Renal Lesions in IgG4-Related Systemic Disease

• Saeki Takako

  Department of Internal Medicine, Nagaoka Red Cross Hospital

• Nishi Shinichi

  Divisions of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences

• Ito Tomoyuki

  Department of Internal Medicine, Nagaoka Red Cross Hospital

• Yamazaki Hajime

  Department of Internal Medicine, Nagaoka Red Cross Hospital

• Miyamura Syoji

  Department of Internal Medicine, Nagaoka Red Cross Hospital

• Emura Iwao

  Department of Pathology, Nagaoka Red Cross Hospital

• Imai Naofumi

  Divisions of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences

• Ueno Mitsuhiro

  Joetsu University of Education

• Saito Akihiko

  Department of Applied Molecular Medicine, Niigata University Graduate School of Medical and Dental Sciences

• Gejyo Fumitake

  Divisions of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences

抄録

Objective Recently, a new concept of IgG4-related systemic disease including autoimmune pancreatitis, characterized by a high serum IgG4 level and tissue infiltration by IgG4-positive plasma cells, has been proposed. Our aim was to investigate the renal involvement in this condition.<br> Patients and Methods We investigated the results of laboratory and imaging studies of the kidneys in 7 patients with IgG4-related systemic disease, and examined the renal histology in four of them. All patients showed elevated serum IgG4 levels, and 4 had autoimmune pancreatitis. The other three patients showed involvement of various extrapancreatic organs (lymphadenopathy, sialadenitis or renal insufficiency), and abundant IgG4-positive plasma cell infiltration was confirmed in their affected tissues.<br> Results Six of the 7 patients showed some renal abnormalities. In one patient, hydronephrosis was observed accompanied by retroperitoneal fibrosis. Another patient showed multiple low-density areas in both kidneys by computed tomography, and gallium citrate scintigraphy showed gallium-67 accumulation in both kidneys, although renal function was normal. Four patients had tubulointerstitial nephritis. In two of them, the tubulointerstitial nephritis was diffuse. In one patient, marked diffuse but patchily distributed lymphoplasmacytic infiltration of the renal interstitium was observed. In another patient, computed tomography showed a tumor-like low-density mass; open biopsy of the mass showed aggregates of lymphocytes and plasma cells in the renal interstitium.<br> Conclusion Renal parenchymal lesions in IgG4-related systemic disease are due to dense lymphoplasmacytic infiltration of the renal interstitium, and the lesions vary from diffuse tubulointerstitial nephritis to tumor-like masses according to the distribution patterns of the infiltrating cells.<br>

収録刊行物

• Internal Medicine

  Internal Medicine 46 (17), 1365-1372, 2007

  一般社団法人 日本内科学会

キーワード

• Autoimmune pancreatitis
• tubulointerstitial nephritis
• sclerosing sialadenitis
• Mikulicz's disease

詳細情報

• CRID: 1390001204871448320
• NII論文ID: 130000076056
• DOI: 10.2169/internalmedicine.46.0183
• ISSN: 13497235
• ISSN: 09182918
• Web Site: http://www.jstage.jst.go.jp/article/internalmedicine/46/17/46_17_1365/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2008154110
• 本文言語コード: en
• データソース種別:
  • JaLC
  • Crossref
  • CiNii Articles
• 抄録ライセンスフラグ: 使用不可