Background:IgG4-related disease (IgG4-RD) is an autoimmune disorder and frequently involve multiple organs. The respiratory tract is one of the most frequently involved sites.Objectives:This study aimed to compare the demographic and clinical characteristics of IgG4-related respiratory disease (IgG4-RRD) and non-IgG4-RRD patients in a large cohort.Methods:We carried out a retrospective study of 452 cases of IgG4-RD (104 IgG4-RRD patients and 348 non-IgG4-RRD patients) diagnosed at Peking University People’s Hospital between 2003 and 2020.Results:IgG4-RRD patients had an elder age of disease onset and diagnosis. Multiorgan involvement and hypocomplementemia were more common in IgG4-RRD. Besides, the level of ESR, eosinophilia, IgG and IgG4 were higher in IgG4-RRD patients. In IgG4-RRD group, salivary gland, lacrimal gland, lymph nodes, biliary system and kidney were more commonly involved than those in the non-IgG4-RRD group. Also, more numbers of organ involvement and biliary involvement were independent risk factors for the development of respiratory involvement in IgG4-RD patients.Conclusion:Our study revealed demographic, clinical, laboratory and imaging features of IgG4-RRD patients and the underlying differences in pathogenesis between the two phenotypes, which have important implications for the diagnosis and treatment of the disease.References:[1]Morales AT, Cignarella AG, Jabeen IS, Barkin JS, Mirsaeidi M. An update on IgG4-related lung disease. European journal of internal medicine. 2019;66:18-24.[2]Stone JH, Zen Y, Deshpande V. IgG4-related disease. The New England journal of medicine. 2012;366(6):539-51.[3]Vasaitis L. IgG4-related disease: A relatively new concept for clinicians. European journal of internal medicine. 2016;27:1-9.[4]Matsui S, Yamamoto H, Minamoto S, Waseda Y, Mishima M, Kubo K. Proposed diagnostic criteria for IgG4-related respiratory disease. Respiratory investigation. 2016;54(2):130-2.[5]Cao L, Chen YB, Zhao DH, Shi WF, Meng S, Xie LX. Pulmonary function tests findings and their diagnostic value in patients with IgG4-related disease. Journal of thoracic disease. 2017;9(3):547-54.[6]Wallace ZS, Perugino C, Matza M, Deshpande V, Sharma A, Stone JH. Immunoglobulin G4-related Disease. Clinics in chest medicine. 2019;40(3):583-97.[7]Matsui S. IgG4-related respiratory disease. Modern rheumatology. 2019;29(2):251-6.[8]Johansson SG, Hourihane JO, Bousquet J, Bruijnzeel-Koomen C, Dreborg S, Haahtela T, et al. A revised nomenclature for allergy. An EAACI position statement from the EAACI nomenclature task force. Allergy. 2001;56(9):813-24.[9]Fei Y, Shi J, Lin W, Chen Y, Feng R, Wu Q, et al. Intrathoracic Involvements of Immunoglobulin G4-Related Sclerosing Disease. Medicine. 2015;94(50):e2150.[10]Wallace ZS, Deshpande V, Mattoo H, Mahajan VS, Kulikova M, Pillai S, et al. IgG4-Related Disease: Clinical and Laboratory Features in One Hundred Twenty-Five Patients. Arthritis & rheumatology (Hoboken, NJ). 2015;67(9):2466-75.[11]Yamada K, Yamamoto M, Saeki T, Mizushima I, Matsui S, Fujisawa Y, 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 research & therapy. 2017;19(1):262.[12]Borges T, Silva S. IgG4-related disease: How to place it in the spectrum of immune-mediated and rheumatologic disorders? Modern rheumatology. 2020;30(4):609-16.[13]Liu Y, Xue M, Wang Z, Zeng Q, Ren L, Zhang Y, et al. Salivary gland involvement disparities in clinical characteristics of IgG4-related disease: a retrospective study of 428 patients. Rheumatology (Oxford, England). 2020;59(3):634-40.[14]Matsui S, Taki H, Shinoda K, Suzuki K, Hayashi R, Tobe K, et al. Respiratory involvement in IgG4-related Mikulicz’s disease. Modern rheumatology. 2012;22(1):31-9.Disclosure of Interests:None declared
Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan