How do you manage pemphigus patients who have persistent disease and circulating autoantibodies despite two full courses of rituximab?

I currently manage two recalcitrant pemphigus patients, both of whom have undergone various courses of typical systemic immunosuppression (e.g., CellCept, MTX, steroids) but continued to flare. Combinations of these traditional therapies, along with topicals, were not particularly helpful.

While disease activity moderately improved with Ritux q6mo, eventually the addition of IVIG q4w (0.8 mg/kg/day) led to significant disease improvement, down-trending titers, and disease remission.

Patients with persistent pemphigus disease and circulating autoantibodies after two full rituximab courses should be re-treated with an additional cycle of rituximab 2000 mg, with consideration for alternative immunosuppressive therapies or intravenous immunoglobulin (IVIG) if rituximab remains ineffective. I recommend checking B cell counts (CD19, CD20)

Pemphigus is caused by autoantibodies against desmoglein 1 and 3, and persistence of autoreactive B-cell populations—particularly CD20-negative...