Background/Purpose: The mainstay treatment of pulmonary artery involvement (PAI) in Behcet’s syndrome (BS) is immunosuppresion and corticosteroids (1). The role of surgical intervention in the management of PAI can be questionable in many cases considering the severe outcome of our previous attempts (2-3). For the past few years we observed that there are indeed several conditions associated with PAI which require surgical operations.   In this study, we described disease characteristics, management and outcome of a group of BS patients who underwent surgical procedures for complications due to PAI.

Methods: There were 9 patients with BS (8 M/1 F) who underwent surgery for PAI from 2003 to 2016 at the Department of Thoracic and Cardiovascular Surgery, Cerrahpaþa Medical Faculty. The medical records, outpatient charts, radiological and pathological studies of these patients were reviewed retrospectively.

Results: The mean age of the patients was 24.8± 7.5 years (range: 12-35). The mean duration of the disease at the time of the surgery was 4.3±3.8 years. The main symptom was haemoptysis. The most common surgery type was lobectomy which was done in 6 patients, followed by decortications in 3. The reason for the surgical procedures were variable as shown in Table. It was giant pulmonary arterial aneurysms refractory to the medical treatment in 4 patients (patients no.  1, 2, 3 and 4), hydropneumothorax due to cavitary lesions in two (patients no. 5 and 6), pleural effusions refractory to the medical treatment in one (patient no. 7), bronchopleural fistula after embolization in one (patient no. 8) and bronchiectasis in another (patient no. 9).  All patients received cyclophosphamide treatment with different total doses. Seven patients (patient no. 2, 4, 5, 6, 7, 8 and 9) are still alive and are being followed in our clinic for a median of 7.5 years (IQR:1.5-11).  Patient no. 1 had died because of hepatic failure due to Budd-Chiari syndrome after 1 year from the surgery and patient no. 3 had died because of massive haemoptysis within 2 months of lobectomy. 

Conclusion: We now think that lobectomies in patients with giant aneurysms refractory to medical treatment can be done with successful results. Also, complications such as refractory pleural effusions, bronchiectasies and broncopleural fistula can be managed with surgical interventions successfully in patients with PAI. 

References:

1-      Seyahi E et al. Medicine (Baltimore). 2012.

2-      Tüzün H et al. Ann Thorac Surg. 1996.

3-      Hamuryudan V et al. Br J Rheumatol. 1994. 

Table. Main symptom and type of surgical intervention in 9 patients