Internal Medicine, Madigan Army Medical Center, JBLM, Tacoma, Washington, USA
Internal Medicine/Infectious Disease, Madigan Army Medical Center, JBLM, Tacoma, Washington, USA.
BMJ Case Rep. 2020 Nov 23;13(11):e237614. doi: 10.1136/bcr-2020-237614.
IgG4-related autoimmune diseases (IgG4 RD) are a relatively recently recognised group of disease processes that can affect multiple organ systems and result in protean symptoms. Here, we present a rare case of a 69-year-old man with a history of IgG4 RD affecting his lacrimal gland and pancreas who developed symptoms of severe laryngitis not responsive to usual therapy. He presented with non-productive cough, hoarseness and dyspnoea. Imaging findings suggestive of aortitis and laryngeal inflammation in the setting of his IgG4 RD history prompted treatment with rituximab, which resulted in resolution of his laryngeal symptoms. Subsequently, his cough returned and he required periodic rituximab infusions to stay symptom-free. IgG4 RD of the larynx is an uncommonly reported manifestation in literature. This disease is very responsive to anti-CD20 monoclonal antibody treatment. IgG4 RD should be considered in patients with airway symptoms that are especially refractory to usual therapy.
IgG4 相关自身免疫性疾病（IgG4RD）是一组相对较新认识的疾病过程，可影响多个器官系统，并导致多种症状。在此，我们报告了一例罕见病例，一名 69 岁男性患有 IgG4RD，影响其泪腺和胰腺，出现严重喉炎症状，对常规治疗无反应。他表现为无痰咳嗽、声音嘶哑和呼吸困难。影像学检查提示大动脉炎和喉炎，结合 IgG4RD 病史，提示使用利妥昔单抗治疗，导致其喉症状缓解。随后，他的咳嗽又回来了，需要定期输注利妥昔单抗才能无症状。喉 IgG4RD 在文献中报道较少。这种疾病对抗 CD20 单克隆抗体治疗非常敏感。对于气道症状特别难治的患者，应考虑 IgG4RD。
