Clinical and immunological features of pemphigus relapse
Clinical and immunological features of pemphigus relapse
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DOI：
10.1111/bjd.17591
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发表时间：
2019-06-01
影响因子：
10.3
通讯作者：
Shimizu, H.
中科院分区：
文献类型：
--
作者：
Ujiie, I;Ujiie, H.;Shimizu, H.
背景：超过一半的天疱疮患者在病程中会复发。复发的危险因素以及临床和免疫学特征在很大程度上仍不清楚。目的探讨天疱疮复发的危险因素及临床特点。方法对同一中心收治的42例天疱疮的临床资料进行回顾性分析。结果62%的患者复发，通常在口服泼尼松龙至0中心点1 mg kg(-1)左右时复发。在寻常型天疱疮(McPV)中，复发组强的松龙初始剂量(0中心点78+/-0中心点24 mg kg(-1))显著低于未复发组(1中心点01+/-0中心点01 mg kg-1)。复发时，mcpv转变为黏膜占优势的pv(40%)、叶天疱疮(Pf)(20%)或其他(20%)。相反，复发的MPV和PF具有与初始表型相同的临床表型。起病时同时抗桥粒芯糖蛋白(Dsg1)和抗Dsg3抗体的患者，复发时仅有抗Dsg3抗体(40%)，同时有抗Dsg1和抗Dsg3抗体(30%)，单独抗DSG1抗体(20%)或亚阈值(10%)。结论mcpv在复发时表现为临床表型和自身抗体谱的转变。每天至少服用1 mg kg(-1)强的松龙，尤其是对于患有McPV的患者，谨慎地减少0中心点1 mg kg(-1)可能会导致更好的结果。
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.