Rare presentation of immunoglobulin G4-related disease as tracheal stenosis: a case report and review of the literature.

Abstract

Immunoglobulin G4-related disease (IgG4- RD) is a systemic inflammatory condition that can affect any part of the body, although airway involvements are rarely seen. 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 treated 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.