涙腺生検を施行した免疫グロブリンG4関連慢性涙腺炎（Mikulicz病）の1例

A case of IgG4-related chronic dacryoadenitis（Mikulicz desease）diagnosed by lacrimal biopsy

要約　目的：両側の涙腺腫脹があり，生検でMikulicz病と診断された症例の報告。症例：70歳女性が2か月前からの両側の上眼瞼腫脹と唾液分泌低下で受診した。糖尿病と胃潰瘍の既往歴があった。所見：両側の上眼瞼耳側に弾性硬の腫瘤と両側の顎下腺腫脹があった。眼内には異常所見はなかった。血清のIgG4値が588mg/dlと高値であった。生検で得られた涙腺の免疫染色で，多数のIgG4陽性の形質細胞の浸潤があり，IgG4関連慢性涙腺炎と診断した。プレドニゾロンの内服で1か月後に涙腺腫脹は寛解した。6か月後に涙腺炎が再発し，硬化性胆管炎が生じ，IgG4関連多臓器疾患と診断された。結論：慢性涙腺炎ではIgG4関連多臓器疾患との鑑別が必要である。

Abstract.　Purpose：To report a case with bilateral swelling of lacrimal gland that was diagnosed with Mikulicz disease by biopsy. Case：A 70-year-old female presented with swelling of bilateral upper eyelid and decreased salivation since 2 months before. She had a history of diabetes mellitus and peptic ulcer. Findings：An elastic tumor was present temporal to the upper eyelids. Both parotid glands were swollen. Serum level of lgG4 was elevated at 588 mg/dl. The lacrimal gland obtained by biopsy showed numerous infiltration by plasma cells positive for lgG4 by immune staining. These findings led to the diagnosis of lgG4-related chronic dacryoadenitis or Mikulicz disease. Swelling of lacrimal glands subsided after one month of peroral prednisolone. Dacryoadenitis recurred 6 months later associated with sclerosing cholangitis. She was diagnosed with lgG4-related multisystem disease. Conclusion：This case illustrates that dacryoadenitis may be a manifestation of lgG4-related multisystem disease.