Pemphigus vulgaris is a potentially fatal autoimmune mucocutaneous blistering disease.
Conventional therapy consists of high-dose corticosteroids, immunosuppressive agents, and intravenous immune globulin.
Dr Ahmed and colleagues from Massachusetts studied patients with refractory pemphigus vulgaris.
The patients had pemphigus vulgaris involving 30% or more of their body-surface area, 3 or more mucosal sites, or both.
The patients also had inadequate responses to conventional therapy and intravenous immune globulin.
|Immunoglobin G4 antikeratinocyte antibody titers correlated with disease activity
|New England Journal of Medicine|
The researchers treated the patients with 2 cycles of rituximab at 375 mg/m2 of body-surface area once weekly for 3 weeks.
The team also treated the patients with intravenous immune globulin 2 g/kg of body weight in the fourth week.
This induction therapy was followed by a monthly infusion of rituximab and intravenous immune globulin for 4 consecutive months.
The researchers monitored titers of serum antibodies against keratinocytes and numbers of peripheral-blood B cells.
Of 11 patients, 9 had rapid resolution of lesions and a clinical remission lasting 22 to 37 months.
The team observed that all immunosuppressive therapy, including prednisone, could be discontinued before ending rituximab treatment in all patients.
The researchers treated 2 patients with rituximab only during recurrences, and noted that these had sustained remissions.
Titers of immunoglobin G4 antikeratinocyte antibodies correlated with disease activity.
Peripheral-blood B cells became undetectable shortly after initiating rituximab therapy but subsequently returned to normal values.
The team did not observe side effects that have been associated with rituximab.
In addition, the researchers noted no infections with rituximab.
Dr Ahmed's team comments, “The combination of rituximab and intravenous immune globulin is effective in patients with refractory pemphigus vulgaris.”