Bronchial Artery Enlargement May Cause Recurring Hemoptysis in Behçet's Syndrome Patients with Pulmonary Artery Involvement Despite Response to Treatment

Vedat Hamuryudan¹, Emire Seyahi², Serdal Ugurlu³, Fatih Gulsen⁴, Canan Akman⁴, Murat Cantasdemir⁴, Furuzan Numan⁴, Hasan Tuzun⁵ and Hasan Yazici⁶, ¹Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, ²Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Turkey, Istanbul, Turkey, ³Rheumatology, Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, ⁴Cerrahpasa Medical Faculty, University of Istanbul, Department of Radiology, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, ⁵Cerrahpasa Medical Faculty, University of Istanbul, Department of Vascular Surgery, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, ⁶Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Pulmonary Involvement and vasculitis

Session Information

Date: Tuesday, November 10, 2015

Title: Vasculitis Poster III

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose:

Hemoptysis is the most frequent and feared symptom of pulmonary artery involvement (PAI) in Behçet’s syndrome (BS). Some BS patients with PAI experience recurrent hemoptysis after achieving clinical response and regression or even resolution of abnormal radiologic findings with treatment. Enlargement of bronchial arteries may be the source of recurring hemoptysis in these patients.

Methods:

Retrospective chart review of 6 BS patients (all men) having repeated hemoptysis with no overt clinical or radiologic findings for active PAI.

Results:

The mean age of the patients at the diagnosis of PAI was 28.8± 5.8 SD years and their mean disease duration was 2±1.5 SD years. Five patients had pulmonary artery aneurysms (PAA) and 1 had pulmonary artery thrombosis (PAT). Consolidation treatment consisted of monthly cyclophosphamide pulses and corticosteroids (1.7±1.2 mean±SD years) and this was followed by maintenance with 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 in all after a mean interval of 5.8±6.2 SD years (range 1 – 17 years). Thorax CT evaluations did not show any signs for new or worsening of existing PAI. Rather, there was complete resolution of PAA in 4 patients, regression of PAA in 1 patient and regression of PAT in 1 patient. However, enlargement of bronchial arteries and enlarged collaterals were evident in 5 patients. Four of them underwent transcatheter bronchial artery embolization that was complicated by hemiparesis in 1 patient. This procedure was repeated in 1 patient 2 years later because of new enlarged epigastric 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 emergency unit in another center before undergoing embolization. His last CT did not show any evidence of PAI. The source of bleeding could not be demonstrated in the last patient despite repeated bronchoscopies and pulmonary angiography who is now completely symptom free since 4 years and does not take any medications either.

Conclusion:

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: V. Hamuryudan, None; E. Seyahi, None; S. Ugurlu, None; F. Gulsen, None; C. Akman, None; M. Cantasdemir, None; F. Numan, None; H. Tuzun, None; H. Yazici, None.

To cite this abstract in AMA style:

Hamuryudan V, Seyahi E, Ugurlu S, Gulsen F, Akman C, Cantasdemir M, Numan F, Tuzun H, Yazici H. Bronchial Artery Enlargement May Cause Recurring Hemoptysis in Behçet’s Syndrome Patients with Pulmonary Artery Involvement Despite Response to Treatment [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/bronchial-artery-enlargement-may-cause-recurring-hemoptysis-in-behcets-syndrome-patients-with-pulmonary-artery-involvement-despite-response-to-treatment/. Accessed .

« Back to 2015 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/bronchial-artery-enlargement-may-cause-recurring-hemoptysis-in-behcets-syndrome-patients-with-pulmonary-artery-involvement-despite-response-to-treatment/