Bronchial artery enlargement may be the cause of recurrent haemoptysis in Behçet's syndrome patients with pulmonary artery involvement during follow-up.

Objectives: Haemoptysis occurring in a Behçet's syndrome (BS) patient with pulmonary artery involvement (PAI) during follow-up is usually regarded as PAI relapse. However, bronchial artery enlargement (BAE) may be the source of haemoptysis in some patients.

Methods: A chart review at the end of December 2014 revealed 118 patients with PAI in our centre since 1979. Nine (all men) had recurrent haemoptysis during follow-up which could not be explained with relapse of PAI.

Results: Haemoptysis recurred a median of 1.5 years (IQR: 9 months-5 years) during follow-up. Thorax CT scans did not show relapse of PAI or emergence of BAE. The patients were treated empirically but continued to complain of occasional haemoptysis thereafter. BAE was detected in 8 patients after a median follow-up of 9 years (IQR: 5-12 years). Six patients underwent bronchial artery embolisation that was repeated in 3. One patient with severe pulmonary hypertension died 3 weeks later. The remaining 5 are under follow-up for between 5 months-9 years. Pulmonary infarction and mild hemiparesis occurred in 2 patients after embolisation. One patient died with haemoptysis before undergoing embolisation. Another one with small BAE is under follow-up for 8 years without embolisation. The source of bleeding could not be determined in 1 patient who is now haemoptysis free for 5 years.

Conclusions: BAE may be the source of recurring and fatal haemoptysis in BS patients with PAI during follow-up. Embolisation appears to be a life-saving procedure.