We report the case of a 62-year-old woman undergoing study for progressive dyspnea. In the chest CT scan, concentric tracheal stenosis was visualized, caused by a right paratracheal mass measuring 5×2cm.
Fiberoptic bronchoscopy revealed 75% stenosis with normal tracheal mucosa. The percutaneous fine needle aspiration performed endoscopically showed respiratory epithelial cells without atypia.
We performed rigid bronchoscopy (RB) to obtain a new biopsy. The histopathological and immunohistochemical studies found tracheal mucosa with fibrosis and lymphoplasmacytic infiltration consistent with IgG4-related fibrosing mediastinitis (FM).
After obtaining the diagnosis, treatment with corticosteroids began. Subsequently, RB was programmed for placement of a Dumon® tracheal stent measuring 18×40mm.
FM is a rare entity that can be classified as idiopathic or secondary. IgG4-related FM has recently been linked to some cases defined until now as idiopathic. This disease must be included in the differential diagnosis of fibrosing mediastinitis1 and mediastinal and tracheal neoplasms, because, as in our patient, these entities can mimic even malignant tracheal disease.
Although treatment can consist of corticosteroids, our patient required placement of a tracheal stent due to critical stenosis of the airway (Fig. 1).
Please cite this article as: Arroyo Pareja L, Triviño A, España C. Estenosis traqueal por mediastinitis fibrosante relacionada con enfermedad por IgG4. Arch Bronconeumol. 2019;55:533–534.
