Treatment of pemphigus vulgaris with brief, high-dose intravenous glucocorticoids.

OBJECTIVES

To determine if intravenous high-dose "pulse" glucocorticoid therapy induced remission or reduced subsequent requirements for orally administered glucocorticoids in pemphigus vulgaris.

DESIGN

Retrospective, case-controlled study.

SETTING

Academic referral center.

PATIENTS OR OTHER PARTICIPANTS

Fifteen patients, all of whom had pemphigus vulgaris, were included in the analysis. The group comprised all patients with pemphigus vulgaris seen during 11.5 years who did not initially respond to low doses (< 40 mg/d) of prednisone and who were followed up for at least 500 days after beginning treatment.

INTERVENTIONS

One group received very high-dose intravenous pulse(s) of methylprednisolone sodium succinate (Solu-Medrol) (n = 9). The other group did not receive intravenous pulse therapy (n = 6).

MAIN OUTCOME MEASURES

Glucocorticoid dose and status of disease.

RESULTS

Patients treated with pulse therapy and control patients treated with conventional oral prednisone had similar initial disease severity. Six of the 9 patients who received pulse therapy showed improvement of their pemphigus vulgaris, and 4 of the 9 had discontinued all glucocorticoids and were in remission at mean (+/- SEM) 269 +/- 27 days. They have continued in remission without receiving prednisone for a mean of 714 +/- 142 days. Therapeutic benefit was seen in patients who received pulse therapy early or late after beginning glucocorticoid therapy. In contrast, none of the 6 control patients has achieved long-term remissions without therapy. All of the controls have had long courses of glucocorticoid therapy, with a mean of 1467 +/- 112 days of prednisone treatment. Overall, the mean (+/- SEM) prednisone dose between days 350 and 500 from onset of glucocorticoid therapy was 9.2 +/- 4.2 mg/d for those who received pulse therapy vs 21.0 +/- 3.3 mg/d (P < .05) for those who did not (unpaired 2-tailed t test).

CONCLUSIONS

High-dose pulse administration of glucocorticoids is a potentially effective therapy to be considered in the treatment of patients with severe pemphigus vulgaris. Similar patients treated with conventional orally administered doses of prednisone had protracted courses requiring years of glucocorticoid therapy with no long-term remissions.