Patients With Pemphigus Vulgaris May Benefit From Early Rituximab

Pemphigus vulgaris (PV) is a rare immune-mediated skin disorder, typically occurring in middle-aged and older adults, that involves painful blistering of the skin and mucous membranes. While many patients are well controlled on systemic corticosteroid treatment, steroid-sparing therapy can be desirable for treating moderate to severe forms of the disease, and rituximab is one such agent to show promise in this indication.

Pemphigus vulgaris (PV) is a rare immune-mediated skin disorder, typically occurring in middle-aged and older adults, that involves painful blistering of the skin and mucous membranes. The disease has a relapsing and remitting course that can, in severe instances, be life-threatening. While many patients are well controlled on systemic corticosteroid treatment, steroid-sparing therapy can be desirable for treating moderate to severe forms of the disease, and rituximab is one such agent to show promise in this indication.

A recent, single-center, retrospective, case-control study reported on 40 patients with PV, treated at Duke University from 1999 to 2015, who required steroids plus conventional adjuvant therapy with agents such as mycophenolate mofetil, azathioprine, adalimumab, or infliximab (n = 27), or steroids and conventional therapy followed by rituximab (n = 13).

The group who received rituximab was able to reduce median monthly prednisone intake from pre-rituximab levels of 658.6 mg per month (interquartile range [IQR], 600.0-724.8) to 177.2 mg per month (IQR, 97.3-309.5) after starting rituximab.

There was no difference in monthly prednisone intake between the conventional therapy group and the rituximab group after treatment, but the patients who received conventional treatment only had significantly lower monthly prednisone intake (141 mg per month; IQR, 79.3-428.8) versus the pre-rituximab patients. There was no difference in the likelihood of being able to discontinue prednisone between the patient groups.

Additionally, patients who received rituximab had lower numbers of infections, hospital admissions, and emergency department visits per year after initiating rituximab.

Between the patients receiving conventional treatment alone and those receiving rituximab, there was no difference in the likelihood of complete remission, but those who received rituximab gained remission in a median time period of 6 months, while those who received conventional treatments gained remission after a median of 10.7 months.

“Rituximab may facilitate remission by a different, more efficient mechanism compared to [conventional therapy] as it decreased prednisone intake dramatically and significantly in the [rituximab] group despite their more severe disease,” write the authors, who add that even those who respond well to conventional drugs may benefit from earlier intervention with rituximab in terms of fewer infections, hospitalizations, laboratory draws, and clinic visits.

Reference

AgarwalA, Hall RP, Bañez LL, Cardones AR. Comparison of rituximab and conventional adjuvant therapy for pemphigus vulgaris: a retrospective analysis [published online September 25, 2018]. PLOS One. https://doi.org/10.1371/journal.pone.0198074.