A Serological and Pathological Study of IgG4-related Plasmacytic Disease: Mikulicz’s Disease and Chronic Sclerosing Sialadentis of the Submandibular Gland (Küttner’s Tumor)

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  • IgG4関連疾患としてのミクリッツ病，慢性硬化性顎下腺炎（キュットナー腫瘍）の血清学的および臨床病理学的検討
  • 臨床 IgG4関連疾患としてのミクリッツ病,慢性硬化性顎下腺炎(キュットナー腫瘍)の血清学的および臨床病理学的検討
  • リンショウ IgG4 カンレン シッカン ト シテ ノ ミクリッツビョウ,マンセイ コウカセイ ガク カセンエン(キュットナー シュヨウ)ノ ケッセイガクテキ オヨビ リンショウ ビョウリガクテキ ケントウ

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Abstract

IgG4-related plasmacytic disease is associated with the dissemination of CD4 or CD8 positive T lymphocytes and IgG4-positive plasma cells in numerous internal organs. Mikulicz’s disease has been characterized by bilateral swelling of the lacrimal glands, parotid and submandibular glands, whereas Küttner’s tumor has been reported as bilateral submandibular gland swelling, and has been referred to as chronic sclerosing sialadentis. Mikulicz’s disease has recently been categorized as a systemic IgG4-related disease. In the present study, 31 cases of Mikulicz’s disease and 20 cases of chronic sclerosing sialadentis were investigated from various points of view such as by visual confirmation, laboratory findings, histopathological findings, and coexisting diseases. Laboratory findings showed that total IgG and IgG4 levels were significantly higher in both diseases. Histopathologically, IgG4 positive plasma cell infiltration with fibrosis was remarkable in both diseases. These results suggest that Küttner’s tumor is also categorized in Mikulicz’s disease as an IgG4-related disease.<br>

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