Adjuvant high-dose intravenous immunoglobulin therapy can be easily and safely introduced as an alternative treatment in patients with severe pemphigus vulgaris: a retrospective preliminary study

Long-term corticosteroid therapy, often in association with other immunosuppressive agents, is considered the mainstay of pemphigus vulgaris (PV) therapy. Recent evidence has been changing this paradigm as patients who are non-responsive to conventional therapies or who experience severe adverse effects have been successfully treated with high-dose intravenous immunoglobulin (IVIg). However, the shift from conventional therapies to IVIg represents a major challenge in the daily practice of non-experienced clinicians because of potential adverse effects and other issues relevant to IVIg therapy such as the necessity for premedication, selection of cases, modality of infusion, patient monitoring, and the cost and length of hospital stay. The purpose of this preliminary study was to evaluate and report outcomes of treatment with IVIg in eight selected PV patients meeting clearly defined criteria for initiation of this therapy. Available guidelines for IVIg therapy in patients with autoimmune mucocutaneous blistering diseases were followed. Clinical response, induction and duration of remission, strategies for prevention of adverse effects, and total days of hospital stay in eight patients with severe PV treated with IVIg were retrospectively evaluated. All patients had an effective clinical response without adverse reactions, leading to a significant corticosteroid-sparing effect. Our results indicate that, when current guidelines are followed, IVIg therapy can be easily and safely introduced as a treatment alternative in patients with severe PV