{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,6]],"date-time":"2026-06-06T15:20:05Z","timestamp":1780759205763,"version":"3.54.1"},"reference-count":123,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2025,7,5]],"date-time":"2025-07-05T00:00:00Z","timestamp":1751673600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder primarily affecting adults. The disease in pediatric age is unusual and preferentially affects adolescents. In contrast to adults, who commonly exhibit the involvement of multiple organs simultaneously or sequentially over time, young patients tend to present with a localized disease, typically affecting the orbits. Proptosis, ptosis, diplopia, and restricted eye movement may be observed in these patients. Symptoms are proteiform, and the disease is chronic and indolent with a relapsing\u2013remitting course. Diagnostic criteria have been developed for adults, which may not fully capture the pediatric disease phenotype. If untreated or poorly managed, IgG4-RD can lead to progressive fibrosis and scarring of affected organs, potentially causing irreversible damage. We conducted a narrative review using the IMRAD approach, presenting a nonsystematic analysis of the literature on pediatric IgG4-RD. Original papers, case reports\/series, and relevant reviews in English were selected from PubMed, EMBASE, and Web of Science up to January 2024. Keywords included \u201cIgG4-Related Disease\u201d and \u201cpediatric\u201d and, additionally, we presented two original pediatric cases. Our purpose is to offer an overview of IgG4-RD manifestations, and challenges in diagnosing and managing this rare condition in children.<\/jats:p>","DOI":"10.3390\/children12070888","type":"journal-article","created":{"date-parts":[[2025,7,7]],"date-time":"2025-07-07T11:19:27Z","timestamp":1751887167000},"page":"888","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["IgG4-Related Disease in Childhood: Clinical Presentation, Management, and Diagnostic Challenges"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-8113-5829","authenticated-orcid":false,"given":"Silvia","family":"Taranto","sequence":"first","affiliation":[{"name":"Pediatric Department, \u201cVittore Buzzi\u201d Children\u2019s Hospital, 20154 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Bernardo","sequence":"additional","affiliation":[{"name":"Pediatric Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, 20121 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7008-3409","authenticated-orcid":false,"given":"Angela","family":"Mauro","sequence":"additional","affiliation":[{"name":"Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, 20121 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8108-7790","authenticated-orcid":false,"given":"Anna","family":"Perrone","sequence":"additional","affiliation":[{"name":"Radiology Unit, Meyer Children\u2019s Hospital IRCCS, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agnese","family":"Tamborino","sequence":"additional","affiliation":[{"name":"Infectious Disease Unit, Meyer Children\u2019s Hospital IRCCS, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9292-7601","authenticated-orcid":false,"given":"Teresa","family":"Giani","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Meyer Children\u2019s Hospital IRCCS, 50139 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,7,5]]},"reference":[{"key":"ref_1","unstructured":"Nambiar, S., and Oliver, T.I. (2024, April 07). IgG4-Related Disease, StatPearls, Available online: http:\/\/www.ncbi.nlm.nih.gov\/books\/NBK499825\/."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1812","DOI":"10.1097\/PAS.0b013e3181f7266b","article-title":"IgG4-Related Disease: A Cross-sectional Study of 114 Cases","volume":"34","author":"Zen","year":"2010","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1146\/annurev-med-050219-034449","article-title":"Clinical Perspectives on IgG4-Related Disease and Its Classification","volume":"73","author":"Katz","year":"2022","journal-title":"Annu. Rev. Med."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"732","DOI":"10.1056\/NEJM200103083441005","article-title":"High serum IgG4 concentrations in patients with sclerosing pancreatitis","volume":"344","author":"Hamano","year":"2001","journal-title":"N. Engl. J. Med."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"957","DOI":"10.1136\/ard-2023-223950","article-title":"Incidence, prevalence and mortality of IgG4-related disease in the USA: A claims-based analysis of commercially insured adults","volume":"82","author":"Wallace","year":"2023","journal-title":"Ann. Rheum. Dis."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"100074","DOI":"10.1016\/j.jtauto.2020.100074","article-title":"IgG4-related disease: Changing epidemiology and new thoughts on a multisystem disease","volume":"4","author":"Floreani","year":"2020","journal-title":"J. Transl. Autoimmun."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1186\/s12969-016-0079-3","article-title":"IgG4-related disease: A systematic review of this unrecognized disease in pediatrics","volume":"14","author":"Karim","year":"2016","journal-title":"Pediatr. Rheumatol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"2466","DOI":"10.1002\/art.39205","article-title":"IgG4-Related Disease: Baseline clinical and laboratory features in 125 patients with biopsy-proven disease","volume":"67","author":"Wallace","year":"2015","journal-title":"Arthritis Rheumatol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1616","DOI":"10.3906\/sag-2006-375","article-title":"IgG4-related disease: A contemporary review","volume":"50","author":"Karadeniz","year":"2020","journal-title":"Turk. J. Med. Sci."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1080\/14397595.2020.1859710","article-title":"The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD","volume":"31","author":"Umehara","year":"2021","journal-title":"Mod. Rheumatol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1002\/art.41120","article-title":"The 2019 American College of Rheumatology\/European League Against Rheumatism Classification Criteria for IgG4-Related Disease","volume":"72","author":"Wallace","year":"2020","journal-title":"Arthritis Rheumatol."},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Feng, X., Zhang, L., Chen, F., and Yuan, G. (2022). Multi-System Langerhans Cell Histiocytosis as a Mimic of IgG4-Related Disease: A Case Report and Literature Review. Front. Endocrinol., 13.","DOI":"10.3389\/fendo.2022.896227"},{"key":"ref_13","first-page":"409","article-title":"Immunoglobulin G4-related orbital disease: Report of two pediatric cases","volume":"33","author":"Batu","year":"2015","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/S0899-3467(07)60142-6","article-title":"Writing narrative literature reviews for peer-reviewed journals: Secrets of the trade","volume":"5","author":"Green","year":"2006","journal-title":"J. Chiropr. Med."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"21","DOI":"10.3109\/s10165-011-0571-z","article-title":"Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011","volume":"22","author":"Umehara","year":"2012","journal-title":"Mod. Rheumatol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1186\/s12969-023-00799-7","article-title":"IgG4 related pericardium and lung disease in pediatric patient complicated with fatal massive hemoptysis: A case report and review of literature","volume":"21","author":"Saad","year":"2023","journal-title":"Pediatr. Rheumatol. Online J."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1177","DOI":"10.1007\/s00296-021-04885-5","article-title":"IgG4-related disease in pediatric patients: A single-center experience","volume":"42","author":"Atalay","year":"2022","journal-title":"Rheumatol. Int."},{"key":"ref_18","first-page":"389","article-title":"A clinicopathologic case study of two patients with pediatric orbital IgG4-related disease","volume":"32","author":"Griepentrog","year":"2013","journal-title":"Orbit Amst. Neth."},{"key":"ref_19","first-page":"137","article-title":"IgG4 orbital inflammation in a 5-year-old child presenting as an orbital mass","volume":"32","author":"Kalapesi","year":"2013","journal-title":"Orbit Amst. Neth."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1055\/s-0034-1398475","article-title":"IgG4-sclerosing cholangitis in a pediatric patient","volume":"35","author":"Rosen","year":"2015","journal-title":"Semin. Liver Dis."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"e8194","DOI":"10.1002\/ccr3.8194","article-title":"Association of autoimmune pancreatitis with Raghib syndrome","volume":"11","author":"Yousefi","year":"2023","journal-title":"Clin. Case Rep."},{"key":"ref_22","unstructured":"Singla, T., and Bagri, N.K. (2024, April 09). IGG4 Related Disease-A Case Series of Four Pediatric Patients-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=1&page=1&id=L642856541."},{"key":"ref_23","unstructured":"Singh, S., Singh, A., and Ahluwalia, J. (2024, February 22). IgG4 Related Disorder: A Clinical Possibility in Children with Peripheral Gangrene-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=4&page=8&id=L624232028."},{"key":"ref_24","unstructured":"Navetta-Modrov, B., Stone, J., and Bonagura, V. (2024, April 09). IgG4 Related Disease (IgG4RD) in an Adolescent Male Misdiagnosed as Autoimmune lymphoproliferative Syndrome-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=2&page=1&id=L622389217."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"e115","DOI":"10.14309\/crj.2016.88","article-title":"Autoimmune Pancreatitis and IgG4 Related Disease in Three Children","volume":"3","author":"Bolia","year":"2016","journal-title":"ACG Case Rep. J."},{"key":"ref_26","unstructured":"Jamee, M., Fallahi, M., and Enayat, J. (2024, February 21). Chronic Granulomatous Disease with Associated IgG4-Related Disease Masquerading as Pulmonary Pseudotumor-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=3&page=5&id=L634909463."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"611","DOI":"10.5858\/arpa.2021-0041-OA","article-title":"Clinicopathologic Characterization of IgG4-Rich Pediatric Head and Neck Lesions","volume":"146","author":"Bu","year":"2022","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"S21","DOI":"10.5152\/eurjrheum.2019.19165","article-title":"Orbital inflammation and colitis in pediatric IgG4-related disease: A case report and review of the literature","volume":"7","author":"Tille","year":"2020","journal-title":"Eur. J. Rheumatol."},{"key":"ref_29","first-page":"136","article-title":"Tumour of the orbit and pterygopalatine fossa: Delayed recognition of possible IgG4-related disease","volume":"24","author":"Dylewska","year":"2020","journal-title":"Contemp. Oncol. Onkol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1721","DOI":"10.1007\/s10067-017-3934-9","article-title":"Ocular manifestations of IgG4-related disease in children. More common than anticipated? Review of the literature and case report","volume":"37","author":"Smerla","year":"2018","journal-title":"Clin. Rheumatol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"742","DOI":"10.1111\/jgh.14525","article-title":"Clinical and histopathological features of immunoglobulin G4-associated autoimmune hepatitis in children","volume":"34","author":"Aydemir","year":"2019","journal-title":"J. Gastroenterol. Hepatol."},{"key":"ref_32","first-page":"e8846","article-title":"Pediatric Parotid Chronic Sclerosing Sialadenitis in an African-American Female: A Rare Case and Review of the Literature","volume":"12","author":"Keidar","year":"2020","journal-title":"Cureus"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"e125","DOI":"10.1097\/RLU.0000000000003358","article-title":"FDG-Avid Pulmonary Nodules and Tracheobronchial Mural Inflammation in IgG4-Related Disease","volume":"46","author":"Namireddy","year":"2021","journal-title":"Clin. Nucl. Med."},{"key":"ref_34","first-page":"140753","article-title":"Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge","volume":"2015","author":"Corujeira","year":"2015","journal-title":"Case Rep. Pediatr."},{"key":"ref_35","first-page":"226","article-title":"Immunoglobulin G4-related disease with recurrent uveitis and kidney tumor mimicking childhood polyarteritis nodosa: A rare case report","volume":"43","author":"Nastri","year":"2018","journal-title":"Acta Reumatol. Port."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1423","DOI":"10.1007\/s00381-019-04187-z","article-title":"Large dural-based mass with bony hyperostosis in a 16-year-old male: IgG4-related disease mimicking lymphoplasmacyte-rich meningioma","volume":"35","author":"Nambirajan","year":"2019","journal-title":"Childs Nerv. Syst."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"3817","DOI":"10.1007\/s10067-021-05635-5","article-title":"IgG4-related disease and ANCA positive vasculitis in childhood: A case-based review","volume":"40","author":"Demir","year":"2021","journal-title":"Clin. Rheumatol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1059","DOI":"10.1007\/s13312-019-1691-3","article-title":"IgG4-related Disease at Rectovesical Pouch Mimicking Inflammatory Myofibroblastic Tumor","volume":"56","author":"Chakrabarti","year":"2019","journal-title":"Indian. Pediatr."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"495","DOI":"10.24953\/turkjped.2020.03.020","article-title":"A new location for pediatric immunoglobulin G4 related disease: The biceps muscle","volume":"62","author":"Ekim","year":"2020","journal-title":"Turk. J. Pediatr."},{"key":"ref_40","first-page":"191","article-title":"IgG4 related disease in a seven year old girl with multiple organ involvement: A rare presentation","volume":"55","author":"Akkelle","year":"2020","journal-title":"Turk. Arch. Pediatr. Pediatri Ar\u015f."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1186\/s13052-016-0269-0","article-title":"Pulmonary manifestation of immunoglobulin G4-related disease in a 7-year-old immunodeficient boy with Epstein-Barr virus infection: A case report","volume":"42","author":"Breborowicz","year":"2016","journal-title":"Ital. J. Pediatr."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1016\/j.radcr.2016.10.008","article-title":"IgG4-related disease in an adolescent with radiologic-pathologic correlation","volume":"12","author":"Lieberman","year":"2017","journal-title":"Radiol. Case Rep."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/j.jaapos.2017.08.013","article-title":"Lacrimal gland abscess in a child as a rare manifestation of IgG4-related disease","volume":"22","author":"Raab","year":"2018","journal-title":"J. Am. Assoc. Pediatr. Ophthalmol. Strabismus"},{"key":"ref_44","doi-asserted-by":"crossref","unstructured":"Gabrovska, N., Velizarova, S., Spasova, A., Kostadinov, D., Yanev, N., Shivachev, H., Rangelov, E., Pahnev, Y., Antonova, Z., and Kartulev, N. (2021). A Case of Tracheal Stenosis as an Isolated Form of Immunoproliferative Hyper-IgG4 Disease in a 17-Year-Old Girl. Children, 8.","DOI":"10.3390\/children8070589"},{"key":"ref_45","doi-asserted-by":"crossref","unstructured":"Timeus, F., Calvo, M.M., Caci, A.M., Gallone, G.O., and Vittone, F. (2021). IgG4-related chronic sclerosing sialadenitis in a child with recurrent parotitis: A case report. BMC Pediatr., 21.","DOI":"10.1186\/s12887-021-03004-4"},{"key":"ref_46","first-page":"e15014","article-title":"Immunoglobulin G4-related orbital disease in an 8-year-old girl","volume":"64","author":"Hoshiyama","year":"2022","journal-title":"Pediatr. Int. Off. J. Jpn. Pediatr. Soc."},{"key":"ref_47","first-page":"255","article-title":"IgG4-related disease of the orbit in an infant","volume":"25","author":"Tong","year":"2021","journal-title":"J. AAPOS Off. Publ. Am. Assoc. Pediatr. Ophthalmol. Strabismus"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"140","DOI":"10.4103\/0022-3859.65294","article-title":"Immunoglobulin G4-associated cholangitis mimicking cholangiocarcinoma in a young boy","volume":"56","author":"Miglani","year":"2010","journal-title":"J. Postgrad. Med."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1097\/MPG.0b013e31820e82a1","article-title":"A 3-year-old with immunoglobulin G4-associated cholangitis","volume":"53","author":"Ibrahim","year":"2011","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/1546-0096-9-1","article-title":"Successful treatment of pediatric IgG4 related systemic disease with mycophenolate mofetil: Case report and a review of the pediatric autoimmune pancreatitis literature","volume":"9","author":"Mannion","year":"2011","journal-title":"Pediatr. Rheumatol. Online J."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"709264","DOI":"10.1155\/2011\/709264","article-title":"Variable Clinical Presentations of Riedel\u2019s Thyroiditis: Report of Two Cases","volume":"2011","author":"Zakeri","year":"2011","journal-title":"Case Rep. Med."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"165","DOI":"10.2350\/11-04-1023-OA.1","article-title":"Pediatric chronic sclerosing sialadenitis: K\u00fcttner tumor","volume":"15","author":"Melo","year":"2012","journal-title":"Pediatr. Dev. Pathol. Off. J. Soc. Pediatr. Pathol. Paediatr. Pathol. Soc."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"e81","DOI":"10.1016\/j.crohns.2012.05.003","article-title":"The successful treatment of IgG4-positive colitis with adalimumab in a patient with IgG4-related sclerosing disease--a new subtype of aggressive colitis?","volume":"7","author":"Naghibi","year":"2013","journal-title":"J. Crohns Colitis"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1378\/chest.12-3088","article-title":"Uncommon pulmonary presentation of IgG4-related disease in a 15-year-old boy","volume":"144","author":"Pifferi","year":"2013","journal-title":"Chest"},{"key":"ref_55","first-page":"548","article-title":"Immunoglobulin G4-related sclerosing disease with orbital inflammation in a 12-year-old girl","volume":"17","author":"Sane","year":"2013","journal-title":"J. AAPOS Off. Publ. Am. Assoc. Pediatr. Ophthalmol. Strabismus"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"190","DOI":"10.1016\/j.jbspin.2013.07.011","article-title":"Successful use of rituximab in a young patient with immunoglobulin G4-related disease and refractory scleritis","volume":"81","author":"Caso","year":"2014","journal-title":"Jt. Bone Spine"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"385","DOI":"10.4103\/1319-3767.145333","article-title":"IgG4-related sclerosing mesenteritis in a 7-year-old Saudi girl","volume":"20","author":"Hasosah","year":"2014","journal-title":"Saudi J. Gastroenterol. Off. J. Saudi Gastroenterol. Assoc."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"1108","DOI":"10.1007\/s12098-014-1471-y","article-title":"IgG4-related orbital inflammation presenting as unilateral pseudotumor","volume":"81","author":"Jariwala","year":"2014","journal-title":"Indian. J. Pediatr."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"1264","DOI":"10.1038\/eye.2014.154","article-title":"IgG4-related orbital inflammation presenting as bilateral proptosis in a child","volume":"28","author":"Mittal","year":"2014","journal-title":"Eye"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"e161","DOI":"10.1097\/IOP.0000000000000016","article-title":"IgG4-related dacryoadenitis in a 13-year-old girl","volume":"30","author":"Notz","year":"2014","journal-title":"Ophthal. Plast. Reconstr. Surg."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"117","DOI":"10.4103\/0971-3026.134384","article-title":"IgG4-related disease with sinonasal involvement: A case series","volume":"24","author":"Prabhu","year":"2014","journal-title":"Indian. J. Radiol. Imaging"},{"key":"ref_62","first-page":"549","article-title":"Successful treatment of IgG-4 related sclerosing disease with rituximab: A novel case report","volume":"33","author":"Gillispie","year":"2015","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1977","DOI":"10.1002\/art.39131","article-title":"Hepatic Mass and Coagulopathy in a Ten-Year-Old Boy With Fever","volume":"67","author":"Nada","year":"2015","journal-title":"Arthritis Rheumatol."},{"key":"ref_64","doi-asserted-by":"crossref","unstructured":"Meli, M., Arrabito, M., Salvatorelli, L., Soma, R., Presti, S., Licciardello, M., Miraglia, V., Scuderi, M.G., Belfiore, G., and Magro, G. (2022). Report of Two Cases of Pediatric IgG4-Related Lymphadenopathy (IgG4-LAD): IgG4-Related Disease (IgG4-RD) or a Distinct Clinical Pathological Entity?. Children, 9.","DOI":"10.3390\/children9101472"},{"key":"ref_65","doi-asserted-by":"crossref","unstructured":"Kato, D., Uchida, H., Hinoki, A., Sumida, W., Shirota, C., Makita, S., Okamoto, M., Takimoto, A., Takada, S., and Nakagawa, Y. (2023). IgG4-related disease of duodenal obstruction due to multiple ulcers in a 12-year-old girl. BMC Pediatr., 23.","DOI":"10.1186\/s12887-023-04190-z"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1016\/j.ehpc.2015.08.002","article-title":"IgG4-related disease simulating Hodgkin lymphoma in a child","volume":"4","author":"Ewing","year":"2016","journal-title":"Hum. Pathol. Case Rep."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"S6","DOI":"10.1016\/j.anai.2016.09.376","article-title":"O016 IgG4-related ophthalmic disease in children: A case report","volume":"117","year":"2016","journal-title":"Ann. Allergy. Asthma. Immunol."},{"key":"ref_68","first-page":"1334","article-title":"Germany IgG4-Related Disease and Successful Treatment with Rituximab in a Three-Year-Old Boy","volume":"8","author":"Marissen","year":"2021","journal-title":"Ann. Hematol. Oncol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"S107","DOI":"10.1016\/j.anai.2021.08.336","article-title":"M195 IGG4-Related Disease with Nasopharyngeal Masses in the Pediatric Patient","volume":"127","author":"Woo","year":"2021","journal-title":"Ann. Allergy Asthma. Immunol."},{"key":"ref_70","first-page":"129","article-title":"Unusual presentation of immunoglobulin G4-related disease: A case report","volume":"36","author":"Zeybek","year":"2021","journal-title":"Arch. Rheumatol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"3699","DOI":"10.1016\/j.radcr.2023.07.062","article-title":"Coronary artery involvement in a patient with IgG4-related disease","volume":"18","author":"Mohammadzadeh","year":"2023","journal-title":"Radiol. Case Rep."},{"key":"ref_72","unstructured":"Pac, M., Obrycki, L., Sarnecki, J., Szymanska, S., and Litwin, M. (2024, February 20). A Rare Cause of Acute Tubulointerstitial Nephritis in a 16.5-Year-Old Boy-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=22&page=1&id=L641736945."},{"key":"ref_73","unstructured":"Rodrigues, S.S.S., Salazar, L., Lima, J.B., Fraga, J., Alves, S., and Zilhao, C. (2024, April 09). Mikulicz Disease-a Sjogren\u2019s Mimicker-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=3&page=1&id=L642856169."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"109455","DOI":"10.1016\/j.clim.2023.109455","article-title":"More Than Meets the Eye: IgG4-related Disease presentation as fluctuating ocular symptoms","volume":"250","author":"Hsueh","year":"2023","journal-title":"Clin. Immunol."},{"key":"ref_75","doi-asserted-by":"crossref","unstructured":"Ma, B., Li, Y., Wang, X., Du, L., Wang, S., Ma, H., Zhou, D., Usman, T., Lu, L., and Qu, S. (2022). Association Between Abdominal Adipose Tissue Distribution and Obstructive Sleep Apnea in Chinese Obese Patients. Front. Endocrinol., 13.","DOI":"10.3389\/fendo.2022.847324"},{"key":"ref_76","unstructured":"Farha, G., and Derouen, J. (2024, February 20). Pediatric IgG4 Related Disease of the Orbit-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=4&page=2&id=L2022350567."},{"key":"ref_77","unstructured":"Niksic, S., Slak, P., Brajovic, S.M., and Plut, D. (2024, February 21). Unusual Case of an IgG4-Related Disease-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=24&page=1&id=L641875120."},{"key":"ref_78","unstructured":"Tsygin, A., Dmitrienko, S., Ananin, P., Vorobyova, O., Savostyanov, K., Vashurina, T., and Fisenko, A. (2024, February 21). Case of IGG-4 Related Disease with Kidney Involvement in Child. Efficacy of Long Term Immunosuppressive Treatment-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=17&page=2&id=L639293233."},{"key":"ref_79","doi-asserted-by":"crossref","unstructured":"Qing, P., Lu, C., Yan, B., Liu, C., Fox, D.A., Zhao, Y., Liu, Y., and Tan, C. (2022). Case report: IgG4-related intracranial lesions mimicking multiple sclerosis in a 14-year-old girl. Front. Neurol., 13, Available online: https:\/\/www.frontiersin.org\/journals\/neurology\/articles\/10.3389\/fneur.2022.1007153.","DOI":"10.3389\/fneur.2022.1007153"},{"key":"ref_80","unstructured":"Kasap-Demir, B. (2024, February 21). Rapid Response to Pulse Methylprednisolone in an Adolescent Girl with IGG4 Disease Related Orbital Mass-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=3&page=3&id=L639228502."},{"key":"ref_81","unstructured":"Qi, S.R., Hebert, M., You, E., Proulx-Gauthier, J.-P., and Legare, M.E. (2024, April 09). Conjunctival Infiltration in a Child as a Rare Manifestation of IgG4-Related Disease-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=20&page=1&id=L2017431387."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"e244202","DOI":"10.1136\/bcr-2021-244202","article-title":"IgG4-related disease at the foramen magnum and craniovertebral junction compressing the medulla","volume":"14","author":"Jesus","year":"2021","journal-title":"BMJ Case Rep."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1007\/s12098-021-03743-3","article-title":"IgG4-Related Coronary Aneurysm in a Child","volume":"88","author":"Vasudevan","year":"2021","journal-title":"Indian. J. Pediatr."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1002\/aid2.13183","article-title":"Immunoglobulin G4-related sclerosing cholangitis in a 3 years of age boy","volume":"8","author":"Hsu","year":"2021","journal-title":"Adv. Dig. Med."},{"key":"ref_85","first-page":"109","article-title":"Resolution of IgG4-related retroperitoneal fibrosis with rituximab in a paediatric patient","volume":"23","author":"Raja","year":"2020","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_86","first-page":"1832","article-title":"IgG4 related disease: Nephropathy and bone marrow failure in a 2 year-old child","volume":"35","author":"Pierri","year":"2020","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"S90","DOI":"10.1016\/j.pathol.2020.01.306","article-title":"Orbital IgG4 Related disease in a patient with irka variant gene: A case report","volume":"52","author":"Tanzifi","year":"2020","journal-title":"Pathology"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"kez416.010","DOI":"10.1093\/rheumatology\/kez416.010","article-title":"P43\u2003Immunoglobulin G4-related disease in a 10 year-old girl with multisystem involvement","volume":"58","author":"Cinar","year":"2019","journal-title":"Rheumatology"},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"1341","DOI":"10.1136\/annrheumdis-2019-eular.7273","article-title":"Sat0504 Igg4 Related Disease in Children: A Single Centre Experience from North-West India","volume":"78","author":"Kumar","year":"2019","journal-title":"Ann. Rheum. Dis."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/j.eucr.2018.09.016","article-title":"A case of IgG4-related renal pseudotumor in a child with history of Wilms tumor","volume":"21","author":"Johnson","year":"2018","journal-title":"Urol. Case Rep."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"key273.024","DOI":"10.1093\/rheumatology\/key273.024","article-title":"P22\u2003Orbital mass in a 9 year old girl","volume":"57","author":"Deepak","year":"2018","journal-title":"Rheumatology"},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"1651","DOI":"10.1136\/annrheumdis-2018-eular.5673","article-title":"AB1082 Characterisation of a group of patients with igg4-related disease: Single centre experience","volume":"77","author":"Kozlova","year":"2018","journal-title":"Ann. Rheum. Dis."},{"key":"ref_93","first-page":"2739","article-title":"Concurrent eosinophilia and IgG4-related disease in a child: A case report and review of the literature","volume":"15","author":"Chen","year":"2018","journal-title":"Exp. Ther. Med."},{"key":"ref_94","unstructured":"Parvaneh, V.J., Shiari, R., and Rahmani, K. (2024, April 09). Orbital Mass: The Presentation of IgG4-Related Disease in 12- Year-Old Children-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=1&page=1&id=L625168244."},{"key":"ref_95","unstructured":"Eng, V., Jean, T., Pourang, D., Ramanathan, A., Eichhorn, K., and Hever, A. (2024, February 23). IGG4-Related Orbital Disease with Anca Positivity: A Case Report-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=21&page=8&id=L619349156."},{"key":"ref_96","unstructured":"Ozdemir, K., Basaran, C., Selver, O.B., Palamar, M., Kutukculer, N., Yagci, A., and Serdaroglu, E. (2024, April 09). IgA Nephropathy and IgG4 Related Orbital Disease: A Child Case-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=15&page=1&id=L618119403."},{"key":"ref_97","unstructured":"Diaz, T., Ramirez, Y., Osorio, S., Brana, M.T., Aparicio, L., Rodriguez, A., Faugier, E., and Maldonado, R. (2024, February 23). Successful Treatment of Pediatric IGG4 Related Ophthalmic Disease with Mycophenolate Mofetil: Case Report and a Review of the Pediatric Autoimmune Dacryoadenitis Literature-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&id=L618421202."},{"key":"ref_98","unstructured":"Okamoto, N., Sugita, Y., Shabana, K., Murata, T., and Tamai, H. (2024, April 09). A Fourteen-Year-Old Girl with Immunoglobulin G4-Related Disease-Record Details-Embase. Available online: https:\/\/www.embase.com\/records?subaction=viewrecord&rid=25&page=1&id=L618532420."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"396","DOI":"10.4046\/trd.2015.78.4.396","article-title":"A Case of Extensive IgG4-Related Disease Presenting as Massive Pleural Effusion, Mediastinal Mass, and Mesenteric Lymphadenopathy in a 16-Year-Old Male","volume":"78","author":"Goag","year":"2015","journal-title":"Tuberc. Respir. Dis."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1007\/s12328-020-01337-8","article-title":"IgG4-related disease manifesting as symptoms of appendicitis: Case report and literature review","volume":"14","year":"2021","journal-title":"Clin. J. Gastroenterol."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1007\/s00256-019-03250-9","article-title":"IgG4-related disease: Rare presentation as a soft-tissue mass in the thigh of an adolescent","volume":"49","author":"Creze","year":"2020","journal-title":"Skelet. Radiol."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1056\/NEJMra1104650","article-title":"IgG4-Related Disease","volume":"366","author":"Stone","year":"2012","journal-title":"N. Engl. J. Med."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"927","DOI":"10.1016\/j.mayocp.2015.03.020","article-title":"Diagnostic Approach to the Complexity of IgG4-Related Disease","volume":"90","author":"Stone","year":"2015","journal-title":"Mayo Clin. Proc."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"e568","DOI":"10.1212\/NXI.0000000000000568","article-title":"Immunoglobulin G4-related hypertrophic pachymeningitis: A case-oriented review","volume":"6","author":"Levraut","year":"2019","journal-title":"Neurol. Neuroimmunol. Neuroinflammation"},{"key":"ref_105","first-page":"e21850","article-title":"IgG4-Related Disease Presenting as Hypertrophic Pachymeningitis","volume":"14","author":"Sapkota","year":"2022","journal-title":"Cureus"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1007\/s00296-019-04502-6","article-title":"Recurrent myelitis and asymptomatic hypophysitis in IgG4-related disease: Case-based review","volume":"40","author":"Vakrakou","year":"2020","journal-title":"Rheumatol. Int."},{"key":"ref_107","first-page":"543","article-title":"Clinical features of hypertrophic pachymeningitis in a center survey","volume":"40","author":"Calejo","year":"2019","journal-title":"Neurol. Sci. Off. J. Ital. Neurol. Soc. Ital. Soc. Clin. Neurophysiol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"2781","DOI":"10.1007\/s00701-022-05340-5","article-title":"IgG4-related hypertrophic pachymeningitis with tumor-like intracranial and intracerebral lesions","volume":"164","author":"Esmaeilzadeh","year":"2022","journal-title":"Acta Neurochir."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"2696","DOI":"10.1016\/j.acra.2023.01.017","article-title":"Immune-Mediated Hypertrophic Pachymeningitis and its Mimickers: Magnetic Resonance Imaging Findings","volume":"30","author":"Matias","year":"2023","journal-title":"Acad. Radiol."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"2290","DOI":"10.1002\/art.39686","article-title":"IgG4-Related Disease: Retrospective Analysis of One Hundred Sixty-Six Patients","volume":"68","author":"Sekiguchi","year":"2016","journal-title":"Arthritis Rheumatol."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"e11413","DOI":"10.1097\/MD.0000000000011413","article-title":"Characteristics, outcome and treatments with cranial pachymeningitis","volume":"97","author":"Mekinian","year":"2018","journal-title":"Medicine"},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1016\/j.jneuroim.2013.10.008","article-title":"Diagnostic value of IgG4 Indices in IgG4-related hypertrophic pachymeningitis","volume":"266","author":"Galli","year":"2014","journal-title":"J. Neuroimmunol."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"236","DOI":"10.1177\/0003489416672476","article-title":"IgG4-Related Disease of Bilateral Temporal Bones","volume":"126","author":"Li","year":"2017","journal-title":"Ann. Otol. Rhinol. Laryngol."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1177\/0003489416678009","article-title":"Diagnostic Challenges in a Case of IgG4-RD Affecting the Temporal Bone","volume":"126","author":"Vuncannon","year":"2017","journal-title":"Ann. Otol. Rhinol. Laryngol."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1016\/j.wneu.2019.10.111","article-title":"IgG4-Related Disease of Skull Base: Case Series of 3 Patients with Headache","volume":"134","author":"Detiger","year":"2020","journal-title":"World Neurosurg."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1016\/j.anl.2023.10.006","article-title":"New insights into chronic rhinosinusitis associated with IgG4-related disease","volume":"51","author":"Takano","year":"2024","journal-title":"Auris. Nasus. Larynx"},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"965","DOI":"10.1177\/0003489415593557","article-title":"Clinical Evaluation of Sinonasal Lesions in Patients With Immunoglobulin G4-Related Disease","volume":"124","author":"Takano","year":"2015","journal-title":"Ann. Otol. Rhinol. Laryngol."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"518","DOI":"10.3109\/00016489.2010.533699","article-title":"IgG4-related chronic rhinosinusitis: A new clinical entity of nasal disease","volume":"131","author":"Moteki","year":"2011","journal-title":"Acta Otolaryngol."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"1688","DOI":"10.1002\/art.39132","article-title":"International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease","volume":"67","author":"Khosroshahi","year":"2015","journal-title":"Arthritis Rheumatol."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1097\/MD.0b013e3182431ef6","article-title":"Rituximab for the treatment of IgG4-related disease: Lessons from 10 consecutive patients","volume":"91","author":"Khosroshahi","year":"2012","journal-title":"Medicine"},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"1171","DOI":"10.1136\/annrheumdis-2014-206605","article-title":"Rituximab for IgG4-related disease: A prospective, open-label trial","volume":"74","author":"Carruthers","year":"2015","journal-title":"Ann. Rheum. Dis."},{"key":"ref_122","unstructured":"(2014, March 13). International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Available online: https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/10.1002\/art.39132."},{"key":"ref_123","doi-asserted-by":"crossref","unstructured":"Sapountzi, E., Kotanidou, E.P., Tsinopoulou, V.-R., Fotis, L., Fidani, L., and Galli-Tsinopoulou, A. (2025). The Management of IgG4-Related Disease in Children: A Systematic Review. 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