofetil to Treat Wegener's Granulomatosis and Related Vascular Inflammatory Conditions

INCLUSION CRITERIA: Documentation of Wegener's Granulomatosis (WG) based on clinical characteristics and histopathological evidence of vasculitis. Patient with a positive C- or P-ANCA and glomerulonephritis as evidenced by the presence of red blood cell casts and proteinuria or renal biopsy showing necrotizing glomerulonephritis in the absence of positive immunofluorescence for immunoglobulin and complement will also be eligible. Patients must be of the ages of 18-80 years. Patients on the CYC to MTX protocol (#95-I-0091) who experience a relapse of disease while on MTX maintenance therapy. Relapse is defined by a Vasculitis Disease Activity Index of greater than or equal to 3. Patients from outside the NIH will also be eligible if they have been treated with a CYC to MTX regimen identical to that used in #95-I-0091 and experience a relapse of disease while on MTX maintenance therapy. If treatment for this relapse has already been commenced at the outside institution with daily CYC and glucocorticoid, patients will still be eligible if there is a history of a Vasculitis Disease Activity Index greater than or equal to 3 at the time of CYC and glucocorticoid initiation. Patients who experience a relapse of disease after MTX has been stopped or while tapering the MTX dose (following 2 years of maintenance therapy) will not be eligible. Patients with active disease who have a contraindication to MTX therapy will be eligible. Evidence of active disease as defined by a Vasculitis Disease Activity Index of greater than or equal to 3. Patients with inactive disease who have a contraindication to CYC and. Evidence of active disease as defined by a Vasculitis Disease Activity Index of greater than or equal to 3. Patients with inactive disease on MTX while on the CYC to MTX protocol (95-I-0091) or the MTX protocol (90-I-0086) who develop an contraindication necessitating discontinuation of MTX. Patients from outside the NIH will also be eligible if they similarly develop a contraindication to MTX while on treatment. Patients with inactive disease on the CYC protocol (#76-I-0041 or 76-I-0042) who develop a contraindication necessitating CYC discontinuation and also have a contraindication to receiving MTX. Patients from outside the NIH will also be eligible if they similarly develop a contraindication to CYC while on treatment and cannot receive MTX. Patients with inactive disease who are receiving treatment with CYC and prednisone in a manner similar to #76-I-0042 will be eligible if they have a contraindication to receiving MTX and have been in remission for less 3 months. EXCLUSION CRITERIA: Evidence of active infection which, in the judgment of the investigator, is of greater danger to the patient than the underlying vasculitis. In those instances in which infection cannot be ruled out by gram stain and culture of secretions or collections of fluid in involved organs, it may be necessary to obtain a biopsy of the affected tissue for microbiological and histopathological studies. Patients who are pregnant or who are nursing infants will not be eligible. Fertile women must have a negative pregnancy test within one week prior to study entry and must be using an effective means of birth control. Patients with active disease who are eligible for the CYC to MTX protocol (#95-I-0091) or the MTX protocol (#90-I-0086). Active peptic ulcer disease. Serological evidence of infection with human immunodeficiency virus. A serological determination will be performed within two weeks of beginning study participation. Inability to comply with study guidelines. Creatinine clearance less than 25ml/min.