Immunoglobulin G4-related disease (IgG4RD) is a systemic fibro-inflammatory disease of unknown aetiology, which is characterized by tumefactive lymphoplasmatocytic infiltrative lesions, with a predominance of IgG4 positive plasma cells, fibrosis and obliterative phlebitis. Adult-onset asthma and periocular xanthogranuloma (AAPOX) is a rare disease of unknown aetiology characterized by violaceous or yellow cutaneous papules and nodules usually accompanied with adult-onset asthma. We report a case of IgG4RD associated with AAPOX. We also conducted a literature search with keywords including IgG4RD and AAPOX. A 36-year-old woman presented with bilateral exophthalmos and periorbital oedema. Keratoconjunctivitis sicca, painless left parotid gland and submandibular left lymph node enlargement were also noted. Two and half years ago biopsy of yellow plaques of her right lower eyelid confirmed periorbital xanthogranuloma. She underwent parotid gland biopsy which showed IgG4RD. She was treated with steroids and two cycles of rituximab with complete remission. The literature review revealed 8 articles describing 14 cases with IgG4RD and AAPOX, 9 men and 5 women (ratio M:F = 1.8:1) were affected. The age at diagnosis was greater in men compared to women. In the majority of patients, ophthalmic presentation included bilateral upper and lower eyelid swelling while systemic features were asthma, lacrimal and parotid involvement, lymphadenopathy, sclerosing pancreatitis and sclerosing cholangitis. Prednisone and rituximab were effective treatments. It has to be clarified whether the association between IgG4RD and AAPOX represents shared pathophysiology, a common underlying cause or coincidence. Rituximab added to steroids resulted in complete remission of the two entities.

中文翻译：

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利妥昔单抗成功治疗IgG4相关疾病并与成年哮喘和眼周黄原肉芽肿并存。

免疫球蛋白G4相关疾病（IgG4RD）是一种病因不明的全身性纤维炎性疾病，其特征是肿瘤性淋巴浆细胞浸润性病变，主要是IgG4阳性浆细胞，纤维化和闭塞性静脉炎。成人发作的哮喘和眼周黄肉芽肿（AAPOX）是一种病因不明的罕见疾病，其特征是紫色或黄色的皮肤丘疹和结节，通常伴有成人发作的哮喘。我们报告一例与AAPOX相关的IgG4RD。我们还使用IgG4RD和AAPOX等关键字进行了文献搜索。一名36岁的女性出现双侧眼球突出和眶周水肿。还发现干燥性角膜结膜炎，左腮腺无痛和下颌下左淋巴结肿大。两年半前，她的右下眼睑黄色斑块活检证实为眶周黄肉芽肿。她接受腮腺活检，显示IgG4RD。她接受了类固醇治疗和两个周期的利妥昔单抗治疗，完全缓解。文献综述发现有8篇文章描述了14例IgG4RD和AAPOX病例，其中9例男性和5例女性（比率M：F = 1.8：1）受到影响。与女性相比，男性的诊断年龄更大。在大多数患者中，眼科表现包括双侧上睑和下睑肿胀，而全身特征是哮喘，泪腺和腮腺受累，淋巴结肿大，硬化性胰腺炎和硬化性胆管炎。泼尼松和利妥昔单抗是有效的治疗方法。必须澄清IgG4RD和AAPOX之间的关联是否代表共同的病理生理，常见的根本原因或巧合。加入类固醇的利妥昔单抗导致两个实体完全缓解。