, All statistical tests were performed using SPSS software (version 22), Statistical signifi

H. Hamano, S. Kawa, A. Horiuchi, H. Unno, N. Furuya et al., High serum IgG4 concentrations in patients with sclerosing pancreatitis, N Engl J Med, vol.344, issue.10, pp.732-740, 2001.

J. H. Stone, Y. Zen, and V. Deshpande, IgG4-related disease, N Engl J Med, vol.366, issue.6, pp.539-51, 2012.

H. Umehara, A new clinical entity: IgG4-related disease (IgG4-RD) discovered in the 21st century, Intern Med, vol.51, issue.8, p.22504232, 2012.

Y. Zen, T. Fujii, K. Harada, M. Kawano, K. Yamada et al., Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis, Hepatology, vol.45, issue.6, p.17518371, 2007.

K. Miyake, M. Moriyama, K. Aizawa, S. Nagano, Y. Inoue et al., Peripheral CD4+ T cells showing a Th2 phenotype in a patient with Mikulicz's disease associated with lymphadenopathy and pleural effusion, Mod Rheumatol, vol.18, issue.1, p.18094933, 2008.

K. Yamada, M. Kawano, R. Inoue, R. Hamano, Y. Kakuchi et al., Clonal relationship between infiltrating immunoglobulin G4 (IgG4)-positive plasma cells in lacrimal glands and circulating IgG4-positive lymphocytes in Mikulicz's disease, Clin Exp Immunol, vol.152, issue.3, p.2453205, 2008.

C. Meagher, Q. Tang, B. T. Fife, H. Bour-jordan, J. Wu et al., Spontaneous development of a pancreatic exocrine disease in CD28-deficient NOD mice, J Immunol, vol.180, issue.12, p.2429990, 2008.

C. S. Boomershine, A. Chamberlain, P. Kendall, A. R. Afshar-sharif, H. Huang et al., Autoimmune pancreatitis results from loss of TGFbeta signalling in S100A4-positive dendritic cells, Gut, vol.58, issue.9, p.2719085, 2009.

I. Haruta, N. Yanagisawa, S. Kawamura, T. Furukawa, K. Shimizu et al., A mouse model of autoimmune pancreatitis with salivary gland involvement triggered by innate immunity via persistent exposure to avirulent bacteria, Lab Invest, vol.90, issue.12, p.20733561, 2010.

G. M. Seleznik, T. Reding, F. Romrig, Y. Saito, A. Mildner et al., Lymphotoxin beta receptor signaling promotes development of autoimmune pancreatitis, Gastroenterology, vol.143, issue.5, p.22863765, 2012.

H. Kurisaki, Y. Nagao, S. Nagafuchi, and M. Mitsuyama, Autoimmune gastro-pancreatitis with anti-protein disulfide isomerase-associated 2 autoantibody in Aire-deficient BALB/cAnN mice, PLoS One, vol.8, issue.8, p.3753263, 2013.

T. L. Freitag, C. Cham, H. H. Sung, G. F. Beilhack, I. Durinovic-bello et al., Human risk allele HLA-DRB1*0405 predisposes class II transgenic Ab0 NOD mice to autoimmune pancreatitis, Gastroenterology, vol.139, issue.1, p.2902648, 2010.
DOI : 10.1053/j.gastro.2010.03.038

URL : http://europepmc.org/articles/pmc2902648?pdf=render

M. Koyabu, K. Uchida, Y. Sakaguchi, N. Fukata, T. Kusuda et al., Possible Involvement of Foxp3(+) Regulatory T Cells in the Development of Immune-Mediated Pancreatitis in MRL/Mp Mice Treated with Polyinosinic:Polycytidylic Acid, Int J Rheumatol, p.3678441, 2013.

M. Shiokawa, Y. Kodama, K. Kuriyama, K. Yoshimura, T. Tomono et al., Pathogenicity of IgG in patients with IgG4-related disease, Gut, vol.65, issue.8, p.26964842, 2016.

R. Roncagalli, M. Mingueneau, C. Gregoire, M. Malissen, and B. Malissen, LAT signaling pathology: an "autoimmune" condition without T cell self-reactivity, Trends Immunol, vol.31, issue.7, p.20542732, 2010.
DOI : 10.1016/j.it.2010.05.001

URL : https://hal.archives-ouvertes.fr/hal-00605842

E. Aguado, S. Richelme, S. Nunez-cruz, A. Miazek, A. M. Mura et al., Induction of T helper type 2 immunity by a point mutation in the LAT adaptor, Science, vol.296, issue.5575, p.12065839, 2002.

C. Genton, Y. Wang, S. Izui, B. Malissen, G. Delsol et al., The Th2 lymphoproliferation developing in LatY136F mutant mice triggers polyclonal B cell activation and systemic autoimmunity, J Immunol, vol.177, issue.4, p.16887989, 2006.
URL : https://hal.archives-ouvertes.fr/hal-00165594

S. Koonpaew, S. Shen, L. Flowers, and W. Zhang, LAT-mediated signaling in CD4+CD25+ regulatory T cell development, J Exp Med, vol.203, issue.1, p.2118069, 2006.
DOI : 10.1084/jem.20050903

URL : http://jem.rupress.org/content/203/1/119.full.pdf

J. Mestas and C. C. Hughes, Of mice and not men: differences between mouse and human immunology, J Immunol, vol.172, issue.5, p.14978070, 2004.
DOI : 10.4049/jimmunol.172.5.2731

URL : http://www.jimmunol.org/content/172/5/2731.full.pdf

K. Yamada, M. Yamamoto, T. Saeki, I. Mizushima, S. Matsui et al., New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases, Arthritis Res Ther, vol.19, issue.1, p.5709928, 2017.

Z. S. Wallace, V. Deshpande, H. Mattoo, V. S. Mahajan, M. Kulikova et al., IgG4-Related Disease: Clinical and Laboratory Features in One Hundred Twenty-Five Patients, Arthritis Rheumatol, vol.67, issue.9, p.4621270, 2015.
DOI : 10.1002/art.39205

URL : https://onlinelibrary.wiley.com/doi/pdf/10.1002/art.39205

A. Fernandez-codina, F. Martinez-valle, B. Pinilla, C. Lopez, I. Detorres et al., IgG4-Related Disease: Results From a Multicenter Spanish Registry, Medicine (Baltimore), vol.94, issue.32, p.4616706, 2015.

C. Campochiaro, G. A. Ramirez, E. P. Bozzolo, M. Lanzillotta, A. Berti et al., IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients, Scand J Rheumatol, vol.45, issue.2, p.26398142, 2016.

M. Ebbo, L. Daniel, M. Pavic, P. Seve, M. Hamidou et al., IgG4-related systemic disease: features and treatment response in a French cohort: results of a multicenter registry, Medicine (Baltimore), vol.91, issue.1, p.22198501, 2012.

D. Inoue, K. Yoshida, N. Yoneda, K. Ozaki, T. Matsubara et al., IgG4-related disease: dataset of 235 consecutive patients, Medicine (Baltimore), vol.94, issue.15, p.4602507, 2015.

A. Tanaka, M. Moriyama, H. Nakashima, K. Miyake, J. N. Hayashida et al., Th2 and regulatory immune reactions contribute to IgG4 production and the initiation of Mikulicz disease, Arthritis Rheum, vol.64, issue.1, p.21898360, 2012.

S. Hara, M. Kawano, I. Mizushima, K. Harada, T. Takata et al., Distribution and components of interstitial inflammation and fibrosis in IgG4-related kidney disease: analysis of autopsy specimens, Hum Pathol, vol.55, p.27246178, 2016.

V. Deshpande, Y. Zen, J. K. Chan, E. E. Yi, Y. Sato et al., Consensus statement on the pathology of IgG4-related disease, Mod Pathol, vol.25, issue.9, p.22596100, 2012.

T. Saeki, M. Kawano, I. Mizushima, M. Yamamoto, Y. Wada et al., The clinical course of patients with IgG4-related kidney disease, Kidney Int, vol.84, issue.4, p.23698232, 2013.

I. Mizushima, M. Yamamoto, D. Inoue, S. Nishi, Y. Taniguchi et al., Factors related to renal cortical atrophy development after glucocorticoid therapy in IgG4-related kidney disease: a retrospective multicenter study, Arthritis Res Ther, vol.18, issue.1, p.5123425, 2016.

M. C. Chang, J. I. Liang, P. C. Jeng, Y. M. Yang, C. Y. Tien et al., Human cationic trypsinogen but not serine peptidase inhibitor, Kazal type 1 variants increase the risk of type 1 autoimmune pancreatitis, J Gastroenterol Hepatol, vol.29, issue.12, p.24909264, 2014.

F. Gao, Y. M. Li, G. L. Hong, Z. F. Xu, Q. C. Liu et al., Leu81Met mutation results in autoimmune pancreatitis, World J Gastroenterol, vol.19, issue.21, p.3671086, 2013.

M. C. Chang, J. I. Liang, P. C. Jeng, Y. M. Yang, C. Y. Tien et al., Cystic fibrosis transmembrane conductance regulator gene variants are associated with autoimmune pancreatitis and slow response to steroid treatment, J Cyst Fibros, vol.14, issue.5, p.25869325, 2015.

Q. C. Liu, F. Chen, C. Y. Wu, F. Gao, Z. H. Zhuang et al., CALCB splice region pathogenic variants leading to plasma cell neurotropic enrichment in type 1 autoimmune pancreatitis, Cell Death Dis, 2017.

, , vol.8, p.5386480

M. C. Chang, Y. T. Chang, Y. W. Tien, P. C. Liang, J. I. Wei et al., T-cell regulatory gene CTLA-4 polymorphism/haplotype association with autoimmune pancreatitis, Clin Chem, vol.53, issue.9, p.17712006, 2007.

Y. Kinugawa, T. Uehara, K. Sano, K. Matsuda, Y. Maruyama et al., Methylation of Tumor Suppressor Genes in Autoimmune Pancreatitis, Pancreas, vol.46, issue.5, p.28196014, 2017.