Treatment of non-life threatening Wegener's granulomatosis with methotrexate and daily prednisone as the initial therapy of choice.

OBJECTIVE

To examine our experience with methotrexate (MTX) and daily prednisone (PRED) as the initial treatment of Wegener's granulomatosis (WG).

METHODS

Between November 1992 and November 1997, we treated 19 patients with non-life threatening WG with the combination of oral weekly MTX (starting at 7.5-10.0 mg/week) and daily PRED (median starting dose 40 mg/day, range 20-60). The MTX dose was increased to 15 mg/week by the end of the first month, and then by 2.5 mg/week until the disease was controlled. We attempted to taper PRED to 20 mg/day by the end of the second month, but did not use alternate day corticosteroids. Before treatment with this regimen, no patient had received previous treatment for WG.

RESULTS

At presentation, the average number of organ systems involved was 3.6. Nine of the 19 patients (47%) had glomerulonephritis, but none had a serum creatinine > 1.2 mg/dl at presentation. Only 37% of the patients were hospitalized at presentation. Seventeen of 19 patients (89%) improved with treatment, and 14 (74%) achieved remission. However, half those who achieved remission suffered relapses, and no patient achieved a durable, complete remission (disease-free status free of all medications). Fifteen patients (79%) were able to taper PRED to < 10 mg/day. Seven of 8 disease relapses responded to increases of MTX and/or PRED. Only one patient developed glomerulonephritis while receiving treatment and required a change of therapy to cyclophosphamide. There were no deaths among patients in this series. Treatment with MTX and PRED was well tolerated: only 2 (11%) of the patients stopped treatment because of side effects (major liver function test abnormalities in both cases). No patient suffered permanent morbidity from MTX treatment.

CONCLUSION

In selected patients with WG, the combination of MTX and daily PRED effectively controls the disease. However, chronic disease courses are the rule with this treatment regimen, and the likelihood of disease relapse is high. In our experience, the use of MTX and PRED in WG was safer than previously described, despite the use of daily corticosteroids.