Effect of oral or pulse cyclosphosphamide in recalcitrant pemphigus: an audit of eighteen patients
- Author:
Tang MM
;
Priya G
;
Suganthi T
- Publication Type:Journal Article
- Keywords:
Pemphigus vulgaris, pemphigus foliaceous, cyclophosphamide
- From:Malaysian Journal of Dermatology
2012;28(-):34-40
- CountryMalaysia
- Language:English
-
Abstract:
Background Autoimmune pemphigus is a potentially life threatening bullous disease. The
cornerstone of treatment is systemic corticosteroids. However, adjuvant therapy with
immunosuppressant drugs is commonly used to improve disease control and alleviate the high
morbidity and mortality associated with the use of corticosteroids. Adjunctive treatment with pulse
intravenous cyclophosphamide may be more efficacious and less toxic than other
immunosuppressants.
Objective To retrospectively review the clinical outcome of 18 patients with recalcitrant pemphigus
who were treated with cyclophosphamide over the past 10 years.
Methodology A retrospective study was conducted between 1985 and 2009 in thirteen Malaysian
dermatology centres. Data collected were analysed for comparison of relapse rates, compliance rates
and adverse drug effects between the 2 regimes.
Results Eighteen patients were included in this audit of which 12 patients had pemphigus vulgaris
and 6 patients had pemphigus foliaceous. Prior to treatment with cyclophosphamide, fourteen
patients were on azathioprine, three were given intravenous immunoglobulin, and two were
prescribed dapsone; however all these patients were either unresponsive, intolerant or suffered
serious side-effects with these drugs. Subsequently, 7 patients (median age: 31 years) received a
combination of pulse intravenous cyclophosphamide and either intravenous dexamethasone or
methylprednisolone. These seven patients received between 2 to 21 pulses of intravenous
cyclophosphamide and steroids at monthly intervals with oral prednisolone and cyclophosphamide
(50-100mg) in between pulses. The remaining 11 patients (median age: 46 years) received oral
cyclophosphamide and corticosteroids. Of the 18 patients in our cohort, 15 achieved control and
consolidation of disease activity at an average of 4 weeks and 10 weeks respectively. The remaining
three patients are yet to achieve disease control. The total duration of treatment with
cyclophosphamide ranged from 2 to 62 months with a cumulative dose ranging from 2.95g to
93.55g. Four patients achieved partial remission on minimal therapy and 3 achieved complete
remission. None of patients experienced serious side effects.
Conclusion Cyclophosphamide may be an alternative treatment option in patients in patients with
pemphigus who fail to respond to standard therapy. Controlled trials are needed to further evaluate
the efficacy and safety of this therapy.
