Consensus statement on the pathology of IgG4-related disease

Vikram Deshpande, Yoh Zen, John K.C. Chan, Eunhee E. Yi, Yasuharu Sato, Tadashi Yoshino, Günter Klöppel, J. Godfrey Heathcote, Arezou Khosroshahi, Judith A. Ferry, Rob C. Aalberse, Donald B. Bloch, William R. Brugge, Adrian C. Bateman, Mollie N. Carruthers, Suresh T. Chari, Wah Cheuk, Lynn D. Cornell, Carlos Fernandez-Del Castillo, David G. ForcioneDaniel L. Hamilos, Terumi Kamisawa, Satomi Kasashima, Shigeyuki Kawa, Mitsuhiro Kawano, Gregory Y. Lauwers, Yasufumi Masaki, Yasuni Nakanuma, Kenji Notohara, Kazuichi Okazaki, Ji Kon Ryu, Takako Saeki, Dushyant V. Sahani, Thomas C. Smyrk, James R. Stone, Masayuki Takahira, George J. Webster, Motohisa Yamamoto, Giuseppe Zamboni, Hisanori Umehara, John H. Stone

Research output: Contribution to journalArticlepeer-review

1975 Citations (Scopus)


IgG4-related disease is a newly recognized fibro-inflammatory condition characterized by several features: a tendency to form tumefactive lesions in multiple sites; a characteristic histopathological appearance; andoften but not alwayselevated serum IgG4 concentrations. An international symposium on IgG4-related disease was held in Boston, MA, on 4-7 October 2011. The organizing committee comprising 35 IgG4-related disease experts from Japan, Korea, Hong Kong, the United Kingdom, Germany, Italy, Holland, Canada, and the United States, including the clinicians, pathologists, radiologists, and basic scientists. This group represents broad subspecialty expertise in pathology, rheumatology, gastroenterology, allergy, immunology, nephrology, pulmonary medicine, oncology, ophthalmology, and surgery. The histopathology of IgG4-related disease was a specific focus of the international symposium. The primary purpose of this statement is to provide practicing pathologists with a set of guidelines for the diagnosis of IgG4-related disease. The diagnosis of IgG4-related disease rests on the combined presence of the characteristic histopathological appearance and increased numbers of IgG4 plasma cells. The critical histopathological features are a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. We propose a terminology scheme for the diagnosis of IgG4-related disease that is based primarily on the morphological appearance on biopsy. Tissue IgG4 counts and IgG4:IgG ratios are secondary in importance. The guidelines proposed in this statement do not supplant careful clinicopathological correlation and sound clinical judgment. As the spectrum of this disease continues to expand, we advocate the use of strict criteria for accepting newly proposed entities or sites as components of the IgG4-related disease spectrum.

Original languageEnglish
Pages (from-to)1181-1192
Number of pages12
JournalModern Pathology
Issue number9
Publication statusPublished - Sept 2012


  • IgG4
  • IgG4-related disease
  • consensus statement

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


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