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Herein, we report a case of young female with tracheal involvement associated with IgG4-RD. She was a 16-year-old patient admitted to the hospital with dyspnea and\u00a0treated with methylprednisolone and methotrexate. We reviewed the clinical characteristics of 18 previously reported cases of tracheal stenosis with IgG4-RD. The most common symptoms were dyspnea (63.2%), cough, and wheezing. Nine patients (47.4%) had isolated tracheal involvement, and five (26.3%) had organ involvement other than airway disease. Most of the patients (14, 73.7%) received systemic glucocorticoid therapy for remission induction, while surgical procedures (10, 52.6%) were the second preferred treatment options. No relapse was observed in any patient who received immunosuppressives and disease-modifying antirheumatic drugs (DMARDs) such as methotrexate, rituximab, azathioprine, tocilizumab, and cyclophosphamide during or after remission induction therapy. In conclusion, IgG4-RD should be kept in mind in the differential diagnosis of patients presenting with clinical findings of tracheal stenosis. This study has shown that although most IgG4-RD patients with tracheal stenosis are controlled with glucocorticoids or surgical procedures, steroid-sparing agents may be needed to prevent relapses.<\/jats:p>","DOI":"10.1007\/s10067-025-07541-6","type":"journal-article","created":{"date-parts":[[2025,6,25]],"date-time":"2025-06-25T20:25:08Z","timestamp":1750883108000},"page":"3369-3376","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Rare presentation of immunoglobulin G4-related disease as tracheal stenosis: a case report and review of the literature"],"prefix":"10.1007","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9749-5585","authenticated-orcid":false,"given":"Emine B\u00fc\u015fra","family":"Ata","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emre","family":"Tekg\u00f6z","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seda","family":"\u00c7olak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ebru","family":"Kara\u00e7al\u0131","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fatih Mehmet","family":"Do\u011fan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muhammet","family":"\u00c7\u0131nar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sedat","family":"Y\u0131lmaz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,6,26]]},"reference":[{"issue":"9","key":"7541_CR1","doi-asserted-by":"publisher","first-page":"591","DOI":"10.1016\/j.autrev.2010.05.003","volume":"9","author":"H Takahashi","year":"2010","unstructured":"Takahashi H, Yamamoto M, Suzuki C, Naishiro Y, Shinomura Y, Imai K (2010) The birthday of a new syndrome: IgG4-related diseases constitute a clinical entity. 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