Barmatz Shira, Baniel Avital, Eremenko Ron, Neiderman Narin Nard Carmel, Wasserzug Oshri, Sprecher Eli, Oestreicher-Kedem Yael
Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel; Sackler School of Medicine, Tel-Aviv University, Tel Aviv, Israel.
Department of Otolaryngology, Head and Neck Surgery and Maxillofacial Surgery, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel; Sackler School of Medicine, Tel-Aviv University, Tel Aviv, Israel.
J Voice. 2023 May;37(3):471.e7-471.e14. doi: 10.1016/j.jvoice.2021.01.012. Epub 2021 Feb 12.
This study aimed to present our experience in treating laryngeal pemphigoid (LP) patients, including disease course, treatment and treatment response, and to search for predictors of response to treatment.
A retrospective cohort study.
The medical records of all patients with LP from March 2013 to August 2020 were reviewed. Potential relationships between disease severity and response to treatment and demographics, diagnosis, extent of laryngeal and extra-laryngeal involvement, comorbidities, immunostaining, and serology profile were explored.
Eight patients were included in the study (seven females, one male, mean age 79 years, mean follow-up 22 months). Diagnoses included mucous membrane pemphigoid (MMP, n = 5), bullous pemphigoid (BP, n = 3). Two patients achieved complete laryngeal remission, four achieved partial remission, and two had no remission. The time to achieve laryngeal disease control was longer than for extra-laryngeal disease (P = 0.02). Potential associations were found between the absence of immunoglobulin G (IgG)-type auto-antibodies deposits in the basement membrane zone and a laryngeal disease that responded to topical corticosteroids and between the presence of BP180-C-terminal IgG auto-antibodies and a resistant rapidly progressive laryngeal disease.
LP has a spectrum of severity, variable response and is more resistant to treatment. The absence of IgG-type auto-antibodies may indicate a response to topical corticosteroids. Based on our limited observation, the presence of IgG-type auto-antibodies that target BP180-C-terminal domain may indicate a more severe scarring disease. Early recognition of these "high-risk patients" will allow early initiation of advanced systemic treatment that may prevent the irreversible effects of scarring.
本研究旨在介绍我们治疗喉类天疱疮（LP）患者的经验，包括病程、治疗及治疗反应，并寻找治疗反应的预测因素。
一项回顾性队列研究。
回顾了2013年3月至2020年8月期间所有LP患者的病历。探讨了疾病严重程度与治疗反应以及人口统计学、诊断、喉及喉外受累范围、合并症、免疫染色和血清学特征之间的潜在关系。
本研究纳入8例患者（7例女性，1例男性，平均年龄79岁，平均随访22个月）。诊断包括黏膜类天疱疮（MMP，n = 5）、大疱性类天疱疮（BP，n = 3）。2例患者实现喉部完全缓解，4例部分缓解，2例未缓解。实现喉部疾病控制的时间比喉外疾病更长（P = 0.02）。发现基底膜区无免疫球蛋白G（IgG）型自身抗体沉积与对局部糖皮质激素有反应的喉部疾病之间以及存在BP180 - C末端IgG自身抗体与耐药性快速进展的喉部疾病之间存在潜在关联。
LP有一系列严重程度，反应各异且对治疗更具抗性。无IgG型自身抗体可能表明对局部糖皮质激素有反应。基于我们有限的观察，靶向BP180 - C末端结构域的IgG型自身抗体的存在可能表明疾病瘢痕形成更严重。早期识别这些“高危患者”将有助于早期启动可能预防瘢痕形成不可逆影响的高级全身治疗。
