Cyclophosphamide is another adjuvant drug often used in the treatment of pemphigus. A single small RCT and a few case series report on the use of cyclophosphamide in pemphigus vulgaris. Although there are a few reports of successful treatment with cyclophosphamide monotherapy, the most substantive evidence in support of its use comes from studies in which cyclophosphamide is used in combination with corticosteroids. Chrysomallis et al.14 studied
28 patients with newly diagnosed pemphigus vulgaris restricted to the oral cavity. Patients were randomly allocated to treatment with prednisone equivalent 40 mg daily (n = 10) alone or in combination with either cyclophosphamide 100 mg/day (n = 10), or ciclosporin 5 mg/day (n = 8). All patients were followed for 5 years. There was no significant difference in the duration of treatment required to achieve partial remission or in the relapse rate in the three groups. All patients achieved partial remission within 40 days, and no patient died in the study. The incidence of complete remission in each group was not reported. The incidence of complications was higher with combination treatments.
In one of the largest case series, Piamphongsant15 described 12 patients (six with pemphigus vulgaris and six with pemphigus foliaceus) treated with corticosteroid plus oral cyclophosphamide. Patients received an initial dose of prednisone of either 60 or 120 mg/day depending on disease severity. After 2 weeks, all patients were started on cyclophosphamide 100 mg daily. Each month both prednisone and cyclophosphamide were gradually tapered. Three patients (25%) achieved complete remission of at least a year after 2 years of therapy. All other patients achieved partial remission and at the time of publication were on a "maintenance dose" of prednisone <15 mg/ day plus cyclophosphamide 50 mg on alternate days or once a week. There were no serious complications from cyclophosphamide therapy.
Block et al.6 reported a series of 13 patients with "moderate-to-severe" pemphigus vulgaris treated with prednisone plus cyclophosphamide. Initially, patients were given prednisone alone until "control of acute generalised lesions was achieved". At that time, cyclophosphamide (dosage unspecified) was added. It is not clear how long patients were treated with prednisone before disease control was achieved, and rates of remission were not mentioned. There was one treatment-related death. Otherwise, side-effects were not addressed.
Pulse intravenous cyclophosphamide is a relatively recent use of cyclophosphamide in pemphigus. Because pulse cyclophosphamide has been shown in randomised trials to be more effective than oral cyclophosphamide in treating lupus nephritis16,17 and autoimmune thrombocytopenic purpura,18 it was anticipated that this mode of therapy might also be effective in treating pemphigus and may minimise the side-effects associated with prolonged daily oral therapy. However, to date no study has directly compared oral and intravenous cyclophosphamide. Three studies of five or more patients have investigated the use of pulse cyclophosphamide in pemphigus. Virtually all patients were simultaneously treated with either pulse or oral corticosteroids. Pasricha et al.19 have summarised their experience with 300 patients over a 12-year period20 using intravenous dexamethasone 100 mg on three consecutive days and intravenous cyclophosphamide 500 mg on one day, repeating these pulses every 2-4 weeks. Between pulses, patients received oral cyclophosphamide 50 mg every day and "generally no corticosteroids", although it is not known what percentage of patients received oral corticosteroids. They reported that 190 of the original 300 patients (63%) were in "remission", which was described as "disease-free" and on no medication. The remission lasted over 2 years in 123 patients. At the time of publication, 13 patients (4%) had continuing disease activity through the pulse treatments. It is not known how many pulses (i.e. months of treatment), on average, were required to achieve remission. Sixty-one patients (20%) were lost to follow up, and no other information about them was provided. Of the 12 patients who died during the study period, 11 (4%) died of possible treatment-related causes. Forty patients relapsed during the study period; no average length of remission before relapse was given. Patients who relapsed were treated again with the pulse regimen. Infections, amenorrhoea, weight gain or loss and alopecia were common side-effects. There was one report of haemorrhagic cystitis.
Kaur and Kanwar21 used an almost identical regimen of pulse dexamethasone plus cyclophosphamide to treat 50 patients with pemphigus over a course of 2 years. At the time of publication, almost half the patients had improved to some degree, but were still receiving monthly pulse treatments (range 2-15 months of treatment). Another five patients had responded well and were receiving only oral cyclophosphamide 50 mg/day. Six patients "did not respond" and 13 patients were lost to follow up, all of whom had reportedly improved on pulse treatment. Thus no patients achieved complete remission. Three patients (6%) died of septicaemia. Other side-effects reported included cardiac arrhythmias, "frequent" oral candidiasis and hair loss, and amenorrhoea in almost all women treated for at least 6 months. Haemorrhagic cystitis, sterility and avascular necrosis were not seen. It is difficult to know whether the beneficial results in this study and in that by Pasricha et al.20 were due to cyclophosphamide or pulse steroids.
Fleischli et al.22 studied the use of pulse cyclophosphamide without concomitant intravenous corticosteroids in nine patients. Monthly pulse treatments consisted of intravenous cyclophosphamide 0-5-1-0 g/m2. Between pulse treatments patients received oral cyclophosphamide 50 mg and all but one patient received prednisone (dose not specified). The number of pulses necessary to "control disease" ranged from three to 24. Six patients had an "excellent or good" response, but no objective criteria for this evaluation were described. One patient did not respond to therapy, even after
20 pulse treatments. One patient died of cardiac failure after three pulses, but her disease was reportedly under good control at the time of death.
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