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Comparative Study
. 2013 Oct;69(4):583-8.
doi: 10.1016/j.jaad.2013.05.016. Epub 2013 Jul 12.

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

Naif Almugairen  1 , Valerie Hospital, Christophe Bedane, Sophie Duvert-Lehembre, Damien Picard, Anne-Fleur Tronquoy, Estelle Houivet, Michel D'incan, Pascal Joly
Affiliations
Comparative Study

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

Naif Almugairen et al. J Am Acad Dermatol. 2013 Oct.

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.

Keywords: CI; CR; CRoffT; CS; MMF; PF; PNP; PV; complete remission; complete remission off therapy; confidence interval; corticosteroids; follow-up; mycophenolate mofetil; paraneoplastic pemphigus; pemphigus; pemphigus foliaceus; pemphigus vulgaris; treatment.

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