Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on medication adherence

LIVE: Thursday 27th June 2024, 18:00-19:30 (CEST)
Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.
ADVANCED SEARCH
Log in
Top
Autoimmunity Highlights
Published in: Autoimmunity Highlights 1-2/2015

Open Access 01-08-2015 | Review Article

IgG4-related disease: case report and literature review

Authors: Abo-Helo Nizar, Elias Toubi

Published in: Autoimmunity Highlights | Issue 1-2/2015

Login to get access

Abstract

IgG4-related disease (IgG4-RD) is relatively a new growing entity of immune-mediated origin, characterized by a mass-forming lesion, the infiltration of IgG4-positive plasma cells and occasionally elevated serum IgG4. It is considered to be both a systemic inflammation and sclerosing disease. The most common manifestations are parotid and lacrimal swelling, lymphadenopathy and autoimmune pancreatitis. Sclerosing cholangitis and retroperitoneal fibrosis are among the other mentioned frequent manifestations. The diagnosis should be approved histo-pathologically but other conditions such as lymphoma should be carefully excluded. Patients with IgG4-RD respond beneficially to glucocorticoid therapy especially when given at early onset stages. In some cases, the combination of immunosuppressive agents is required.
Literature
1.
2.
Zen Y, Nakanuma Y (2010) IgG4-related disease: a cross-sectional study of 114 cases. Am J Surg Pathol 34:1812–1819CrossRefPubMed
3.
Nishimori I, Tamakoshi A, Otsuki M et al (2007) Prevalence of autoimmune pancreatitis in Japan from a nationwide survey in 2002. J Gastroenterol 42(Suppl 18):6–8CrossRefPubMed
4.
Aalberse RC, Stapel SO, Schuurman J et al (2009) Immunoglobulin G4: an odd antibody. Clin Exp Allergy 39:469–477CrossRefPubMed
5.
Nirula A, Glaser SM, Kalled SL et al (2011) What is IgG4? A review of the biology of a unique immunoglobulin subtype. Curr Opin Rheumatol 23:119–124CrossRefPubMed
6.
Canfield SM, Morrison SL (1991) The binding affinity of human IgG for its high affinity Fc receptor is determined by multiple amino acids in the CH2 domain and is modulated by the hinge region. J Exp Med 173:1483–1491CrossRefPubMed
7.
Huizinga TW, Roos D, von dem Borne AE et al (1990) Neutrophil Fc-gamma receptors: a two-way bridge in the immune system. Blood 75(6):1211–1214PubMed
8.
Van der Neut Kolfschoten M, Schuurman J, Losen M et al (2007) Anti-inflammatory activity of human IgG4 antibodies by dynamic Fab arm exchange. Science 317:1554–1557CrossRef
9.
Nouri-Aria Kayhan T, Petra A et al (2004) Grass pollen immunotherapy induces mucosal and peripheral IL-10 responses and blocking IgG activity. J Immunol 172(5):3252–3259CrossRefPubMed
10.
Cheuk W, Chan JK (2010) IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol 17:303CrossRefPubMed
11.
12.
Guarneri F, Guarneri C, Benvenga S et al (2005) Helicobacter pylori and autoimmune pancreatitis: role of carbonic anhydrase via molecular mimicry? J Cell Mol Med 9:741–744CrossRefPubMed
13.
Frulloni L, Lunardi C, Simone R et al (2009) Identification of a novel antibody associated with autoimmune pancreatitis. N Engl J Med 361:2135–2142CrossRefPubMed
14.
Kamisawa T, Anjiki H, Egawa N et al (2009) Allergic manifestations in autoimmune pancreatitis. Eur J Gastroenterol Hepatol 21:1136–1139CrossRefPubMed
15.
Miyoshi H, Uchida K, Taniguchi T et al (2008) Circulating naïve and CD4 + CD25high regulatory T cells in patients with autoimmune pancreatitis. Pancreas 36:133CrossRefPubMed
16.
Okazaki K, Uchida K, Koyabu M et al (2011) Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease. J Gastroenterol 46:277CrossRefPubMed
17.
Khosroshahi A, Stone JH (2011) A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol 23:57CrossRefPubMed
18.
Cheuk W, Yuen HK, Chu SY et al (2008) Lymphadenopathy of IgG4-related sclerosing disease. Am J Surg Pathol 32:671CrossRefPubMed
19.
Divatia M, Kim SA, Ro JY et al (2012) IgG4-related sclerosing disease, an emerging entity: a review of a multi-system disease. Yonsei Med J 53(1):15–34PubMedCentralCrossRefPubMed
20.
Hamano H, Arakura N, Muraki T et al (2006) Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis. J Gastroenterol 41:1197CrossRefPubMed
21.
Mehta M, Jakobiec F, Fay A et al (2009) Idiopathic fibroinflammatory disease of the face, eyelids, and periorbital membrane with immunoglobulin G4-positive plasma cells. Arch Pathol Lab Med 133:1251PubMed
22.
Zen Y, Onodera M, Inoue D et al (2009) Retroperitoneal fibrosis: a clinicopathologic study with respect to immunoglobulin G4. Am J Surg Pathol 33:1833CrossRefPubMed
23.
Stone JR (2011) Aortitis, periaortitis, and retroperitoneal fibrosis, as manifestations of IgG4-related systemic disease. Curr Opin Rheumatol 23:88CrossRefPubMed
24.
Zen Y, Sawazaki A, Miyayama S et al (2006) A case of retroperitoneal and mediastinal fibrosis exhibiting elevated levels of IgG4 in the absence of sclerosing pancreatitis (autoimmune pancreatitis). Hum Pathol 37:239CrossRefPubMed
25.
Stone JH, Khosroshahi A, Deshpande V et al (2010) IgG4-related systemic disease accounts for a significant proportion of thoracic lymphoplasmacytic aortitis cases. Arthritis Care Res (Hoboken) 62:316CrossRef
26.
Li Y, Nishihara E, Kakudo K et al (2011) Hashimoto’s thyroiditis: old concepts and new insights. Curr Opin Rheumatol 23:102CrossRefPubMed
27.
Zen Y, Kitagawa S, Minato H et al (2005) IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung. Hum Pathol 36:710CrossRefPubMed
28.
Murashima M, Tomaszewski J, Glickman JD et al (2007) Chronic tubulointerstitial nephritis presenting as multiple renal nodules and pancreatic insufficiency. Am J Kidney Dis 49:e7CrossRefPubMed
29.
Ikeda T, Oka M, Shimizu H et al (2013) IgG4-related skin manifestations in patients with IgG4-related disease. Eur J Dermatol 23:241PubMed
30.
31.
Uehara T, Hamano H, Kawa S et al (2010) Chronic gastritis in the setting of autoimmune pancreatitis. Am J Surg Pathol 34:1241CrossRefPubMed
32.
Cheuk W, Chan AC, Lam WL et al (2009) IgG4-related sclerosing mastitis: description of a new member of the IgG4-related sclerosing diseases. Am J Surg Pathol 33:1058CrossRefPubMed
33.
Sugimoto T, Morita Y, Isshiki K et al (2008) Constrictive pericarditis as an emerging manifestation of hyper-IgG4 disease. Int J Cardiol 130:e100CrossRefPubMed
34.
Takahashi N, Ghazale AH, Smyrk TC et al (2009) Possible association between IgG4-associated systemic disease with or without autoimmune pancreatitis and non-Hodgkin lymphoma. Pancreas 38:523CrossRefPubMed
35.
Shiokawa M, Kodama Y, Yoshimura K et al (2013) Risk of cancer in patients with autoimmune pancreatitis. Am J Gastroenterol 108:610CrossRefPubMed
36.
Sah RP, Chari ST (2011) Serologic issues in IgG4-related systemic disease and autoimmune pancreatitis. Curr Opin Rheumatol 23:108–113CrossRefPubMed
37.
Deshpande V, Zen Y, Chan JK et al (2012) Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25:1181CrossRefPubMed
38.
Sato Y, Notohara K, Kojima M et al (2010) IgG4-related disease: historical overview and pathology of hematological disorders. Pathol Int 60:247–258CrossRefPubMed
39.
40.
Kuo TT, Chen TC, Lee LY et al (2009) Sclerosing angiomatoid nodular transformation of the spleen (SANT): clinicopathological study of 10 cases with or without abdominal disseminated calcifying fibrous tumors, and the presence of a significant number of IgG4 + plasma cells. Pathol Int 59:844–850CrossRefPubMed
41.
Masaki Y, Dong L, Kurose N et al (2009) Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis 68:1310CrossRefPubMed
42.
Masaki Y, Sugai S, Umehara H et al (2010) IgG4-related diseases including Mikulicz’s disease and sclerosing pancreatitis: diagnostic insights. J Rheumatol 37:1380CrossRefPubMed
43.
Webster GJ, Pereira SP, Chapman RW et al (2009) Autoimmune pancreatitis/IgG4-associated cholangitis and primary sclerosing cholangitis–overlapping or separate diseases? J Hepatol 51:398CrossRefPubMed
44.
Nakazawa T, Ohara H, Sano H et al (2006) Schematic classification of sclerosing cholangitis with autoimmune pancreatitis by cholangiography. Pancreas 32:229CrossRefPubMed
45.
Tsushima K, Yokoyama T, Kawa S et al (2011) Elevated IgG4 levels in patients demonstrating sarcoidosis-like radiologic findings. Medicine (Baltimore) 90:194CrossRef
46.
Kamisawa T, Shimosegawa T, Okazaki K et al (2009) Standard steroid treatment for autoimmune pancreatitis. Gut 58:1504–1507CrossRefPubMed
47.
Kamisawa T, Okazaki K, Kawa S et al (2010) Japanese consensus guidelines for management of autoimmune pancreatitis. III. Treatment and prognosis of AIP. J Gastroenterol 45:471–477CrossRefPubMed
48.
Mollie N. Carruthers, John H. Stone, Vikram Deshpande et al (2012) Development of an IgG4-RD Responder Index. Inter J Rheumatol, Article ID 259408
49.
Khosroshahi A, Carruthers M, Deshpande V et al (2012) Rituximab for the treatment of IgG4-related disease: lessons from ten consecutive patients. Medicine (Baltimore) 91:57–66CrossRef
50.
Khosroshahi A, Bloch DB, Deshpande V et al (2010) Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease. Arthritis Rheum 62:1755–1762CrossRefPubMed
Metadata
Title
IgG4-related disease: case report and literature review
Authors
Abo-Helo Nizar
Elias Toubi
Publication date
01-08-2015
Publisher
Springer International Publishing
Published in
Autoimmunity Highlights / Issue 1-2/2015
Print ISSN: 2038-0305
Electronic ISSN: 2038-3274
DOI
https://doi.org/10.1007/s13317-015-0069-3
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Reserve your place

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

Read more

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine
Image Credits