Department of Otorhinolaryngology, Kanto Rosai Hospital, Nakahara-ku, Kawasaki, Japan.
Department of Otorhinolaryngology, Showa University Fujigaoka Hospital, Aoba-ku, Yokohama, Japan.
Medicine (Baltimore). 2022 Dec 30;101(52):e32617. doi: 10.1097/MD.0000000000032617.
IgG4-related diseases cause lesions in various organs throughout the body. In otorhinolaryngology, IgG4-related Mikulicz's disease is suspected and diagnosed based on the presence of lesions of the head and neck, salivary and lacrimal gland enlargement, and bilateral sinus opacity concentrated on the maxillary sinuses. However, in some cases, it is necessary to consider about differentiation between IgG4-related Mikulicz's disease and Sjögren syndrome.
A 75-years-old male patient visited our hospital with bilateral otitis media with effusion, which was resistant to conservative treatment. Other symptoms at presentation included enlarged bilateral submandibular and sublingual glands marked oral dryness, severe decrease in saliva secretion (1 mL/10 minutes), and dry eyes. We conducted a Schirmer's and fluorescent dye tests, both of which were positive. High serum IgG4 levels were observed, and although the Sjögren syndrome (SS)-A/SS-B antibodies were negative, marked hypolacrimation and tear secretion were observed. Therefore, a detailed examination considering both IgG4-related Mikulicz's disease and SS was conducted. Salivary gland scintigraphy performed prior to the salivary gland biopsy revealed a marked decrease in uptake, which satisfied the diagnostic criteria for SS; however, it was difficult to diagnose IgG4-related disease based on the diagnostic definition.
Although a definitive diagnosis of SS was made, the persistent otitis media with effusion that was resistant to conservative treatment and bilateral mixed hearing loss were confirmed. As mixed hearing loss is considered an otological symptom of IgG4-related disease, oral steroid treatment was administered.
Thereafter, marked recovery of hearing and reduced swelling and induration of the bilateral parotid and submandibular glands were observed. Clinically, IgG4-related Mikulicz's disease was strongly suspected, but a definite diagnosis of SS was made.
In the absence of an IgG4-related Mikulicz's disease diagnosis, careful differentiation between IgG4-related Mikulicz's disease and 2 diseases and their diagnostic criteria was essential.
IgG4 相关疾病可导致全身各种器官的病变。在耳鼻喉科，根据头颈部病变、唾液腺和泪腺肿大以及双侧上颌窦集中的鼻窦不透光，怀疑并诊断 IgG4 相关 Mikulicz 病。然而，在某些情况下，有必要考虑 IgG4 相关 Mikulicz 病与干燥综合征之间的鉴别诊断。
一名 75 岁男性患者因双侧分泌性中耳炎就诊，经保守治疗无效。就诊时的其他症状包括双侧颌下和舌下腺肿大、明显口干、唾液分泌严重减少（10 分钟 1 毫升）和干眼症。我们进行了 Schirmer 和荧光染料试验，均为阳性。观察到血清 IgG4 水平升高，尽管 Sjögren 综合征（SS）-A/SS-B 抗体阴性，但明显低泪液和泪液分泌。因此，进行了详细的检查，同时考虑了 IgG4 相关 Mikulicz 病和 SS。在进行唾液腺活检之前进行的唾液腺闪烁扫描显示摄取明显减少，符合 SS 的诊断标准；然而，根据诊断标准，难以诊断 IgG4 相关疾病。
尽管做出了明确的 SS 诊断，但仍存在对保守治疗无效的持续性分泌性中耳炎和双侧混合性听力损失。由于混合性听力损失被认为是 IgG4 相关疾病的耳科症状，因此给予口服类固醇治疗。
此后，观察到听力显著恢复，双侧腮腺和颌下腺肿胀和硬结减少。临床上强烈怀疑 IgG4 相关 Mikulicz 病，但做出了明确的 SS 诊断。
在没有 IgG4 相关 Mikulicz 病诊断的情况下，仔细区分 IgG4 相关 Mikulicz 病与 2 种疾病及其诊断标准至关重要。
