Idiopathic fibrosing mediastinitis

Fibrosing mediastinitis is rare in settings where histoplasmosis is not endemic. An idiopathic form of the disease may present with indistinguishable features and requires methodical exclusion of competing differential diagnoses. We report the case of a 30-year old female patient who presented with intermittent haemoptysis for the past 2 years with no constitutional symptoms. Computed tomography of the chest revealed a prominent right bronchial arterial circulation with a mass-like lesion, which encased and attenuated the right pulmonary trunk and adjacent structures. Endobronchial ultrasonography with transbronchial fine-needle aspiration showed a paucicellular aspirate with no evidence of malignancy or granulomas. Fungal infection, tuberculosis, sarcoidosis, IgG4-disease, and connective tissue disease were ruled out by appropriate serological, molecular, and microbiological tests. A diagnosis of idiopathic fibrosing mediastinitis was therefore made by exclusion and the patient was successfully treated with oral corticosteroids.