IgG4-related disease: case report and literature review

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

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.

Related collections

Most cited references 45

Record: found
Abstract: found
Article: not found

Immunoglobulin G4: an odd antibody.

R Aalberse, S Stapel, J Schuurman … (2009)

Despite its well-known association with IgE-mediated allergy, IgG4 antibodies still have several poorly understood characteristics. IgG4 is a very dynamic antibody: the antibody is involved in a continuous process of half-molecules (i.e. a heavy and attached light-chain) exchange. This process, also referred to as 'Fab-arm exchange', results usually in asymmetric antibodies with two different antigen-combining sites. While these antibodies are hetero- bivalent, they will behave as monovalent antibodies in most situations. Another aspect of IgG4, still poorly understood, is its tendency to mimic IgG rheumatoid factor (RF) activity by interacting with IgG on a solid support. In contrast to conventional RF, which binds via its variable domains, the activity of IgG4 is located in its constant domains. This is potentially a source of false positives in IgG4 antibody assay results. Because regulation of IgG4 production is dependent on help by T-helper type 2 (Th2) cells, the IgG4 response is largely restricted to non-microbial antigens. This Th2-dependency associates the IgG4 and IgE responses. Another typical feature in the immune regulation of IgG4 is its tendency to appear only after prolonged immunization. In the context of IgE-mediated allergy, the appearance of IgG4 antibodies is usually associated with a decrease in symptoms. This is likely to be due, at least in part, to an allergen-blocking effect at the mast cell level and/or at the level of the antigen-presenting cell (preventing IgE-facilitated activation of T cells). In addition, the favourable association reflects the enhanced production of IL-10 and other anti-inflammatory cytokines, which drive the production of IgG4. While in general, IgG4 is being associated with non-activating characteristics, in some situations IgG4 antibodies have an association with pathology. Two striking examples are pemphigoid diseases and sclerosing diseases such as autoimmune pancreatitis. The mechanistic basis for the association of IgG4 with these diseases is still enigmatic. However, the association with sclerosing diseases may reflect an excessive production of anti-inflammatory cytokines triggering an overwhelming expansion of IgG4-producing plasma cells. The bottom line for allergy diagnosis: IgG4 by itself is unlikely to be a cause of allergic symptoms. In general, the presence of allergen-specific IgG4 indicates that anti-inflammatory, tolerance-inducing mechanisms have been activated. The existence of the IgG4 subclass, its up-regulation by anti-inflammatory factors and its own anti-inflammatory characteristics may help the immune system to dampen inappropriate inflammatory reactions.

0 comments Cited 266 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Standard steroid treatment for autoimmune pancreatitis.

T Kamisawa, T. Shimosegawa, K Okazaki … (2009)

To establish an appropriate steroid treatment regimen for autoimmune pancreatitis (AIP). A retrospective survey of AIP treatment was conducted in 17 centres in Japan. The main outcome measures were rate of remission and relapse. Of 563 patients with AIP, 459 (82%) received steroid treatment. The remission rate of steroid-treated AIP was 98%, which was significantly higher than that of patients without steroid treatment (74%, 77/104; p<0.001). Steroid treatment was given for obstructive jaundice (60%), abdominal pain (11%), associated extrapancreatic lesions except the biliary duct (11%), and diffuse enlargement of the pancreas (10%). There was no relationship between the period necessary to achieve remission and the initial dose (30 mg/day vs 40 mg/day) of prednisolone. Maintenance steroid treatment was given in 377 (82%) of 459 steroid-treated patients, and steroid treatment was stopped in 104 patients. The relapse rate of patients with AIP on maintenance treatment was 23% (63/273), which was significantly lower than that of patients who stopped maintenance treatment (34%, 35/104; p = 0.048). From the start of steroid treatment, 56% (55/99) relapsed within 1 year and 92% (91/99) relapsed within 3 years. Of the 89 relapsed patients, 83 (93%) received steroid re-treatment, and steroid re-treatment was effective in 97% of them. The major indication for steroid treatment in AIP is the presence of symptoms. An initial prednisolone dose of 0.6 mg/kg/day, is recommend, which is then reduced to a maintenance dose over a period of 3-6 months. Maintenance treatment with low-dose steroid reduces but dose not eliminate relapses.

0 comments Cited 178 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

IgG4-related disease: a cross-sectional study of 114 cases.

Yoh Zen, Yasuni Nakanuma (2010)

IgG4-related disease has been identified in various organs, but whether or not there are organ-specific characteristics related to the etiologic factors is still unknown. Here, we carried out a cross-sectional study of 114 patients with IgG4-related disease. On the basis of the location of the lesions, the patients were classified into 5 groups: head and neck (n=23), thoracic (n=16), hepatic and pancreatobiliary (n=27), retroperitoneal (n=13), and systemic (n=35). All groups had similar clinicopathologic features in various aspects. However, there were some organ-specific features: for example, the proportion of the female patients was significantly higher in the head and neck group, serum IgG4 concentrations were significantly higher in the head/neck and systemic groups, and all kidney lesions were associated with extrarenal disease. Unique pathologic features were dense fibrosis in dacryoadenitis, numerous lymph follicles in sialadenitis and dacryoadenitis, and obliterative arteritis in lung lesions. In addition, an epithelioid granuloma and rheumatoid nodule were noted within IgG4-related lesions in 2 patients, 1 each with a history of tuberculosis and rheumatoid arthritis, respectively. Malignant tumors (2 lung cancers and 1 malignant lymphoma) were identified after the diagnosis of IgG4-related disease in 3 patients, all in the systemic group. In conclusion, this study showed organ-specific features of IgG4-related disease. Further study is necessary to conclude whether these features reflect different manifestations of a single disease entity or suggest different underlying etiologic factors.

0 comments Cited 147 times – based on 0 reviews      Review now

Bookmark

All references

Author and article information

Contributors

Elias Toubi: elias.toubi@b-zion.org.il

Journal

Journal ID (nlm-ta): Auto Immun Highlights

Journal ID (iso-abbrev): Auto Immun Highlights

Title: Auto-Immunity Highlights

Publisher: Springer International Publishing (Cham )

ISSN (Print): 2038-0305

ISSN (Electronic): 2038-3274

Publication date (Electronic): 28 July 2015

Publication date Collection: August 2015

Volume: 6

Issue: 1-2

Pages: 7-15

Affiliations

Division of Allergy and Clinical Immunology, Faculty of Medicine, Bnai Zion Medical Center, Technion-Israel Institute of Technology, Haifa, Israel

Article

Publisher ID: 69

DOI: 10.1007/s13317-015-0069-3

PMC ID: 4536235

PubMed ID: 26216362

SO-VID: 7a1d99f9-8b05-4761-8a69-9c2459713fc5

History

Date received : 6 July 2015

Date accepted : 7 July 2015

Custom metadata

Keywords: igg4 + plasma cells,autoimmunity,immunoglobulins,autoimmune pancreatitis,sialadenitis,retroperitoneal fibrosis

Data availability:

Keywords: igg4 + plasma cells, autoimmunity, immunoglobulins, autoimmune pancreatitis, sialadenitis, retroperitoneal fibrosis

Comments

Comment on this article

scite_

Smart Citations

Citing PublicationsSupportingMentioningContrasting

View Citations

See how this article has been cited at scite.ai

scite shows how a scientific paper has been cited by providing the context of the citation, a classification describing whether it supports, mentions, or contrasts the cited claim, and a label indicating in which section the citation was made.