ミクリッツ病と全身性IgG4関連疾患 : 当科における systemic IgG4-related plasmacytic syndrome (SIPS) 40例の臨床的検討から [in Japanese] Mikulicz's disease and systemic IgG4-related plasmacytic syndrome (SIPS) [in Japanese]
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ミクリッツ病は涙腺・唾液腺腫脹を，自己免疫性膵炎は膵のびまん性腫脹を呈し，ともに腺組織中へのIgG4陽性形質細胞浸潤を特徴とする疾患である．私たちは，当科における全身性IgG4関連疾患（systemic IgG4-related plasmacytic syndrome ; SIPS）40例の臨床的特徴（腺分泌機能，血清学的評価，合併症，治療および予後）を解析した．男性は11例，女性は29例で，診断時の平均年齢は58.9歳であった．疾患の内訳は，ミクリッツ病33例，キュッツナー腫瘍3例，IgG4関連涙腺炎4例であった．涙腺・唾液腺分泌低下は，約6割の症例にみられたが，軽度であった．抗核抗体陽性率は15%，抗SS-A抗体陽性は1例のみ，低補体血症は30%に認められた．また自己免疫性膵炎，間質性腎炎，後腹膜線維症，前立腺炎などの合併を認めた．治療は，臓器障害を有する症例で治療開始時のステロイド量が多く，観察期間は最長16年のうち，臓器障害の有無に関わらず3例で再燃を認めた．ミクリッツ病をはじめとするSIPSの現時点における問題点と今後の展望について述べてみたい．<br>
Mikulicz's disease represents persistent enlargement of the lacrimal and salivary glands, and autoimmune pancreatitis is shown with diffuse pancreatic swelling. Both diseases are characterized with elevated IgG4 concentrations in the serum and prominent infiltration by plasmacytes expressing IgG4 in the glands. Clinical analyses were performed in 40 patients with systemic IgG4-related plasmacytic syndrome (SIPS) who consulted the doctors in Sapporo Medical University Hospital. Our patients were mainly middle-aged or elderly females. The average age was 58.9 years. The diagnosis was following ; 33 cases with Mikulicz's disease, 3 cases with Küttner's tumor, and 4 cases with IgG4-related dacryoadenitis. Slight dysfunction of lacrimal and salivary gland was observed in about 60% of them. Antinuclear antibodies were detected in only 15% of the cases with SIPS. Almost all, except one case, did not have anti-SS-A or anti-SS-B antibodies. Interestingly, hypocomplementemia was revealed in 30% of them. The complications of SIPS include autoimmune pancreatitis, tubulointerstitial nephritis, retroperitoneal fibrosis, prostatitis, and so on. SIPS is mainly treated by the administration of steroids. We started to prescribe much quantity of prednisolone to the patients with organ failure. The recurrence was admitted in the 3 patients for the followed 16 years. We present here the problems and prospects in SIPS.<br>
- Jpn. J. Clin. Immunol.
Jpn. J. Clin. Immunol. 31(1), 1-8, 2008-02-28
The Japan Society for Clinical Immunology