Clinical and immunological features of pemphigus relapse

被引:24
作者
Ujiie, I [1 ]
Ujiie, H. [1 ]
Iwata, H. [1 ]
Shimizu, H. [1 ]
机构
[1] Hokkaido Univ, Grad Sch Med, Dept Dermatol, Sapporo, Hokkaido, Japan
基金
日本学术振兴会;
关键词
LINKED-IMMUNOSORBENT-ASSAY; VULGARIS; TRANSITION; FOLIACEUS; DISEASE; PROFILE; DESMOGLEIN-1; PHENOTYPE; SEVERITY; ELISA;
D O I
10.1111/bjd.17591
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
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.
引用
收藏
页码:1498 / 1505
页数:8
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