TY - JOUR
T1 - Results of the guideline-based treatment for pemphigus
T2 - a single-centre experience with 84 cases
AU - Kakuta, R.
AU - Kurihara, Yuichi
AU - Yamagami, Jun
AU - Miyamoto, J.
AU - Funakoshi, T.
AU - Tanikawa, A.
AU - Amagai, M.
N1 - Funding Information:
Funding sources Health and Labor Sciences Research Grants for Research on Measures for Intractable Disease from the Ministry of Health, Labor and Welfare of Japan, and Intramural research fund of Keio University School of Medicine.
Funding Information:
Health and Labor Sciences Research Grants for Research on Measures for Intractable Disease from the Ministry of Health, Labor and Welfare of Japan, and Intramural research fund of Keio University School of Medicine.
Publisher Copyright:
© 2020 European Academy of Dermatology and Venereology
PY - 2020/6/1
Y1 - 2020/6/1
N2 - 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.
AB - 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.
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U2 - 10.1111/jdv.16188
DO - 10.1111/jdv.16188
M3 - Article
C2 - 31923338
AN - SCOPUS:85079222984
VL - 34
SP - 1324
EP - 1330
JO - Journal of the European Academy of Dermatology and Venereology
JF - Journal of the European Academy of Dermatology and Venereology
SN - 0926-9959
IS - 6
ER -