{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T00:50:31Z","timestamp":1760057431302,"version":"build-2065373602"},"reference-count":22,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2025,2,2]],"date-time":"2025-02-02T00:00:00Z","timestamp":1738454400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: IgG4-related disease is a rare, chronic inflammatory disorder characterized by lymphoplasmacytic infiltration, \u2018storiform\u2019 fibrosis, and elevated IgG4 levels in affected tissues. This disease has a broad and heterogeneous clinical spectrum that includes four main phenotypes: pancreatic\u2013hepatobiliary disease, retroperitoneal\/aortic fibrosis, head and neck disease, and Mikulicz syndrome. Case Description: An 85-year-old male patient with a clinical presentation, which is unusual outside Asia, of IgG4-related disease phenotype Mikulicz syndrome, characterized by bilateral dacryoadenitis, orbital pseudotumor, and no evidence of significant systemic participation. Despite extensive involvement in the orbital and glandular region, the patient did not develop serious organ complications, a behavior rarely documented in the literature. Despite the serum IgG4 levels being normal (&lt;135 mg\/dL), the clinical and radiological picture suggested IgG4-RD, emphasizing the need for a biopsy for a definitive diagnosis. Histopathological examination revealed a dense lymphoplasmacytic infiltrate, storiform fibrosis, and more than 40% IgG4-positive cells, confirming the diagnosis. Results: Treatment with prednisone was initiated alongside azathioprine for long-term control. Calcium and vitamin D3 supplementation were added to prevent glucocorticoid-induced osteoporosis. Remarkable clinical improvement was observed within 24 h, with progressive orbital and glandular symptoms resolution. Over a year, the patient exhibited complete resolution of the orbital tumors, total recovery of vision, and no relapses. The only sequelae observed were dry eye. Conclusions: This case highlights the need to consider IgG4-RD with normal serum IgG4 levels, the importance of histopathology for diagnosis, and the efficacy of steroids as first-line treatment. A multidisciplinary approach is essential for timely treatment.<\/jats:p>","DOI":"10.3390\/jcm14030958","type":"journal-article","created":{"date-parts":[[2025,2,4]],"date-time":"2025-02-04T05:55:22Z","timestamp":1738648522000},"page":"958","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["IgG4-RD-Associated Mikulicz Syndrome Without Classic Systemic Involvement\u2014A Case Report"],"prefix":"10.3390","volume":"14","author":[{"given":"Luis \u00c1ngel","family":"Mendoza-Vargas","sequence":"first","affiliation":[{"name":"Hospital General de Zona 1 \u201cEmilio Varela Luj\u00e1n\u201d, Zacatecas 98000, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Samuel","family":"Sevilla-Fuentes","sequence":"additional","affiliation":[{"name":"Hospital General de Zona 1 \u201cEmilio Varela Luj\u00e1n\u201d, Zacatecas 98000, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5975-6724","authenticated-orcid":false,"given":"Brandon","family":"Bautista-Becerril","sequence":"additional","affiliation":[{"name":"Laboratorio HLA, Instituto Nacional de Enfermedades Respiratorias Ismael Cos\u00edo Villegas, Mexico City 14080, Mexico"},{"name":"Secci\u00f3n de Estudios de Posgrado e Investigaci\u00f3n, Escuela Superior de Medicina, Instituto Polit\u00e9cnico Nacional, Mexico City 11340, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bertha","family":"Bertha\u00fad-Gonz\u00e1lez","sequence":"additional","affiliation":[{"name":"Hospital General de Zacatecas \u201cLuz Gonz\u00e1lez Cos\u00edo\u201d, Zacatecas 98160, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6877-8124","authenticated-orcid":false,"given":"Ramc\u00e9s","family":"Falf\u00e1n-Valencia","sequence":"additional","affiliation":[{"name":"Laboratorio HLA, Instituto Nacional de Enfermedades Respiratorias Ismael Cos\u00edo Villegas, Mexico City 14080, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Linda P.","family":"F\u00e9lix-Mart\u00ednez","sequence":"additional","affiliation":[{"name":"Hospital San Agust\u00edn, El Carmen 98610, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mauricio","family":"Avila-P\u00e1ez","sequence":"additional","affiliation":[{"name":"Facultad de Medicina, Universidad Nacional Aut\u00f3noma de M\u00e9xico, Campus Ciudad Universitaria, Mexico City 04510, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer","family":"Manilla-Gonz\u00e1lez","sequence":"additional","affiliation":[{"name":"Facultad de Medicina, Universidad Popular Aut\u00f3noma del Estado de Puebla, Campus Puebla, Puebla 72410, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,2,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1111\/apm.12845","article-title":"IgG4-related disease: Review of the histopathologic features, differential diagnosis, and therapeutic approach","volume":"126","author":"Bledsoe","year":"2018","journal-title":"APMIS"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1111\/cei.12641","article-title":"Immunology of IgG4-related disease","volume":"181","author":"Lanzillotta","year":"2015","journal-title":"Clin. 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