Babu Manu, Goyal Akshita, Chavan Paresh
Department of Otorhinolaryngology and Head Neck Surgery, Dr D.Y. Patil College, Hospital and Research Center, Dr D. Y. Patil Vidyapeeth, Pimpri, Pune, Maharashtra 411018 India.
Indian J Otolaryngol Head Neck Surg. 2025 Apr;77(4):1891-1895. doi: 10.1007/s12070-025-05398-4. Epub 2025 Mar 10.
Immunoglobulin G4 related disease (IgG4-RD) is a rare immune mediated condition characterized by extensive tissue fibrosis and infiltration by immunoglobulin G4 positive plasma cells in a single or multiple organs. Earlier known to be a rare occurrence, it is becoming increasingly documented worldwide. Organs predominantly involved in this condition are pancreas, salivary glands and biliary tract while in the head and neck region, the salivary, lacrimal and pituitary glands are commonly affected structures. There are limited documented cases reporting IgG4-RD affecting the nasal cavity and paranasal sinuses to the best of our knowledge. Here in we report a case of a young female with complains of chronic rhinosinusitis, previously misdiagnosed on multiple occasions, later diagnosed with IgG4-RD on the basis of histopathology. Even though the prognosis is favorable, but delay in diagnosis and therapy can lead to multi organ failure and premature death hence warranting the need for discussion.
免疫球蛋白G4相关性疾病（IgG4-RD）是一种罕见的免疫介导疾病，其特征为单个或多个器官出现广泛的组织纤维化以及免疫球蛋白G4阳性浆细胞浸润。该疾病此前被认为较为罕见，但如今在全球范围内的报道日益增多。主要受累器官为胰腺、唾液腺和胆道，而在头颈部区域，唾液腺、泪腺和垂体是常见的受累结构。据我们所知，报道IgG4-RD累及鼻腔和鼻窦的病例有限。在此，我们报告一例年轻女性病例，该患者主诉慢性鼻窦炎，此前曾多次误诊，后来根据组织病理学诊断为IgG4-RD。尽管预后良好，但诊断和治疗的延迟可能导致多器官功能衰竭和过早死亡，因此有必要进行讨论。
