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Current Concept of IgG4-Related Disease

Chapter
First Online:
Part of the Current Topics in Microbiology and Immunology book series (CT MICROBIOLOGY, volume 401)

Abstract

IgG4-related disease (IgG4-RD) is a fibroinflammatory disease of unknown etiology, which is characterized by a tendency to form tumefactive lesions, increased serum levels of IgG4, and massive infiltration of IgG4-positive plasma cells with storiform fibrosis and/or obliterative phlebitis. Patients with IgG4-RD have frequently multiorgan involvements such as the pancreas, biliary tree, salivary glands, periorbital tissues, kidneys, lungs, lymph nodes, and retroperitoneum. IgG4-RD mainly affects middle-aged to elderly men except for involvement in lachrymal and salivary glands, so-called Mikulicz’s disease. The clinical manifestations of IgG4-RD depend on individually involved organs and respond well to steroid, but the prognosis still remains unclear. Some patients develop serious complications such as obstructive jaundice due to hepatic, gallbladder, or pancreatic lesions; hydronephrosis due to retroperitoneal fibrosis; or respiratory symptoms due to pulmonary lesions. Nomenclatures of individual organ manifestation of IgG4-RD have been internationally consented.

Autoimmune pancreatitis IgG4-related disease Disease concept 

Abbreviations

AIP

Autoimmune pancreatitis

CDC

Comprehensive diagnostic criteria

GEL

Granulocytic epithelial lesion

ICDC

International consensus diagnostic criteria

IDCP

Idiopathic duct-centric pancreatitis

IgG4-RD

IgG4-related disease

IgG4-SC

IgG4-related sclerosing cholangitis

IPC

The international pathologic consensus criteria

JPS

Japan Pancreas Society

LPSP

Lymphoplasmacytic sclerosing pancreatitis

MD

Mikulicz’s disease

MOLPS

Multiorgan lymphoproliferative disease

OOI

Other organ involvement

SjS

SJÖGREN’S syndrome

PSC

Primary sclerosing cholangitis

RF

Rheumatoid factor

SIPS

Systemic IgG4-related plasmacytic syndrome

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Notes

Acknowledgment

This study was partially supported by (1) Grant-in-Aid for Scientific Research (C) of the Ministry of Culture and Science of Japan (20590810, 24591020, 12008507), (2) the Research Program on Intractable Diseases, from the Ministry of Labor and Welfare of Japan, and (3) grants-in-aid from the Ministry of Education, Culture, Sports, Science and Technology of Japan, from CREST Japan Science, and Technology Agency.

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© Springer International Publishing Switzerland 2016

Authors and Affiliations

  1. 1.Department of Gastroenterology and HepatologyKansai Medical UniversityOsakaJapan
  2. 2.The Third Department of Internal Medicine, Division of Gastroenterology and HepatologyKansai Medical UniversityShinmachi, HirakataJapan
  3. 3.Northern County Center for RA and Autoimmune DiseaseHayashi HospitalFukuiJapan

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