Current medical treatment of ocular mucous membrane pemphigoid

Ocular mucous membrane pemphigoid (MMP), as a potentially blinding disease, is an indication for systemic immunosuppressive treatment. Immunosuppressive agents are chosen with a "stepladder" approach, beginning with drugs having the fewest side effects. Dapsone, sulfapyridine/sulfasalazine and azathioprine are less successful in controlling inflammation than mycophenolate mofetil (MMF) and methotrexate (MTX). Moreover, compared to other immunosuppressive agents, MMF, followed by MTX, has the lowest rate of discontinuation due to side effects. Cyclophosphamide is the most potent immunosuppressive agent used for ocular MMP, but it should be used with caution because of life-threatening adverse effects. Intravenous immunoglobulin therapy (IVIg) should be considered for patients who are resistant to conventional immunosuppressive therapy, have significant adverse effects or contraindications to conventional therapy, or have uncontrolled rapidly progressive disease. If IVIg monotherapy is not successful after a period of ≥ 1 year, therapy with biological agents, such as rituximab or anti-TNF-α drugs, is suggested.