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Autoimmune Pancreatitis and IgG4-Related Disease: The Storiform Discovery to Treatment

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Abstract

Autoimmune pancreatitis (AIP) is an entity that has been recognized since 1961. Prior to the discovery of elevated serum IgG4 as a useful biomarker for its diagnosis, Dr. Yoshida in 1995 first described the entity of AIP, which in retrospect closely resembles the current concept of type 1 AIP. Since the discovery of IgG4 as a biomarker (the IgG4-era), a novel concept of IgG4-related disease (IgG4-RD) has been accepted as being comprised of two subtypes of AIP: type 1 defined as the pancreatic manifestation of IgG4-RD, and type 2 characterized by granulocytic epithelial lesions. The characteristic features of type 1 AIP are increased serum IgG4 levels, lymphoplasmacytic sclerosing pancreatitis (abundant infiltration of IgG4+ plasmocytes and lymphocytes, storiform fibrosis, and obliterative phlebitis), extrapancreatic manifestations of IgG4-RD (e.g., sclerosing cholangitis, sclerosing sialadenitis, retroperitoneal fibrosis), and steroid responsiveness. These entities can be differentiated from mimickers by a combination of serum IgG4 level, imaging features, and histopathological findings. The current first-line therapy is corticosteroids, or rituximab in high-risk patients with steroid intolerance. Although relapse rates are high, treatment of relapsed disease remains experimental.

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Fig. 1

Reproduced from Ref. [48, 51] with permission

Fig. 2

Reproduced from Ref. [1] with permission

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Reproduced from Ref. [1] with permission

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Reproduced from Ref. [1] with permission

Fig. 5

Reproduced from Ref. [1] with permission

Fig. 6

Reproduced from Ref. [26] with permission

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Abbreviations

AIP:

Autoimmune pancreatitis

ANA:

Anti-nuclear antibody

CDC:

Comprehensive diagnostic criteria

ERCP:

Endoscopic retrograde cholangiopancreatography

GEL:

Granulocytic epithelial lesion

ICDC:

International consensus of diagnostic criteria

IDCP:

Idiopathic duct-centric pancreatitis

IgG4-RD:

IgG4-related disease

JPS:

Japan Pancreas Society

LPSP:

Lymphoplasmacytic sclerosing pancreatitis

MD:

Mikulicz disease

MIF:

Multifocal idiopathic fibrosclerosis

MOLPS:

Multi-organ lymphoproliferative disease

MPD:

Main pancreatic duct

OOI:

Other organ involvement

PSC:

Primary sclerosing cholangitis

RF:

Rheumatoid factor

SIPS:

IgG4-systemic plasmacytic syndrome

SjS:

Sjögren’s syndrome

SLE:

Systemic lupus erythematosus

UNL:

Upper normal limit

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Acknowledgments

This study was partly supported by a Grant-in-Aid for Scientific Research from the Ministry of Culture and Science of Japan (23591017), and a Grant-in-Aid for “Research for Intractable Disease” Program from the Ministry of Health, Labor and Welfare of Japan.

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  1. Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, Hirakata, Osaka, 573-1197, Japan

    Kazuichi Okazaki

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Okazaki, K. Autoimmune Pancreatitis and IgG4-Related Disease: The Storiform Discovery to Treatment. Dig Dis Sci 64, 2385–2394 (2019). https://doi.org/10.1007/s10620-019-05746-9

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