高IgG4血症と間質性腎炎を合併したミクリッツ病の1例

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  • A case of Mikulicz disease with high level of serum IgG4 and interstitial nephritis
  • コウIgG4 ケツショウ ト カンシツセイジンエン オ ガッペイ シタ ミクリッツビョウ ノ 1レイ

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Mikulicz disease is a unique condition involving persistent painless enlargements of lacrimal and salivary glands, but the cause is unknown. Previously, Mikulicz disease was included within the diagnostic category of primary Sjögren's syndrome, but now the concept of this disease has changed, because dryness of the eyes and oral cavity in Mikulicz disease is not so remarkable and is improved in response to glucocorticoid treatment. In cases with Mikulicz disease, the serum concentration of IgG4 is elevated and there is prominent infiltration of plasmacytes, which were shown by the immunoenzyme method to express IgG4, in gland tissue. On the other hand, autoimmune pancreatitis and tubulointerstitial nephritis are all related to IgG4 in their pathogenesis. Mikulicz disease may be a sub-type of systemic IgG4-related plasmacytic disease.<br>A 70-year-old man presented with a chief complaint of bilateral painless submandibular gland enlargement. He had been hospitalized for urethrectomy of interstitial nephritis with retroperitoneal fibrosis at the Urology Department of Tokyo Medical University Hospital 2 months previously. Blood examination revealed high serum IgG4. Needle biopsy of the submandibular gland was performed. Microscopically, severe infiltration of IgG4 positive plasmacytes was recognized in both surgical and needle biopsy specimens of the right submandibular gland. Blood examination also revealed high serum IgG4. We began to administer steroids under a diagnosis of Mikulicz disease with systemic IgG4-related plasmacytic disease. The high serum IgG4 levels decreased, and the bilateral enlargement of the submandibular glands subsided and disappeared.

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