Recurrent endobronchial occlusion and aorto-bronchial fistula formation in Behcet's disease

Abstract

Background: Behcet's disease is a multi-system inflammatory disorder. A small subset of patients with Behcet's develop relapsing polychondritis which is classified as a separate disease known as Mouth and Genital ulcers with inflamed cartilage (MAGIC syndrome). It has previously been observed that this condition can also affect the cartilaginous tissue in the tracheobronchial tree.

Case presentation: We present the case of a 44-year-old lady with Behcet's Disease, Mouth and Genital ulcers with inflamed cartilage (MAGIC) syndrome and an aortic Frozen Elephant Trunk (FET) who presented to hospital with recurrent episodes of left lobar collapse of the lung. During bronchoscopy, we found the presence of multiple inflammatory endobronchial webs occluding segments of the left bronchial tree. Repeated examinations showed evidence that these inflammatory webs were progressing in size, density and location. Furthermore, we noticed herniation of her descending aortic FET into her left bronchial tree forming an aorto-bronchial fistula which was complicated by a graft infection. Her descending aortic FET section was surgically replaced with an open procedure and bronchoscopic interventions attempted to remove the occlusions in her bronchial tree. Despite optimisation of medical management and surgical correction, this patient continued to develop progressive occlusion of her left bronchial tree, resulting in a chronically collapsed left lung.

Conclusions: A multi-disciplinary team approach is of paramount importance in order to optimally manage patients with Behcet's disease, balancing immunosuppressive regimens that need close monitoring and titration in the context of potential surgical intervention and the risk for intercurrent infection.

Keywords: Behcet’s disease; Descending thoracic aortic aneurysm; Endobronchial occlusion; Mouth and Genital ulcers with inflamed cartilage (MAGIC) syndrome.

Fig. 1

PA plain film chest radiograph illustrating complete white out of the left hemi-thorax suggestive of complete left sided collapse

Fig. 2

Bronchoscopic findings in February 2022 identified inflammatory membranes occluding both the left upper and lower lobe bronchi

Fig. 3

Axial slices of PET CT in March 2022 showed foci of increased uptake concerning for a graft infection. Ongoing complete left sided collapse of the lung is also present

Fig. 4

Chest x-ray prior to hospital discharge from July 2022, resolution of left upper lobe collapse

Fig. 5

Chest x-ray one month post hospital discharge from August 2022 (re-collapse of upper lobe)

Fig. 6

Bronchoscopy September 2022 showed complete re-occlusion of left main bronchus with a thick inflammatory exudate