ERN ReCONNET

Disease Info

Disease: IgG4-related disease

IgG4-related disease (IgG4-RDs)

IgG4-related diseases (IgG4-RD) are a group of chronic relapsing remitting inflammatory conditions characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells and subsequent tissue fibrosis. Frequently, tissue-destructive lesions may develop resulting from deposition of connective tissue in affected sites, even with organ failure.

IgG4-RD is often an indolent condition. People are generally well at the time of the diagnosis. Pain is generally a consequence of the obstruction or compression due to the presence of mass lesions and their complications. Multiorgan involvement can be present at the same time (synchronously) or different sites can be affected at different periods (metachronously).

Whatever organ or tissue are involved, a lymphoplasmacytic infiltrate rich in IgG4-positive plasma-cells, storiform fibrosis and obliterative phlebitis are the hystopathological hallmarks. The clinical suspicion may arise in the presence of elevated plasma IgG4 levels but the diagnosis often relies on histologic confirmation, as IgG4 levels may be normal.

To prevent fibrosis progression and organ destruction an induction and maintenance of remission is necessary. The first-line recommended agent are steroids. However, with taperig of steroids relapses are frequent, so steroid-sparing immunosuppressive agents can be considered. Finally, surgery or radiotherapy may be helpful in case of organ damage.

It is with a great pleasure that ERN ReCONNET invites you to register at the 5th International Symposium on IgG4-Related Disease that will be held for the first time in Europe from April 4th to April 6th 2024 at the Star Rosa Grand Hotel (Milan, Italy).

ERN ReCONNET has endorsed this important international meeting chaired by our IgG4-related disease Working Group Coordinator Prof. Della Torre, from Vita-Salute San Raffaele University (Milan, Italy). In addition, experts from our Network will be invited as speakers and moderators of this unique event focused on IgG4-related disease.

Deadline for registration is March 1st 2024.

All the information and links can be found here.

ERN ReCONNET publications are available for consultation.

Tobias Alexander
Disease Coordinator

Ana Vieira
ePAG representative
(ad interim)

ERN RECONNET – ENDORSED EVENT: THE 5TH INTERNATIONAL SYMPOSIUM ON IGG4 – RELATED DISEASE (EN)

Emanuel Della Torre
Disease Coordinator

WEBINARS

IgG4 related disease: a patient experience. Speakers: Mr Patsalias and Mr Ballarin.

IgG4 related disease a challenge in the diagnosis.

ERN RECONNET – ENDORSED EVENT: THE 5TH INTERNATIONAL SYMPOSIUM ON IGG4 – RELATED DISEASE (IT)

LIST OF USEFUL REFERENCES

1. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease
Khosroshahi A, Wallace ZS, Crowe JL, Akamizu T, Azumi A, Carruthers MN, et al.
Arthritis Rheumatol. 2015 Jul;67(7): 1688-99.

2. Incidental neuroendocrine tumors of the pancreas: MDCT findings and features of malignancy
Gallotti A, Johnston RP, Bonaffini PA, Ingkakul T, Deshpande V, Fernández-del Castillo C, Sahani DV
AJR Am J Roentgenol. 2013 Feb;200(2):355-62.

3. Consensus statement on the pathology of IgG4-related disease
Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al.
Mod Pathol. 2012 Sep;25(9):1181-92.

4. Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations
Stone JH, Khosroshahi A, Deshpande V, Chan JK, Heathcote JG, Aalberse R, Stone JH, al.
Arthritis Rheum. 2012 Oct;64(10):3061-7.

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