Treatment of severe pemphigus with a combination of immunoadsorption, rituximab, pulsed dexamethasone and azathioprine/mycophenolate mofetil: A pilot study of 23 patients

BACKGROUND:   It has been previously shown in a relatively small group of patients that a combination of immunoadsorption (IA) and rituximab with daily use of high-dose oral corticosteroids and azathioprine/mycophenolate mofetil may induce a rapid and durable remission in severe, treatment-resistant pemphigus.\n\nOBJECTIVES: To achieve a more rapid reduction of serum autoantibody levels by a more frequent use of IA in the initial phase of treatment and to reduce the number of severe adverse events of continuous oral corticosteroid therapy by switching to pulsed intravenous applications.\n\nMETHODS: Twenty-three consecutive patients with severe pemphigus were included. IA was performed at initially 3- and later 4-week intervals until lesions healed by 90%; 1000mg rituximab was given at weeks 1 and 3, and intravenous dexamethasone pulses were administered at first every 3weeks and then at increasing intervals in addition to daily azathioprine/mycophenolate mofetil.\n\nRESULTS: Along with a fast and durable decline of circulating autoantibody levels, all patients showed improvement of pemphigus lesions within the first weeks of therapy and long-term complete remission was induced in 19 (83%) patients. In the remaining four patients, one (4%) minimal disease and three (13%) partial remissions were observed. Over the long-term follow-up of 11-43 (mean 29) months, six (26%) patients had a recurrence and in two (9%) patients, severe adverse events occurred.\n\nCONCLUSIONS: This novel protocol treatment induces a fast and long-term remission in severe pemphigus and seems to offer an improved side-effect profile compared with daily use of corticosteroids.