中华皮肤科杂志 ›› 2022, Vol. 55 ›› Issue (2): 123-128.doi: 10.35541/cjd.20210318

• 论著 • 上一篇    下一篇

硬皮病样皮肤移植物抗宿主病24例临床特征分析

于聪    周城    张建中   

  1. 北京大学人民医院皮肤科，北京  100044
  • 收稿日期:2021-04-21 修回日期:2021-12-15 发布日期:2022-01-27
  • 通讯作者: 张建中 E-mail:rmzjz@126.com
  • 作者简介:张建中教授要求关照。5.6
  • 基金资助:
    国家自然科学基金（82073459）

Clinical features of 24 cases of scleroderma-like cutaneous graft-versus-host disease

Yu Cong, Zhou Cheng, Zhang Jianzhong   

  1. Department of Dermatology, Peking University People′s Hospital, Beijing 100044, China
  • Received:2021-04-21 Revised:2021-12-15 Published:2022-01-27
  • Contact: Zhang Jianzhong E-mail:rmzjz@126.com
  • Supported by:
    National Natural Science Foundation of China（82073459）

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摘要： 【摘要】 目的 分析异基因造血干细胞移植后硬皮病样皮肤移植物抗宿主病（GVHD）的临床特征和危险因素。方法 回顾2014—2019年间就诊于北京大学人民医院皮肤科硬皮病样皮肤GVHD患者24例的临床资料，分析临床特征、治疗、预后及发展为硬皮病样皮肤GVHD的可能因素。结果 24例硬皮病样皮肤GVHD患者中男11例，女13例，年龄（33 ± 12）岁，HLA全相合20例，半相合4例，发病时间［M（Q1，Q3）］为移植后18.5（8.0，30.9）个月。19例发病前已停用预防排异药或药物处于小剂量。15例表现为泛发性硬皮病样，1例为线状硬皮病样，5例硬斑病样，3例筋膜炎样。泛发性硬皮病样皮肤GVHD无雷诺征。13例伴有其他系统排异，8例合并关节活动受限，1例合并慢性皮肤溃疡并继发皮肤鳞状细胞癌。所有患者均给予系统糖皮质激素或联合免疫抑制剂，11例外用糖皮质激素。对11例患者进行集中随访，其中明显好转3例，好转4例，加重2例，死亡2例。以同时段就诊的223例非硬皮病样皮肤GVHD患者为对照，硬皮病样皮肤GVHD组HLA全相合患者比例（20/24，83.3%）高于非硬皮病样皮肤GVHD患者（47/223，21.1%），两组比较，P < 0.001。结论 硬皮病样皮肤GVHD平均发病时间较晚，可模仿所有4种自发性硬皮病的表现，HLA全相合移植、过早停用或预防排异药减量过快可能是发展至硬皮病样皮肤GVHD的危险因素。

关键词: 移植物抗宿主病, 造血干细胞移植, 硬皮病样, 危险因素

Abstract: 【Abstract】 Objective To investigate clinical features of and risk factors for scleroderma-like cutaneous graft-versus-host disease（GVHD） after allogeneic hematopoietic stem cell transplantation. Methods Clinical data were collected from 24 patients with scleroderma-like cutaneous GVHD in Department of Dermatology, Peking University People′s Hospital from 2014 to 2019. Clinical features, treatment, prognosis, and possible factors influencing the development of scleroderma-like cutaneous GVHD were analyzed retrospectively. Results Among the 24 patients, 11 were males, and 13 were females, aged 33 ± 12 years; 20 were human leukocyte antigen （HLA）-identical recipients, 4 were HLA-haploidentical recipients; GVHD occurred 18.5 （8.0, 30.9） months after transplantation. Nineteen patients had discontinued anti-rejection therapy or received low-dose anti-rejection drugs before the onset of GVHD. Fifteen patients presented with generalized scleroderma-like lesions, 1 with linear scleroderma-like lesions, 5 with morphea-like lesions, and 3 with fasciitis-like lesions. None of the 15 patients with generalized scleroderma-like GVHD had Raynaud syndrome. Thirteen patients were accompanied by graft rejection in other systems, 8 had joint mobility limitations, and 1 developed cutaneous squamous cell carcinoma secondary to chronic skin ulcers. All patients were treated with systemic glucocorticoids and immunosuppressive agents, and 11 also with topical glucocorticoids. An intensive follow-up was carried out in 11 patients, of whom 3 achieved marked improvement, 4 achieved improvement, 2 experienced exacerbation, and 2 died. A total of 223 patients with non-sclerodermatous cutaneous GVHD admitting during the same period served as controls, and the proportion of HLA-identical patients was significantly higher in the scleroderma-like cutaneous GVHD group （20/24, 83.3%） than in the non-sclerodermatous cutaneous GVHD group （47/223, 21.1%; P < 0.001）. Conclusions Scleroderma-like cutaneous GVHD commonly occurs late, and can mimic clinical manifestations of all 4 types of spontaneous scleroderma. HLA-identical transplants, premature discontinuation or excessive dose reduction of anti-rejection drugs may be risk factors for scleroderma-like cutaneous GVHD.

Key words: Graft vs host disease, Hematopoietic stem cell transplantation, Scleroderma-like, Risk factor