Results of the guideline-based treatment for pemphigus: a single-centre experience with 84 cases

R. Kakuta, Yuichi Kurihara, J. Yamagami, J. Miyamoto, T. Funakoshi, A. Tanikawa, M. Amagai

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Background: The Japanese guidelines for the management of pemphigus (JG) were published in 2010. However, further progress in the treatment of pemphigus requires their validation. Objectives: To examine the efficacy and safety of treatments based on the JG. Methods: A retrospective study of 84 Japanese patients with moderate to severe pemphigus, who were initially treated in accordance with the JG and then followed up for >2 years, was performed in a single centre. Treatment typically consisted of 0.5–1 mg prednisone (PSL)/kg/day accompanied by 100 mg azathioprine/day as a steroid-sparing agent. Results: In 83 of the 84 patients (98.8%), complete remission on minimal therapy (≤10 mg PSL/day and concomitant immunosuppressive agent) was achieved. The time between initiation of therapy and remission was 13.9 ± 9.4 months. In 78 patients (92.9%), remission was accomplished within the 2-year follow-up. The 32 patients with recalcitrant disease (38.1%) received additional treatment. Relapse occurred in 12 patients (14.3%) either during tapering of the PSL dose (six patients) or after achieving remission (six patients). Adverse events, mostly liver enzyme elevation, infections and diabetes, occurred in 67 patients (79.8%). One patient (1.2%) died during the observation period after gastrointestinal haemorrhage. Conclusions: Our results suggested that the elderly and patients requiring additional therapies were at higher risk of adverse events, including severe infections, and should thus be monitored carefully. This study provided clinical data that could inform revised guidelines and contribute to the evaluation of future novel therapies.

Original languageEnglish
Pages (from-to)1324-1330
Number of pages7
JournalJournal of the European Academy of Dermatology and Venereology
Volume34
Issue number6
DOIs
Publication statusPublished - 2020 Jun 1

ASJC Scopus subject areas

  • Dermatology
  • Infectious Diseases

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