Liard Yannick, Nigolian Haïg, Lobrinus Johannes Alexander, Lenoir Vincent, Becker Minerva, Nieke Jan Philipp, Seebach Jörg D, Landis Basile, Guinand Nils
Service d'ORL et de chirurgie cervico-faciale, Département des neurosciences cliniques, HUG, 1211 Genève 14.
Service d'immunologie et d'allergologie, Département de médecine, HUG, 1211 Genève 14.
Rev Med Suisse. 2020 Oct 7;16(709):1870-1875.
IgG4-related disease is a rare multi-organic inflammatory disease that frequently involves the ENT and head and neck areas. In these cases, salivary gland and lacrimal gland involvement is very common and includes enlargement, infiltration, and formation of pseudotumours. Diagnosis of IgG4 related disease remains a challenge and relies on several clinical, serological, radiological and histopathological criteria to differentiate from other diseases with similar clinical presentation. Histology reveals IgG4 positive lymphoplasmocytic infiltrates, storiform fibrosis and obliterative phlebitis. Glucocorticoids are the first line of treatment and can be combined with other immunosuppressants. The prognosis is favorable if treatment is initiated early. Recurrences are common. Delay in diagnosis can have severe multi-organic consequences.
IgG4相关性疾病是一种罕见的多器官炎症性疾病，常累及耳鼻喉及头颈部区域。在这些病例中，唾液腺和泪腺受累非常常见，包括腺体肿大、浸润以及假瘤形成。IgG4相关性疾病的诊断仍然是一项挑战，需要依靠多种临床、血清学、影像学和组织病理学标准来与具有相似临床表现的其他疾病进行鉴别。组织学检查显示IgG4阳性淋巴细胞浆细胞浸润、席纹状纤维化和闭塞性静脉炎。糖皮质激素是一线治疗药物，可与其他免疫抑制剂联合使用。如果早期开始治疗，预后良好。复发很常见。诊断延迟可能会导致严重的多器官后果。
