Assessment of the rate of long-term complete remission off therapy in patients with pemphigus treated with different regimens including medium- and high-dose corticosteroids.

Abstract

BACKGROUND:

Few studies have evaluated pemphigus treatments according to the definitions of the consensus statement. Prognostic factors for complete remission off therapy (CRoffT) remain unknown.

OBJECTIVE:

We sought to assess the rate of CRoffT in patients with pemphigus treated with different regimens.

METHODS:

In all, 134 patients with pemphigus were included in a retrospective, multicenter study. Primary end point was the rate of CRoffT. Prognostic factors for CRoffT were determined using univariate and multivariate analyses.

RESULTS:

Eighty patients with pemphigus vulgaris, 47 with pemphigus foliaceus, and 7 with paraneoplastic pemphigus were included. Mean age was 60 ± 18 years. Patients were treated either with medium (≤0.5 mg/kg/d) (n = 32) or high (≥1 mg/kg/d) (n = 59) doses of prednisone, or without systemic corticosteroids (n = 43). Mean follow-up was 77 ± 64 months. In all, 68 patients (50.7%) achieved CRoffT (95% confidence interval 42.3%-59.2%) after a mean treatment duration of 36 ± 39 months, including 47 of 80 patients with pemphigus vulgaris (58.7%) and 21 of 47 with pemphigus foliaceus (44.7%). Main prognostic factors for CRoffT were initial mucosal involvement (hazard ratio 2.2; 95% confidence interval 1.05-4.58; P = .036) and younger age (<61 years) (hazard ratio 2.5; 95% confidence interval 1.18-5.12; P = .0167). The rate of long-lasting CRoffT was 44%, with a mean follow-up after treatment withdrawal of 59 ± 50 months.

LIMITATIONS:

This was a retrospective study.

CONCLUSION:

The rate of CRoffT was 51%. Patients with pemphigus vulgaris were more likely to achieve CRoffT than those with pemphigus foliaceus.

• Authors: Naif Almugairen, Valerie Hospital, Christophe Bedane, Sophie Duvert-Lehembre, Damien Picard, Anne-Fleur Tronquoy, Estelle Houivet, Michel D'incan, Pascal Joly
• Journal: Journal of the American Academy of Dermatology (J Am Acad Dermatol) Vol. 69 Issue 4 Pg. 583-8 (Oct 2013) ISSN: 1097-6787 [Electronic] United States
• PMID: 23850258 (Publication Type: Comparative Study, Evaluation Study, Journal Article)
• Copyright: Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

Chemical References

• Adrenal Cortex Hormones
• Mycophenolic Acid
• Prednisone

Topics

• Adrenal Cortex Hormones (therapeutic use)
• Adult
• Aged
• Cohort Studies
• Disease Progression
• Dose-Response Relationship, Drug
• Drug Administration Schedule
• Drug Therapy, Combination
• Female
• Follow-Up Studies
• Humans
• Kaplan-Meier Estimate
• Logistic Models
• Long-Term Care
• Male
• Middle Aged
• Multivariate Analysis
• Mycophenolic Acid (analogs & derivatives, therapeutic use)
• Pemphigus (drug therapy, mortality, pathology)
• Predictive Value of Tests
• Prednisone (therapeutic use)
• Retrospective Studies
• Risk Assessment
• Severity of Illness Index
• Survival Rate
• Time Factors
• Treatment Outcome