Some Recollections of the History of Research on IgG4-RD

Establishment of the Concept of Lymphoplasmacytic Sclerosing Pancreatitis (LPSP)
  • Kenji Kawaguchi


More than 20 years have passed since a paper describing two cases of pancreatitis associated with cholangitis and showing a distinct clinicopathological picture of lymphoplasmacytic sclerosing pancreatitis (LPSP) was brought to the medical world’s attention. That paper also speculated about the possibility of an association with systemic disease, now recognized as “IgG4-related disease.” In this chapter, detailed clinicopathological features of these two cases as initially described are outlined to explain how the concept of LPSP was established.


Bile Duct Primary Sclerosing Cholangitis Obstructive Jaundice Autoimmune Pancreatitis Serum IgG4 Concentration 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Kawaguchi K, Koike M, Tsuruta K, et al. Lymphoplasmacytic sclerosing pancreatitis with cholangitis: a variant of primary sclerosing cholangitis extensively involving pancreas. Hum Pathol. 1991;22:387–95.PubMedCrossRefGoogle Scholar
  2. 2.
    Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344:732–8.PubMedCrossRefGoogle Scholar
  3. 3.
    Scully RE, Mark EJ, McNeely BU. Case records of the Massachusetts General Hospital (Case 6–1982). N Engl J Med. 1982;306:349–58.CrossRefGoogle Scholar
  4. 4.
    Uehara T, Hamano H, Kawakami M, et al. Autoimmune pancreatitis-associated prostatitis: distinct clinicopathological entity. Pathol Int. 2008;58:118–25.PubMedCrossRefGoogle Scholar
  5. 5.
    Tanaka A, Fukayama M, Funata N, et al. Coronary arteritis and aortoarteritis in the elderly males. Virchows Archiv A Pathol Anat. 1988;414:9–14.CrossRefGoogle Scholar
  6. 6.
    Hamano H, Kawa S, Ochi Y, et al. Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet. 2002;359:1403–4.PubMedCrossRefGoogle Scholar
  7. 7.
    Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982–4.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Japan 2014

Authors and Affiliations

  1. 1.Department of PathologyShinonoi General HospitalNagano CityJapan

Personalised recommendations