Shahid Javaid Akhtar
Punjab Medical College
Pakistan
Title: Cyclophosphamide pulse therapy in pemphigus
Biography
Biography: Shahid Javaid Akhtar
Abstract
Introduction: Pemphigus vulgaris is a potentially fatal disease which has high death rate of up to 100% before the advent of systemic corticosteroids. But now the major cause of mortality is the side effects of these and steroid-sparing agents. Dr. J S Pasricha used Dexamethasone Cyclophosphamide Pulse therapy in 1989 for the first time to overcome this issue. But it has got its own technical flaws. We made some logical modifications in this regime to minimize the side effects and maximum the efficacy of pulse therapy.
Objective: To see the efficacy of cyclophosphamide pulse therapy in pemphigus vulgaris.
Patients & Methods: 20 new patients of pemphigus vulgaris attending the OPDs of hospitals (Allied and DHQ) attached with Punjab Medical College, Faisalabad were enrolled. After getting baseline investigations, IV cyclophosphamide (0.75-1 g/m2) was given after every 3-4 weeks. Oral Ondansetron hydrochloride 8 mg on day 1-5 and 8 mg of IV Dexamethasone on day 1-3 to prevent chemotherapy induced nausea and vomiting was also administered. Patients were advised to drink plenty of water and micturate frequently on day one to safeguard against potential cystitis. Oral prednisolone was also given in the standard dosage of 1 mg/kg, however, it was tapered earlier but gradually first to single morning dose and then to alternate day therapy once the clinical remission was achieved. These were ultimately completely withdrawn but cyclophosphamide pulses were continued 6-12 months after complete clearance of skin lesions.
Results: 15 patients completed the therapeutic trial according to the original protocol; 8 of these are now off all therapy and are in clinical remission for more than two years. Three patients are only receiving their monthly cyclophosphamide pulses and are also symptom free; rest of the enrolled (n=4) are taking 15 mg of prednisolone in addition of cyclophosphamide pulses but are free of skin lesions. Apart from transient nausea and body aches, none of the patient experienced any major side effect.
Conclusion: Cyclophosphamide pulse therapy for pemphigus vulgaris seems to be efficacious and may provide cure in this potentially fatal autoimmune disease though a large number of patients and longer follow up is required before making a final conclusion.