Steroid‐pulse Therapy in Pemphigus Vulgaris Long Term Eollow‐up

Abstract

Pulse therapy is recommended as an adjunct to the total management plan of severely affected patients with pemphigus vulgaris. The addition of cyclophosphamide may prevent the disease from recurring. Alternate-day small-quantity bolus infusions over 16-20 days, may be equally effective as the administration of 15 mg/kg/daily for 3-5 days. The risk of cardiac arrest exists even with this, less aggressive form of PT therapy. A medical history of supraventricular arrhythmias may be considered a risk factor.

Pemphigus Vulgaris IgG and Methylprednisolone Exhibit Reciprocal Effects on Keratinocytes

“…The authors concluded that the major therapeutic effect of CH was due to reduction of antibody synthesis rather than modification of the events that occur within the epidermis after antibody binding. In turn, the adequacy of the conclusion drawn from the in vivo experiment has been challenged by the well established clinical fact that pulse therapy with a high dose of an intravenous administered glucocorticosteroid agent, such as MP, can stop acantholysis in PV patients within 24 -48 h, which is too early to induce changes in the serum titer of pemphigus autoantibodies (22,25,26). Therefore, we hypothesized that the discrepancy among the results of in vitro and in vivo experiments reported previously might be due to limitations of the mouse model used by Anhalt et al (3,84), and we sought to develop an adequate model for in vivo testing the anti-acantholytic efficacy of CH in pemphigus.…”

“…First, although the major decline in antibody titers occurs 3-4 weeks after glucocorticosteroid administration (19 -21), clinical lesions in PV patients usually improve much more rapidly, within 24 -48 h after initiation of a high dose, "pulse" therapy with methylprednisolone (MP) or dexamethasone, when the titer of pemphigus autoantibodies remains unchanged (22)(23)(24)(25)(26).…”

Pemphigus Vulgaris Acantholysis Ameliorated by Cholinergic Agonists

“…Although the antiacantholytic effect of glucocorticosteroids is attributed to immunosuppression, high doses of glucocorticosteroids can directly block PV IgG-induced acantholysis in vitro 1,2 and rapidly (within 48 hours) stop blistering in patients with pemphigus without altering the titer of autoantibodies or blocking antibody binding to keratinocytes ("pulse therapy"). [3][4][5] Patients develop autoantibodies to keratinocyte cholinergic receptors regulating cell adhesion. 6 Activation of these receptors mimics antiacantholytic effects of glucocorticosteroids in vitro.…”

Randomized Controlled Trial of Adjuvant Oral Dexamethasone Pulse Therapy in Pemphigus Vulgaris

“…Adjuvant pulse therapy may have a value for severe or refractory cases of pemphigus. 7,9,12 We saw a significantly larger weight gain in patients in the DP group. This weight gain was probably due to cushingoid obesity, which is a known to be an adverse effect of CS therapy.…”