Use of intravenous immunoglobulin therapy in autoimmune blistering diseases.

Autoimmune mucocutaneous blistering diseases (AMBD) are an interesting group of rare diseases that affect the mucous membranes and the skin and are frequently or potentially fatal. The clinical presentation is significantly variable, as is the course and prognosis. The immunopathology is well characterized and the target antigens to which the autoantibodies are directed have been studied by various investigators. A significant majority of the patients respond to conventional therapy, which consists of high-dose long-term systemic corticosteroids and immunosuppressive agents. This treatment program has significantly improved the prognosis in many patients. In such patients, significant side effects of the drugs may appear and produce a very poor quality of life. In patients with progressive diseases, especially those with mucous membrane pemphigoid, the significant sequela; such as blindness, aphonia, and stenosis of the anal and vaginal canals can occur. In several patients treated with conventional immunosuppressive therapy, death occurs as a consequence of prolonged immune suppression leading to opportunistic infections. In this manuscript, the published data on the use of immunoglobulins intravenous (IGIV) in patients with AMBD is presented. The most important features of IGIV in patients with AMBD are: 1) the ability to clinically control the disease; 2) the ability to induce and maintain a long-term clinical remission; 3) a lower incidence of side effects; and 4) a higher quality of life. The important characteristic of the IGIV therapy in the AMBD is two-fold. First, the therapy, when given according to a published protocol, produces a lasting and long-term clinical remission, rather than a temporary arrest of the disease. Second, the therapy, as described in the protocol, has a very definitive endpoint. Consequently, once the patients are treated and go into long-term remission, the therapy is no longer required. The significant positive results obtained with IGIV are to a large extent also due to the associated aggressive topical therapy that was used and the frequent use of sublesional injections with triamcinolone. The rapid and early detection of cutaneous and mucosal infections and their treatment with systemic antibiotics is also a very important feature of IGIV therapy. When patients are under long-term conventional therapy, the infections are often not detected because they lack the ability to mount signs of inflammation. It is also becoming increasingly clear for patients to have a successful outcome, in treatment with IGIV therapy, it is critical that the physician spends a significant amount of time with each patient, monitor the therapy closely, and be familiar with the overall health of the patient. It is also best if the therapies are instituted by a physician who has significant interest and experience in blistering diseases and IGIV therapy.