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Clinical and immunological features of pemphigus relapse

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Title: Clinical and immunological features of pemphigus relapse
Authors: Ujiie, I Browse this author
Ujiie, H. Browse this author →KAKEN DB
Iwata, H. Browse this author
Shimizu, H. Browse this author →KAKEN DB
Issue Date: Jun-2019
Publisher: John Wiley & Sons
Journal Title: British journal of dermatology
Volume: 180
Issue: 6
Start Page: 1498
End Page: 1505
Publisher DOI: 10.1111/bjd.17591
PMID: 30585310
Abstract: Background More than half of patients with pemphigus experience relapse during the disease course. The risk factors and clinical and immunological characteristics of relapse remain largely unclear. Objectives To elucidate the risk factors and clinical features of pemphigus relapse. Methods We carried out a retrospective review of the clinical records of 42 cases of pemphigus at a single centre. Results Sixty-two per cent of patients experienced relapse, usually when oral prednisolone was tapered to around 0 center dot 1 mg kg(-1). In mucocutaneous pemphigus vulgaris (mcPV), the initial doses (mean +/- SD) of prednisolone were significantly lower in patients with relapse (0 center dot 78 +/- 0 center dot 24 mg kg(-1)) than in those without relapse (1 center dot 01 +/- 0 center dot 01 mg kg(-1)). At relapse, mcPV shifted to mucosal dominant PV (mPV; 40%), pemphigus foliaceus (PF) (20%) or 'other' (20%). In contrast, relapsing mPV and PF had the same clinical phenotypes as the initial phenotypes. Patients with both anti-desmoglein (Dsg)1 and anti-Dsg3 antibodies at onset had recurrence with anti-Dsg3 antibodies alone (40%), with both anti-Dsg1 and anti-Dsg3 antibodies (30%), with anti-Dsg1 antibody alone (20%) or were subthreshold (10%). Conclusions mcPV shows transitions in clinical phenotype and autoantibody profile at relapse. At least 1 mg kg(-1) daily of prednisolone, especially for patients with mcPV, and prudent tapering around 0 center dot 1 mg kg(-1) may lead to better outcomes.
Rights: This is the peer reviewed version of the following article: I Ujiie, H Ujiie, H Iwata, and H Shimizu. (2019) Clinical and immunological features of pemphigus relapse. British Journal of Dermatology, 180(6): 1498-1505., which has been published in final form at This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions.
Type: article (author version)
Appears in Collections:北海道大学病院 (Hokkaido University Hospital) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

Submitter: 氏家 韻欣

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