{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,6]],"date-time":"2026-11-06T09:20:39Z","timestamp":1793956839022,"version":"4.8.0"},"reference-count":21,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,12,1]],"date-time":"2021-12-01T00:00:00Z","timestamp":1638316800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,12,15]],"date-time":"2021-12-15T00:00:00Z","timestamp":1639526400000},"content-version":"vor","delay-in-days":14,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Pulm Med"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Immunoglobulin G4-related lung disease (IgG4-RLD) is a rare entity. We retrospectively analyzed the clinical and histopathological\u00a0characteristics of patients with pathologically confirmed IgG4-RLD to improve the diagnosis rate and reduce the risk of misdiagnosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We screened the pathological reports of 4838 patients with pulmonary surgery and\/or biopsy specimens from April 2017 to April 2021 at Sun Yat-Sen Memorial Hospital affiliated with Sun Yat-Sen University, and specimens from 65 patients with suspected IgG4-RLD were subjected to immunohistochemical staining for IgG4 and IgG. Finally, 10 patients with definite IgG4-RLD that was pathologically confirmed were enrolled and analyzed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The incidence of pathologically confirmed IgG4-RLD was 0.2% (10\/4838). The ten patients had an average age of 59.7\u00a0years at diagnosis, and the male-to-female ratio was 9:1. The initial\u00a0clinical\u00a0manifestations were nonspecific, and cough was the most common symptom (4\/10). More than one organ was involved in most patients (8\/10), and mediastinal\/hilar lymph node involvement was often observed (7\/10). Serum IgG4 was analyzed in 6 patients and found to be elevated. Serum tumor marker levels were within the normal range or were slightly elevated. Computed tomography (CT) of the chest and\/or\n                      <jats:sup>18<\/jats:sup>\n                      F-fluorodeoxyglucose positron\u00a0emission\u00a0tomography-computed\u00a0tomography (\n                      <jats:sup>18<\/jats:sup>\n                      F-FDG PET-CT) imaging revealed that 5 patients had a mixed type, 3 patients had the solid nodular type, and 2 patients had the bronchovascular type. All pulmonary masses and large nodules with solid patterns had spiculated margins and inhomogeneous enhancement with or without pleural\u00a0indentation and a lobulated\u00a0appearance. Abundant lymphoplasmacytic cell infiltration and fibrosis were observed in all patients. The expression of IgG4 and IgG was upregulated in the pulmonary sections. Seven patients were treated with glucocorticoids with or without additional immunosuppressants and responded well.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The results of our study suggest that multiple imaging findings, an elevated serum IgG4 concentration, and no significant increase in serum tumor biomarkers could provide diagnostic support for IgG4-RLD, especially for isolated IgG4-RLD or IgG4-RLD that includes other organ involvement that does not aid in establishing the diagnosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s12890-021-01781-3","type":"journal-article","created":{"date-parts":[[2021,12,15]],"date-time":"2021-12-15T07:02:54Z","timestamp":1639551774000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Clinicopathological characteristics of IgG4-related lung disease"],"prefix":"10.1186","volume":"21","author":[{"given":"Jia","family":"Liu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuxiang","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ximing","family":"Shen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhanghai","family":"He","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tingfeng","family":"Yu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Pang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoyan","family":"Jin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lingyun","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,12,15]]},"reference":[{"key":"1781_CR1","doi-asserted-by":"publisher","first-page":"315","DOI":"10.1146\/annurev-pathol-012513-104708","volume":"9","author":"VS Mahajan","year":"2014","unstructured":"Mahajan VS, Mattoo H, Deshpande V, Pillai SS, Stone JH. IgG4-related disease. Annu Rev Pathol. 2014;9:315\u201347.","journal-title":"Annu Rev Pathol"},{"issue":"3","key":"1781_CR2","doi-asserted-by":"publisher","first-page":"406","DOI":"10.1136\/annrheumdis-2018-214603","volume":"78","author":"ZS Wallace","year":"2019","unstructured":"Wallace ZS, Zhang Y, Perugino CA, Naden R, Choi HK, Stone JH. ACR\/EULAR IgG4-RD Classification Criteria Committee Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts. Ann Rheum Dis. 2019;78(3):406\u201312.","journal-title":"Ann Rheum Dis"},{"issue":"9","key":"1781_CR3","doi-asserted-by":"publisher","first-page":"2397","DOI":"10.1093\/ndt\/gfh050","volume":"19","author":"C Duvic","year":"2004","unstructured":"Duvic C, Desrame J, L\u00e9v\u00eaque C, Nedelec G. Retroperitoneal fibrosis, sclerosing pancreatitis and bronchiolitis obliterans with organizing pneumonia. Nephrol Dial Transplant. 2004;19(9):2397\u20139.","journal-title":"Nephrol Dial Transplant"},{"issue":"9","key":"1781_CR4","doi-asserted-by":"publisher","first-page":"1181","DOI":"10.1038\/modpathol.2012.72","volume":"25","author":"V Deshpande","year":"2012","unstructured":"Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181\u201392.","journal-title":"Mod Pathol"},{"issue":"1","key":"1781_CR5","doi-asserted-by":"publisher","first-page":"260","DOI":"10.1148\/radiol.2511080965","volume":"251","author":"D Inoue","year":"2009","unstructured":"Inoue D, Zen Y, Abo H, Gabata T, Demachi H, Kobayashi T, et al. Immunoglobulin G4-related lung disease: CT findings with pathologic correlations. Radiology. 2009;251(1):260\u201370.","journal-title":"Radiology"},{"issue":"4","key":"1781_CR6","doi-asserted-by":"publisher","first-page":"360","DOI":"10.1097\/RTI.0b013e31813fab9f","volume":"22","author":"H Kobayashi","year":"2007","unstructured":"Kobayashi H, Shimokawaji T, Kanoh S, Motoyoshi K, Aida S. IgG4-positive pulmonary disease. J Thorac Imaging. 2007;22(4):360\u20132.","journal-title":"J Thorac Imaging"},{"issue":"16","key":"1781_CR7","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1186\/s12890-016-0181-9","volume":"25","author":"X Sun","year":"2016","unstructured":"Sun X, Liu H, Feng R, Peng M, Hou X, Wang P, et al. Biopsy-proven IgG4-related lung disease. BMC Pulm Med. 2016;25(16):20.","journal-title":"BMC Pulm Med"},{"issue":"1","key":"1781_CR8","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1186\/s12931-020-01542-6","volume":"21","author":"J Kang","year":"2020","unstructured":"Kang J, Park S, Chae EJ, Song JS, Hwang HS, Kim SJ, et al. Long-term clinical course and outcomes of immunoglobulin G4-related lung disease. Respir Res. 2020;21(1):273.","journal-title":"Respir Res"},{"issue":"1","key":"1781_CR9","doi-asserted-by":"publisher","first-page":"218","DOI":"10.1186\/s12890-020-01250-3","volume":"20","author":"Y Xie","year":"2020","unstructured":"Xie Y, Xiong A, Marion T, Liu Y. Lung nodules and IgG4 related disease: a single-center based experience. BMC Pulm Med. 2020;20(1):218.","journal-title":"BMC Pulm Med"},{"issue":"3","key":"1781_CR10","doi-asserted-by":"publisher","first-page":"410","DOI":"10.1093\/icvts\/ivy279","volume":"28","author":"P Bertoglio","year":"2019","unstructured":"Bertoglio P, Viti A, Paiano S, Assante LR, Bogina GS, Pomari C, et al. IgG4-related disease: a new challenging diagnosis mimicking lung cancer. Interact Cardiovasc Thorac Surg. 2019;28(3):410\u20132.","journal-title":"Interact Cardiovasc Thorac Surg"},{"issue":"3","key":"1781_CR11","doi-asserted-by":"publisher","first-page":"480","DOI":"10.1111\/resp.12016","volume":"18","author":"S Matsui","year":"2013","unstructured":"Matsui S, Hebisawa A, Sakai F, Yamamoto H, Terasaki Y, Kurihara Y, et al. Immunoglobulin G4-related lung disease: clinicoradiological and pathological features. Respirology. 2013;18(3):480\u20137.","journal-title":"Respirology"},{"issue":"10","key":"1781_CR12","doi-asserted-by":"publisher","first-page":"555","DOI":"10.6061\/clinics\/2016(10)01","volume":"71","author":"ZG Chu","year":"2016","unstructured":"Chu ZG, Sheng B, Liu MQ, Lv FJ, Li Q, Ouyang Y. Differential diagnosis of solitary pulmonary inflammatory lesions and peripheral lung cancers with contrast-enhanced computed tomography. Clinics (Sao Paulo). 2016;71(10):555\u201361.","journal-title":"Clinics (Sao Paulo)"},{"issue":"1","key":"1781_CR13","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1148\/radiol.2391050343","volume":"239","author":"HT Winer-Muram","year":"2006","unstructured":"Winer-Muram HT. The solitary pulmonary nodule. Radiology. 2006;239(1):34\u201349.","journal-title":"Radiology"},{"issue":"1","key":"1781_CR14","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1148\/radiology.144.1.6283592","volume":"144","author":"ER Heitzman","year":"1982","unstructured":"Heitzman ER, Markarian B, Raasch BN, Carsky EW, Lane EJ, Berlow ME. Pathways of tumor spread through the lung: radiologic correlations with anatomy and pathology. Radiology. 1982;144(1):3\u201314.","journal-title":"Radiology"},{"issue":"6","key":"1781_CR15","doi-asserted-by":"publisher","first-page":"497","DOI":"10.1080\/03009742.2019.1602882","volume":"48","author":"H Yang","year":"2019","unstructured":"Yang H, Li J, Wang Y, Ye S, Li J. Distribution characteristics of elevated serum immunoglobulin G4 (IgG4) and its relationship with IgG4-related disease. Scand J Rheumatol. 2019;48(6):497\u2013504.","journal-title":"Scand J Rheumatol"},{"issue":"3","key":"1781_CR16","doi-asserted-by":"publisher","first-page":"1825","DOI":"10.21037\/jtd.2018.01.149","volume":"10","author":"A Wang","year":"2018","unstructured":"Wang A, Fan J, Chen X, Wang S. An initial exploration for comprehensive assessment of IgG4-related lung disease: analyses on the cases enrolled from a systematic review. J Thorac Dis. 2018;10(3):1825\u201341.","journal-title":"J Thorac Dis"},{"key":"1781_CR17","first-page":"4716793","volume":"2020","author":"Z Wen","year":"2020","unstructured":"Wen Z, Huang Y, Ling Z, Chen J, Wei X, Su R, et al. Lack of efficacy of combined carbohydrate antigen markers for lung cancer diagnosis. Dis Mark. 2020;2020:4716793.","journal-title":"Dis Mark"},{"issue":"1","key":"1781_CR18","doi-asserted-by":"publisher","first-page":"e80","DOI":"10.5301\/jbm.5000177","volume":"31","author":"L Wang","year":"2016","unstructured":"Wang L, Wang D, Zheng G, Yang Y, Du L, Dong Z, et al. Clinical evaluation and therapeutic monitoring value of serum tumor markers in lung cancer. Int J Biol Mark. 2016;31(1):e80\u20137.","journal-title":"Int J Biol Mark"},{"issue":"1","key":"1781_CR19","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1016\/j.lungcan.2013.01.002","volume":"80","author":"K Okamura","year":"2013","unstructured":"Okamura K, Takayama K, Izumi M, Harada T, Furuyama K, Nakanishi Y. Diagnostic value of CEA and CYFRA 21-1 tumor markers in primary lung cancer. Lung Cancer. 2013;80(1):45\u20139.","journal-title":"Lung Cancer"},{"key":"1781_CR20","first-page":"2013989","volume":"2017","author":"L Liu","year":"2017","unstructured":"Liu L, Teng J, Zhang L, Cong P, Yao Y, Sun G, et al. The combination of the tumor markers suggests the histological diagnosis of lung cancer. Biomed Res Int. 2017;2017:2013989.","journal-title":"Biomed Res Int"},{"key":"1781_CR21","doi-asserted-by":"publisher","first-page":"m1067","DOI":"10.1136\/bmj.m1067","volume":"369","author":"M Lanzillotta","year":"2020","unstructured":"Lanzillotta M, Mancuso G, Della-Torre E. Advances in the diagnosis and management of IgG4 related disease. BMJ. 2020;369:m1067.","journal-title":"BMJ"}],"container-title":["BMC Pulmonary Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12890-021-01781-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12890-021-01781-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12890-021-01781-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,15]],"date-time":"2021-12-15T07:04:30Z","timestamp":1639551870000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcpulmmed.biomedcentral.com\/articles\/10.1186\/s12890-021-01781-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12]]},"references-count":21,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["1781"],"URL":"https:\/\/doi.org\/10.1186\/s12890-021-01781-3","relation":{},"ISSN":["1471-2466"],"issn-type":[{"value":"1471-2466","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,12]]},"assertion":[{"value":"26 October 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 December 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 December 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Approval was obtained from the Ethics Committee of Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University (number, SYSEC-KY-KS-2021-225). This study was exempt from informed consent because of the retrospective nature of the study.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"413"}}