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

Published on Nov 1, 2015in Clinical Imaging1.109
· DOI :10.1016/J.CLINIMAG.2015.07.008
Luis Gorospe6
Estimated H-index: 6
,
Luis Gorospe1
Estimated H-index: 1
(Hospital Universitario de Canarias)
+ 4 AuthorsEva Mañas-Baena1
Estimated H-index: 1
Sources
Abstract
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.
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