A 44-year-old lady with a 27-year history of Behcet’s disease presented to hospital with breathlessness secondary to recurrent collapse of her left lower lobe. Consequent to her underlying disease process, she had incurred numerous vascular complications, including a popliteal aneurysm, a femoral pseudo-aneurysms at previous arterial puncture site, and coronary and descending thoracic aortic aneurysms. She also had a history in keeping with relapsing polychondritis (MAGIC syndrome) and scleritis. Most significantly she received a total aortic arch replacement with a Frozen Elephant Trunk (FET) procedure in 2014 for treatment of extensive aortic aneurysmal disease. Prior to aortic arch replacement she had received treatment sequentially with high dose corticosteroid therapy, azathioprine or methotrexate therapy in combination with infliximab (anti-TNF therapy), pulsed intravenous cyclophosphamide therapy and subsequently B cell depletion therapy in the form of IV Rituximab. At this presentation her Behcet’s was managed with mycophenolate mofetil, IV monthly tocilizumab and long term prednisolone. In September 2021, after presenting with left lower lobar collapse, she underwent a flexible bronchoscopy which showed the presence of an inflammatory membrane occluding the bronchus to her left lower lobe. This was easily cleared and copious volumes of mucus were suctioned. No features of polychondritis or bronchomalacia were found. Biopsies of the membrane did not show any specific pattern of inflammation. Ongoing inflammatory changes were also found in the bronchus to the left upper lobe but without any obstruction to the airways. The airways in her right lung were noted to be completely normal. At first presentation maintenance prednisolone dose was increased from 10 mg OD to 20 mg OD and mycophenolate from 500 mg BD to 1 g BD. She continued to take monthly infusions of tocilizumab.
The following year in February 2022 she again re-presented with worsening breathlessness. A chest x-ray showed a complete white out of her left lung (Fig. 1). A subsequent rigid and flexible bronchoscopy showed the presence of a thick, hyaline, inflammatory membrane occluding both the upper and lower lobe bronchi separately (Fig. 2A). The masses were also noted to be pulsatile and bled quite easily, and on further interrogation of the imaging, it was apparent the truncus arteriosus branches of the left pulmonary artery to the upper lobe were millimetres behind these membranes. The patient was started on a regimen of methylprednisolone and a bronchoscopy was performed a week later after symptomatic improvement in her breathlessness. Repeat bronchoscopy this time showed bulging of the membranous bronchus to the left upper and lower lobes. Significantly, the struts of FET could be seen invading through the bronchus to the upper lobe (red arrow, Fig. 2B).
A PET CT Scan in March showed features concerning of an aortic graft infection and complete collapse of the left lung (Fig. 3). The patient returned to hospital in May for a replacement of her infected FET and repair of her aorto-bronchial fistula. During the operation it was noted that the intima of the aorta in the region of the left main bronchus was thickened and inflammatory. No attempt was made to dissect the bronchus given the potential of fistulation and surrounding tissue fragility. On completion of the operation, bronchoscopy showed a patent left upper lobe bronchus but an oedematous, occluded left lower lobe bronchus. On discharge she was noted to have an isolated left lower lobe collapse on her chest x-rays (Fig. 4).
Follow up in August with a chest x-ray and CT scan showed re-collapse of her left lung, with evidence of her left main bronchus being occluded (Fig. 5). Another bronchoscopy was arranged in September 2022 which showed that her left main bronchus was completely blocked with a hard inflammatory exudate (Fig. 6). It was not possible to dislodge the material with saline injections and it was also not possible to identify the LUL or LLL take-offs as the obstruction was proximal to the secondary carina. Given the recurrence of her condition, a pneumonectomy was considered. However, since the patient felt quite well in herself it was decided to continue managing her obstruction conservatively. Furthermore, there would be a high risk of stump failure with a left pneumectomy as the patient is on a significant immunosuppressive regime.