The impact of stratifying initial dose of corticosteroids by severity of pemphigus vulgaris on long-term disease severity.

BACKGROUND

Pemphigus vulgaris (PV) is a life-threatening disease affecting skin and mucous membranes. The "epitope spreading" theory posits that uncontrolled PV can gradually worsen because of exposure of cellular antigens to the immune system. To this end, high-dose systemic corticosteroids have been advocated as first-line treatment for patients with PV to achieve disease control.

OBJECTIVE

To determine whether the initial dose of prednisone stratified by disease severity affects long-term disease severity.

METHODS

A retrospective study was conducted on 58 patients with PV with at least five years of follow-up from diagnosis. Patients were categorized into three groups according to the initial dose of prednisone treatment. Parameters analyzed included age, gender, disease severity at baseline and follow-up, hospitalizations, prednisone doses and adjuvant therapy at follow-up, and remission rate.

RESULTS

Ten patients received initial low-dose prednisone or were treated initially without systemic CS, 19 patients received intermediate-dose prednisone, and 29 received high-dose prednisone. Disease severity at presentation correlated directly with initial prednisone doses. The duration of the first hospitalization and number of hospitalization days during the five-year follow-up period were significantly lower in the group treated with initial low-dose prednisone and similar for the groups treated with intermediate and high doses.

CONCLUSIONS

Disease severity of PV at presentation is a good predictor of the clinical course. Stratifying initial prednisone dose according to PV disease severity at presentation is appropriate.