Idiopathic fibrosing mediastinitis: spectrum of imaging findings with emphasis on its association with IgG4-related disease

Abstract

Purpose: The purpose of this study was to review the clinicoradiological features of idiopathic fibrosing mediastinitis (FM).

Methods: The records of six patients with idiopathic FM were retrospectively reviewed.

Results: The most common presenting symptom was cough. There were three localized forms and three diffuse forms. Definitive histological diagnosis was most commonly made with mediastinoscopy. Interestingly, three of our patients had disease manifestations of IgG4-related disease (IgG4-RD) outside the mediastinum.

Conclusion: The imaging findings of idiopathic FM vary depending on the involved mediastinal structures. Therapeutic options include medical treatment, stenting, or surgery. Radiologists should pay attention to the possible association of idiopathic FM with extrathoracic manifestations of the IgG4-RD spectrum.

Keywords: Fibrosing mediastinitis; IgG4-related disease; MDCT; Mediastinal fibrosis; Mediastinum.