Adult Onset Asthma and Periocular Xanthogranuloma (AAPOX), a Rare Entity With a Strong Link to IgG4-Related Disease: An Observational Case Report Study.
Adult Onset Asthma and Periocular Xanthogranuloma (AAPOX), a Rare Entity With a Strong Link to IgG4-Related Disease: An Observational Case Report Study.
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DOI：
10.1097/md.0000000000001916
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发表时间：
2015-10
期刊：
影响因子：
1.6
通讯作者：
Abad S
中科院分区：
文献类型：
--
作者：
London J;Martin A;Soussan M;Badelon I;Gille T;Uzunhan Y;Giroux-Leprieur B;Warzocha U;Régent A;Galatoire O;Dhote R;Abad S
成人发病的哮喘和眼周黄色肉芽肿 (AAPOX) 是一种罕见的非朗格汉斯组织细胞增多症，其组织病理学特征为眼周泡沫状组织细胞和 Touton 巨细胞浸润。 AAPOX 患者的眼睑或淋巴结中典型地描述了伴有浆细胞浸润的良性增生。这也是 IgG4 相关疾病 (IgG4-RD) 的一个特征，这是一种由携带 IgG4 的浆细胞浸润器官定义的新疾病。为了确定 AAPOX 综合征是否与 IgG4-RD 具有共同的临床、生物学和组织病理学特征，我们在连续三名经组织学证实的 AAPOX 患者的回顾性病例系列中使用了 IgG4-RD 的综合临床诊断标准。 1996 年 11 月至 2013 年 3 月期间在法国眼眶炎症学术转诊中心诊断为 AAPOX 的患者被纳入研究。对眼附属器或其他器官的活组织检查进行了系统的重新检查。对每位患者的临床和血清学数据、放射学结果和治疗进行回顾性分析。两名 AAPOX 患者满足明确 IgG4-RD 的所有诊断标准。一名缺乏血清学标准的患者符合可能 IgG4-RD 的标准。这3例AAPOX患者均符合IgG4-RD综合临床诊断标准。据我们所知，这是第一个观察性病例报告研究，清楚地表明 IgG4-RD 与 AAPOX 综合征之间存在密切关系。
Adult onset asthma and periocular xanthogranuloma (AAPOX) is a rare non-Langerhans histiocytosis characterized histopathologically by a periocular infiltration of foamy histiocytes and Touton giant cells. Benign hyperplasia with plasma cell infiltration is classically described in eyelids or lymph nodes of AAPOX patients. It is also a characteristic feature of IgG4-related disease (IgG4-RD), a new entity defined by an IgG4-bearing plasma cell infiltration of organs. To determine if AAPOX syndrome shares clinical, biological, and histopathological characteristics with IgG4-RD, we used the comprehensive clinical diagnostic criteria for IgG4-RD in a retrospective case series of three consecutive patients with histologically-proven AAPOX. Patients who were diagnosed with AAPOX at a French academic referral center for orbital inflammation between November 1996 and March 2013 were enrolled. Biopsies from ocular adnexa or other organs were systematically reexamined. For each patient, clinical and serological data, radiologic findings, and treatment were retrospectively analyzed. Two AAPOX patients fulfilled all of the diagnostic criteria for a definite IgG4-RD. One patient who lacked the serological criteria fulfilled the criteria of a probable IgG4-RD. These 3 cases of AAPOX patients fulfilled the IgG4-RD comprehensive clinical diagnostic criteria. To our knowledge, this is the first observational case report study to clearly show a strong relationship between IgG4-RD and AAPOX syndrome.