Long-Term Efficacy of Dexamethasone-Cyclophosphamide Pulse Therapy in Pemphigus

Abstract

Background: Corticosteroids are used as a daily oral therapy or in a pulse form with or without various adjuvant drugs for the treatment of pemphigus. Our long-term experience with the use of dexamethasone-cyclophosphamide pulse therapy as a first-line treatment modality for pemphigus is presented. Methods: A retrospective analysis of records of pemphigus patients treated by dexamethasone-cyclophosphamide therapy was carried out. The treatment regimen consisted of the monthly administration of intravenous dexamethasone (136 mg) for 3 consecutive days with addition of intravenous cyclophosphamide (500 mg) on the second day. Oral cyclophosphamide (50 mg) daily and oral corticosteroids (low tapering doses) were given in the intervals between the pulses, till partial remission was achieved. Pulse therapy was then continued for another 6 months followed by daily oral cyclophosphamide (50 mg) for 1 year, which produced a complete remission. Results: A total of 36 patients, 32 with pemphigus vulgaris and 4 with pemphigus foliaceus, were treated with this regimen. Two to 8 pulses were required to achieve a partial remission, while the total number of pulses given for complete remission ranged from 8 to 32. The duration of pulse therapy correlated with both the disease severity and the time to achieve remission. All patients are now in complete remission with a follow-up of 0.5–12 years. Four patterns of remission were observed, related to the severity of disease. The response to pulse therapy was faster in the milder form of disease. The severe form of disease required more pulses as well as higher doses of intervening oral corticosteroids. Conclusions: Dexamethasone-cyclophosphamide pulse therapy is an effective form of treatment in pemphigus and results in long-lasting remissions.

Randomized controlled open-label trial of four treatment regimens for pemphigus vulgaris

“…18,25 The use of pulsed intravenous cyclophosphamide decreases the adverse effects associated with oral administration, especially with respect to malignancies such as bladder cancer. [26][27][28] In our study, P/PC induced remission in 73.3% of patients after 1 year of treatment. The mean total dose of steroid was lower than in groups P and P/MM, but there were no statistically significant differences between the P/A and P/PC or between the P/MM and P/PC groups.…”

Pemphigus in North India

“…The details of DCP/DP therapy have been discussed elsewhere. 4,6 Essentially, DCP/DP therapy is divided into three phases: phase I, DCP therapy continued until a lesion-free period of 6 months; phase II, DCP is withdrawn, but daily oral cyclophosphamide 50 mg is continued for a minimum period of 12 months; and phase III, the patient is released from treatment but kept under observation for a minimum period of 2 years, with a regular 3-month follow-up. Prednisolone was used during the intervening period only for patients whose disease activity was not controlled with DCP.…”

“…Its long-term and curative efficacy has been established. 4,26 The course in response to DCP therapy followed different patterns and correlated roughly to the severity of the disease. Patients with severe disease required fewer DCPs, and patients with more severe disease required not only more DCPs but also intervening steroids to control the disease activity.…”

Prolonged Clinical Remission of Patients with Severe Pemphigus upon Rapid Removal of Desmoglein-Reactive Autoantibodies by Immunoadsorption

“…Among several reports, Kanwar et al[ 22] demonstrated that CY in a combined dexamethasone pulse regimen is an effective treatment in pemphigus. Studies in patients with systemic lupus erythematosus showed an additional immunomodulatory effect of CY treatment, referring to quantitative changes in both the T and B cell compartment [23, 24].…”