Dexamethasone- cyclophosphamide pulse in collagen vascular diseases: An observation

Abstract

Background:Treatment of collagen vascular diseases like systemic sclerosis, dermatomyositis, systemic lupus erythematosus (SLE) and even overlap syndromes has been difficult since long. Monumental success of dexamethasone-cyclophosphamide pulse (DCP) in pemphigus has prompted many a dermatologist to try it in other autoimmune diseases.Materials and Methods:DCP was given as per standard regimen for six to nine pulses. Immunosuppressives were given for 12–18 months in dermatomyositis, SLE, and overlap syndrome, and for 12 months in systemic sclerosis. Daily dose of steroid was tapered off gradually.Results:The treatment resulted in 90% improvement in skin binding in systemic sclerosis, 80% in exertional dyspnea, 40% in dysphagia, but minimum improvement was seen in Raynauds and digital tip ulcerations. No improvement in pigmentation was noted. In SLE, malar rash cleared in 70%, joint pain in 80%, oral ulcerations reduced in 80%, fever in 98%, and photosensitivity improved in one-third of patients. In dermatomyositis, improvement in muscle tenderness was seen in 100%, improvement in proximal myopathy and heliotrope rash in 80%, and improvement of shawl sign was observed in 80% of the patients. Some flattening of Gottron papules and plaques was noted in some patients. Both overlap patients improved significantly. Out of 24 patients, three were lost to follow-up, one resorted to homeopathic medicine and two expired (one dermatomyositis, one SLE). Side effects like hypertension, hyperglycemia, pyoderma, fungal infections, obesity, psychosis, etc. were seen in 25–30% of patients.Conclusions:We conclude that DCP is relatively safe, effective as well as cheap compared to methylprednisolone pulse. Side effects are also less compared to daily regimen of steroids. We also observed that patients who reported early and put on pulse early responded better.