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How to diagnose IgG4-related disease
  1. Hisanori Umehara1,
  2. Kazuichi Okazaki2,
  3. Mitsuhiro Kawano3,
  4. Tsuneyo Mimori4,
  5. Tsutomu Chiba5
  1. 1Division of RA and Autoimmune Diseases, Nagahama City Hospital, Nagahama, Shiga, Japan
  2. 2Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
  3. 3Division of Rheumatology, Department of Internal Medicine, Graduate School of Medical Science, Kanazawa University, Ishikawa, Japan
  4. 4Department of Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
  5. 5Department of Gastroenterology and Hepatology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
  1. Correspondence to Professor Hisanori Umehara, Division of Rheumatology and Immunology, Nagahama City Hospital, 313, Oinuicho Nagahama, Shiga 526-0043, Japan; umehara606{at}

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We read with great interest the editorial by Fox and Fox1 describing the use of serum immunoglobulin G4 (IgG4) concentrations as a marker for IgG4-related disease (IgG4-RD). IgG4-RD is a fascinating clinical entity including a wide variety of diseases, formerly diagnosed as Mikulicz’s disease, autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis and retroperitoneal fibrosis.2 ,3 However, universal criteria for IgG4-RD have not yet been established at present, making its diagnosis in some patients ambiguous leading to many IgG4-RD mimickers.

A 3-year investigation by the Japanese IgG4 team, organised by the Ministry of Health, Labour and Welfare (MHLW) of Japan, has reached a consensus, in that IgG4-RD can occur in various organs, with clinical symptoms depending on lesion location. Characteristics common to all forms of IgG4-RD include elevated serum IgG4 concentration and tissue infiltration by IgG4-positive plasma cells, accompanied by tissue fibrosis and sclerosis.2 In 2011, the Japanese IgG4 team published comprehensive diagnostic (CD) criteria for IgG4-RD,4 with the major characteristics being serum IgG4 concentration >135 mg/dL, the infiltration of >10 IgG4+ cells …

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