Use of intravenous immunoglobulin therapy during pregnancy in patients with pemphigus vulgaris

Background Pemphigus vulgaris (PV) is a potentially fatal autoimmune disease characterized by the presence of in vivo deposition of antibodies against cell surface antigens desmoglein 1 and desmoglein 2 in the epidermis. Objectives To report the treatment outcomes in pregnant PV patients treated with intravenous immunoglobulin (IVIg) therapy. Methods Eight patients with active disease during pregnancy were treated. Patients were treated with a dose of 2 g/kg/cycle. Seven patients were treated for 2 months on post-partum basis. Main Outcome Measures were as follows: (i) pregnancy outcome; (ii) presence of neonatal pemphigus; (iii) post-partum flare; (iv) effect of IVIg on present and future pregnancies; (v) immediate and long-term side-effects in the mother and child. Results Patients ages ranged from 20 to 43 years (mean 29.6). All patients had severe and widespread disease involving the skin and multiple mucous membranes. Patients one to seven responded to IVIg therapy and did not have a post-partum flare. Patient eight could not tolerate IVIg because of intense headaches and significant postpartum flare. None of the neonates had pemphigus. Three patients who completed the IVIg protocol had normal second pregnancies. One patient who did not complete the protocol had a miscarriage during the second pregnancy. Since last observation, none of the patients have had a recurrence of the disease or another pregnancy. Conclusions The data suggests that IVIg can be useful and safe in treating pregnant patients with PV. No long-term adverse effects of IVIg in the mother or in the child were observed based on a long-term follow-up.