ステロイド療法により耐糖能障害の改善を認めた自己免疫性膵炎合併 Mikulicz 病の1例  [in Japanese] A case of Mikulicz's disease complicated by autoimmune pancreatitis, in which impaired glucose tolerance was improved by glucocorticoid treatment  [in Japanese]

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Abstract

  症例は,73歳女性.1998年頃より口渇,両側上眼瞼腫脹が出現,2003年10月には両側顎下部腫脹を認めた.同時期に近医で糖尿病と診断され,経口血糖降下薬の投与が開始された.2004年夏頃より上眼瞼腫脹が増強したため,当科受診,精査加療目的にて10月に入院となった.頭部CT・MRIでは,両側涙腺・顎下腺腫脹を認めた.血清学的に高γグロブリン血症を認めたが,抗核抗体・抗SS-A抗体は陰性であり,乾燥性角結膜炎も認めなかった.さらなる精査で,高IgG4血症及び小唾液腺生検にて著明なIgG4陽性形質細胞浸潤を認めたため,Mikulicz病と診断した.腹部CTではびまん性膵腫大を認め,ERCPで総胆管・主膵管に狭窄を認めた.自己免疫性膵炎の合併と診断し,プレドニゾロン40 mg/日より治療を開始した.その結果,涙腺・顎下腺腫脹は消失,唾液分泌能も回復した.また膵腫大,総胆管・膵管狭窄も改善した.耐糖能障害も回復傾向にある.Mikulicz病と自己免疫性膵炎は共に高IgG4血症を呈し,組織中にIgG4陽性形質細胞浸潤を認めることから,両疾患の関連を考える上で非常に興味深い症例であると思われた.<br>

  A 73-year-old woman had experienced dry mouth and swellings of both upper eyelids from 1998. In October 2003, she also developed bilateral submandibular swellings, and was diagnosed with diabetes mellitus and prescribed antidiabetic medication. She consulted our hospital in the summer of 2004 due to the exacerbation of eyelid swelling, and was admitted in October 2004. Keratoconjunctivitis sicca was not present. CT and MRI of the head showed bilateral enlargement of the lacrimal and submandibular glands. Serological investigations revealed hypergammaglobulinemia, but as antinuclear antibody and anti-SS-A antibody were absent, further investigation was performed. Serum concentrations of IgG4 were elevated and biopsy of the minor salivary gland revealed a severe infiltration of IgG4-positive plasmacytes. The patient was therefore diagnosed with Mikulicz's disease. Abdominal CT demonstrated diffuse pancreatic swelling, and endoscopic retrograde cholangio-pancreatography revealed stricture of the common bile duct and main pancreatic duct, suggesting the complication of autoimmune pancreatitis. Treatment was commenced with 40 mg/day of prednisolone. This resulted in rapid resolution of the lacrimal and submandibular gland swellings and recovery of salivary gland function. Diffuse swelling of the pancreas and stricture of the common bile duct and main pancreatic duct also improved, and endogenous insulin secretion increased. Both Mikulicz's disease and autoimmune pancreatitis presented with elevated serum IgG4 and infiltration of IgG4-expressing plasma cells into the glandular tissues. We recently proposed the new diagnostic entity of “IgG4-related plasmacytic exocrinopathy” ; however, if diabetes mellitus in autoimmune pancreatitis was caused by direct dysfunction of pancreatic ? cells, we must reconsider this pathogenesis and consider a wider concept including exocrine as well as endocrine glands. This case, in which both types of glands were affected, is therefore of considerable interest.<br>

Journal

  • Japanese Journal of Clinical Immunology

    Japanese Journal of Clinical Immunology 28(5), 349-356, 2005-10-31

    The Japan Society for Clinical Immunology

References:  44

Cited by:  9

Codes

  • NII Article ID (NAID)
    10016912443
  • NII NACSIS-CAT ID (NCID)
    AN00357971
  • Text Lang
    JPN
  • Article Type
    Journal Article
  • ISSN
    09114300
  • Data Source
    CJP  CJPref  J-STAGE 
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