利妥昔单抗治疗黏膜类天疱疮：当前证据与临床展望
Rituximab in Mucous Membrane Pemphigoid: Current Evidence and Clinical Perspectives.
文献信息
| PMID | 42793600 |
|---|---|
| 原文 | 在 PubMed 查看原文 ↗ |
| 发表日期 | 2026 |
| 作者 | Liliana Gabriela Popa |
| 作者单位 | Department of Dermatology, Carol Davila University of Medicine and Pharmacy, No. 37 Dionisie Lupu Street, 030167 Bucharest, Romania. |
| 期刊 | Healthcare (Basel, Switzerland) |
| SCI 分区 | Q1 |
| IF | 3.3 |
| 研究类型 | 临床研究 · 临床 |
| 所属专科 | 咽喉科 |
中文摘要
背景： 黏膜类天疱疮（MMP）是一种慢性、致衰弱性疾病，具有显著的发病率。受累黏膜表面的进行性纤维化可能导致不可逆的并发症，包括继发于瘢痕性结膜炎的失明、食管狭窄、导致气道受损的喉狭窄以及伴功能受损的泌尿生殖道瘢痕形成。MMP的管理仍然极具挑战性。
目的： 评估过去5年在我们诊所诊断和治疗的MMP患者的人口学和临床特征，并分析在传统和生物制剂治疗下疾病的演变。
方法： 我们对2021年1月至2026年5月期间在我们诊所接受治疗的MMP患者进行了回顾性研究。
结果： 我们确定了8例MMP患者。从疾病发作到诊断的时间范围为1个月至10年。7例患者已经出现了该疾病的显著并发症，包括食管狭窄、视力损害或单侧视力丧失。尽管接受了糖皮质激素、免疫抑制剂或免疫调节药物治疗，所有患者的疾病均表现出进行性恶化。6例患者接受了利妥昔单抗治疗。尽管所有病例均实现了疾病控制，但仍需要继续全身传统治疗。达到显著临床改善的平均时间为2个月。1例患者在给予利妥昔单抗后12个月出现了严重复发，需要额外剂量。
结论： 根据我们的经验，我们支持在高危MMP患者中早期使用利妥昔单抗。在炎症期及时诊断和早期、积极治疗对于预防瘢痕形成至关重要。
英文摘要
Background: Mucous membrane pemphigoid (MMP) is a chronic, debilitating disease associated with significant morbidity. Progressive fibrosis of affected mucosal surfaces may result in irreversible complications, including blindness secondary to cicatricial conjunctivitis, esophageal strictures, laryngeal stenosis causing airway compromise, and genitourinary scarring with functional impairment. The management of MMP is still extremely challenging. Objectives: To assess the demographic and clinical characteristics of patients with MMP diagnosed and treated in our clinic during the past 5 years, and to analyze the evolution of the disease under conventional and biologic treatments. Methods: We conducted a retrospective study of patients with MMP treated in our clinic between January 2021 and May 2026. Results: We identified eight patients with MMP. The time from onset of the disease until diagnosis ranged from 1 month to 10 years. Seven patients had already developed significant complications of the disease, including esophageal stenosis, vision impairment, or unilateral vision loss. The disease exhibited progressive worsening in all patients, despite treatment with glucocorticoids, immunosuppressants, or immunomodulatory medication. Rituximab was administered to six patients. Although disease control was achieved in all cases, continuation of systemic conventional treatment was required. The mean time to significant clinical improvement was 2 months. One patient suffered a severe relapse 12 months following rituximab administration and required additional doses. Conclusions: Based on our experience, we support the early use of rituximab in high-risk patients with MMP. Prompt diagnosis and early, aggressive treatment in the inflammatory phase are essential to prevent scarring.