Pemphigus vulgaris (PV) is a rare, potentially fatal autoimmune mucocutaneous blistering disease. The prolonged use of systemic corticosteroids, though clinically effective in high doses, can result in multiple debilitating adverse effects. Immunosuppressive agents, used as adjuvants and as corticosteroid-sparing agents, are not effective in all patients and are contraindicated in some. Therefore, alternative treatment modalities are needed to provide effective control of PV in such patients.
To demonstrate the corticosteroid-sparing effect of intravenous immunoglobulin (IVIg) therapy in patients with moderate to severe PV.
A retrospective analysis in a cohort of 15 patients with moderate to severe PV who were treated with IVIg therapy. All 15 patients were corticosteroid dependent, and the use of other systemic conventional immunosuppressive agents was contraindicated. The patients were followed up over a mean period of 6.2 years.
Ambulatory tertiary medical care facility of a university-affiliated hospital.
All 15 patients received an IVIg dose of 1 to 2 mg/kg per cycle.
Main Outcome Measures
The following information was documented in each of the 15 patients before and after the initiation of IVIg therapy: total dosage and total duration of prednisone therapy and number of relapses. Also, the highest dosage and adverse effects of prednisone therapy, as well as the total duration of observation, were recorded.
All 15 patients had a satisfactory clinical response to IVIg therapy. The use of systemic prednisone was gradually discontinued over a mean period of 4.3 months. A statistically significant difference was noted in the total dose of prednisone (P = .004), total duration of prednisone therapy (P = .003), and number of relapses (P<.001) before and after the initiation of IVIg therapy.
Intravenous immunoglobulin therapy has a demonstrable corticosteroid-sparing effect. It is a safe and effective alternative treatment modality in patients with PV who are dependent on systemic corticosteroids or who develop significant adverse effects as a result of their use.