Gastrointestinal and Extra-Intestinal Manifestations of IgG4–Related Disease

Katsuyuki Miyabe, Yoh Zen, Lynn D. Cornell, Govindarajan Rajagopalan, Vaidehi R. Chowdhary, Lewis Rowland Roberts, Suresh T Chari

Research output: Contribution to journalReview article

5 Citations (Scopus)

Abstract

IgG4-related disease (IgG4-RD) is a chronic relapsing multi-organ fibro-inflammatory syndrome of presumed autoimmune etiology. It is characterized by increased serum levels of IgG4 and tissue infiltration by IgG4+ cells. Increased titers of autoantibodies against a spectrum of self-antigens and response to steroids have led to its characterization as an autoimmune disease. However, the pathognomonic antigens probably differ among manifestations, and different antigens or autoantibodies produce similar immune reactions in different organs. Little is known about the pathogenic effects, if any, of serum IgG4 or IgG4+ plasma cells in tissues. Despite several animal models of the disease, none truly recapitulates human IgG4-RD. Histologic analyses of tissues from patients with IgG4-RD reveal a dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis, although these features vary among organs. Typical presentation and imaging findings include mass-forming synchronous or metachronous lesions in almost any organ, but most commonly in the pancreas, bile duct, retroperitoneum, kidneys, lungs, salivary and lacrimal glands, orbit, and lymph nodes. In all organs, inflammation can be reduced by corticosteroids and drugs that deplete B cells, such as rituximab. Patients with IgG4-RD have relapses that respond to primary therapy. Intense fibrosis accompanies the inflammatory response, leading to permanent organ damage and insufficiency. Death from IgG4-RD is rare. IgG4-RD is a multi-organ disease with predominant pancreatico-biliary involvement. Despite its relapsing–remitting course, patients have an excellent prognosis.

Original languageEnglish (US)
Pages (from-to)990-1003.e1
JournalGastroenterology
Volume155
Issue number4
DOIs
StatePublished - Oct 1 2018

Fingerprint

Immunoglobulin G
Plasma Cells
Autoantibodies
Fibrosis
Animal Disease Models
Antigens
Phlebitis
Lacrimal Apparatus
Autoantigens
Orbit
Rare Diseases
Bile Ducts
Salivary Glands
Serum
Autoimmune Diseases
Pancreas
Adrenal Cortex Hormones
B-Lymphocytes
Lymph Nodes
Steroids

Keywords

  • Autoimmune Pancreatitis
  • Plasmablast
  • Retroperitoneal Fibrosis
  • Sialadenitis

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

Cite this

Miyabe, K., Zen, Y., Cornell, L. D., Rajagopalan, G., Chowdhary, V. R., Roberts, L. R., & Chari, S. T. (2018). Gastrointestinal and Extra-Intestinal Manifestations of IgG4–Related Disease. Gastroenterology, 155(4), 990-1003.e1. https://doi.org/10.1053/j.gastro.2018.06.082

Gastrointestinal and Extra-Intestinal Manifestations of IgG4–Related Disease. / Miyabe, Katsuyuki; Zen, Yoh; Cornell, Lynn D.; Rajagopalan, Govindarajan; Chowdhary, Vaidehi R.; Roberts, Lewis Rowland; Chari, Suresh T.

In: Gastroenterology, Vol. 155, No. 4, 01.10.2018, p. 990-1003.e1.

Research output: Contribution to journalReview article

Miyabe, K, Zen, Y, Cornell, LD, Rajagopalan, G, Chowdhary, VR, Roberts, LR & Chari, ST 2018, 'Gastrointestinal and Extra-Intestinal Manifestations of IgG4–Related Disease', Gastroenterology, vol. 155, no. 4, pp. 990-1003.e1. https://doi.org/10.1053/j.gastro.2018.06.082
Miyabe, Katsuyuki ; Zen, Yoh ; Cornell, Lynn D. ; Rajagopalan, Govindarajan ; Chowdhary, Vaidehi R. ; Roberts, Lewis Rowland ; Chari, Suresh T. / Gastrointestinal and Extra-Intestinal Manifestations of IgG4–Related Disease. In: Gastroenterology. 2018 ; Vol. 155, No. 4. pp. 990-1003.e1.
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