Background Hemoptysis is the most frequent symptom of the feared pulmonary artery involvement (PAI) in Behçet's syndrome (BS). Some patients with PAI experience recurrent hemoptysis despite clinical response to treatment and regression or even resolution of abnormal radiologic findings. Enlargement of bronchial arteries and collateral formation may be the source of recurring hemoptysis in these patients.
Objectives To evaluate the cause of hemoptysis in BS patients with PAI despite achievement of clinical and radiologic treatment response.
Methods Retrospective chart review of 6 BS patients (all men) having repeated hemoptysis with no overt radiologic findings of active PAI.
Results The mean age of the patients and their mean disease duration at the diagnosis of PAI was 28.8±5.8 SD years and 2±1.5 SD years, respectively. All patients had hemoptysis as the initial symptom of PAI that had been in the form of pulmonary artery aneurysms (PAA) in 5 patients and pulmonary artery thrombosis (PAT) in one. All patients received treatment consisting of monthly cyclophosphamide pulses and corticosteroids (1.7±1.2 mean ± SD years) that was followed by azathioprine. This treatment was successful in ameliorating hemoptysis in all patients except 1 PAA patient who experienced gross hemoptysis necessitating pulmonary artery embolization 2 years later.
Hemoptysis recurred after a mean interval of 5.8±6.2 SD years (range 1 – 17 years). Thorax CT evaluations did not show any new development or worsening of existing PAI. Rather, PAA was resolved in 4 patients and regressed in 1 and PAT was also regressed in 1 patient. On the other hand, enlargement of bronchial arteries and collaterals were detected in 5 patients. Four of them underwent transcatheter bronchial artery embolization that was complicated by hemiparesis in 1 patient. This procedure was successful in controlling hemoptysis but was repeated in 1 patient 2 years later because of newly enlarged bronchial arteries. This patient still continues to experience occasional and small hemoptysis 3 years after the last procedure. The other 3 patients are currently free of hemoptysis ranging for between 3 months and 8 years after the procedure. The fifth patient died of massive hemoptysis in the emergency unit in another center before undergoing embolization. His last CT did not show any evidence of PAI. In the sixth patient, the source of bleeding could not be demonstrated despite repeated bronchoscopies and pulmonary angiography. This patient is now completely symptom free after 4 years and does not take any medications.
Conclusions Enlargement of bronchial arteries and formation of collaterals can complicate the course of PAI during remission and may result in recurrent and potentially fatal hemoptysis in BS patients. Whether this enlargement is only due to the compromised pulmonary circulation or there is also accompanying vasculitis remains to be studied. Transcatheter embolization appears to be successful in controlling hemoptysis in such patients.
Disclosure of Interest None declared
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