Article, Pulmonology

An unusual complication of Behcet disease: spontaneous bilateral pneumothorax

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American Journal of Emergency Medicine

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An unusual complication of Behcet disease: spontaneous bilateral pneumothorax?

Abstract

Behcet disease is a multisystem vasculitis of unknown etiology, and pulmonary involvement is unusual. Concurrent bilateral pneumothorax is also seen rarely. To the authors’ knowledge, this is the first reported case of Behcet disease with concurrent bilateral pneumothorax in the literature.

An 18-year-old male patient was admitted to the emergency depart- ment with chest pain and dyspnea with exercise. He had no trauma history. Chest x-ray revealed bilateral partial pneumothorax, and it was also confirmed at thorax computed tomography; no any bullae were detected. Chest drains were inserted both sides with 28F chest tube. At history, he had Behcet disease for 1 year and had been on colchicine treat- ment. Although he was on treatment, he had oral aphthous lesions, uveitis, and genital lesions. During follow-up, both lungs expanded, and air leak stopped. Right-sided chest drain was removed at the fourth day; left-sided drain was removed at the seventh day of follow-up. After removal of the drains, no any complication arose.

To our knowledge, there are no bilateral Spontaneous pneumothorax cases reported due to Behcet disease in the literature. Some rare cases had been reported in Wegener granulomatosis. However, pulmonary abnormalities at Behcet disease are usually seen as loss of lung volume, indistinct nodular or reticular shadows, and well-defined opacities; but most of them have no histopathologic correlation. Pathologic findings are pulmonary infarction, hemorrhage, and organizing pneumonias. Other Pulmonary findings include pleural effusion, pulmonary arteritis or venulitis, bronchial stenosis, abscess, and fibrosis. We submit this case because of that; spontaneous pneumothorax had not been reported yet in Behcet disease.

Behcet disease is a chronic multisystem vasculitis, and lung involve- ment is rare. Pulmonary involvement reportedly emerges as the decrease of pulmonary ventilation and nonspecific nodular and reticular shadows [1]. Among rare findings are pleural effusion, pulmonary arteritis and venulitis, bronchial stenosis, abscess, and fibrosis [2]. Spontaneous bilateral pneumothorax is a rare case in the literature. This is the first spontaneous bilateral pneumothorax case induced by Behcet disease in the English literature and presented due to rare complications.

An 18-year-old male patient applied to the emergency service with complaints of chest pain and dyspnea. There was no history of trauma in anamnesis or of severe exercise. It was detected that he was diagnosed with Behcet disease 1 year ago and he was undergoing a colchicine therapy. It turned out that the patient had oral aphthous lesions, uveitis,

? This case report has been presented in 18th Annual Congress of Turkish Thoracic Society

“Toraks 2015” during April 1 to 5, 2015, as an oral presentation at Antalya, Turkey.

and intermittent genital aphthae. The physical examination pointed to forced respiration, use of accessory respiratory muscles, a slight increase in sonority during percussion, and a slight decrease in bilateral respiratory sounds as a result of auscultation. The chest X-ray radiogra- phy pointed to bilateral partial pneumothorax. thoracic computed tomography confirmed the bilateral pneumothorax. Parenchyma proved no bullous formation (Fig. 1). An underwater seal chest drainage was administered at the emergency department through a 28F chest tube for both hemithoraxes (Fig. 2).

During the follow-up of the patient, both lungs expanded, and the tubes were taken out without any complication.

Behcet disease is characterized by recurrent oral aphthous lesions, and systemic findings were thought to be induced by vasculitis. Morbidity causes include ocular involvement, vascular involvement, central nervous system involvement, and vascular thrombosis complications. Vascular involvement is a vasculitis that can affect both small and large vessels as well as veins and arteries. Pulmonary involvement is seen rarely [1-3].

According to Cochrane review results, the effectiveness of classic therapies such as colchicine, cyclophosphamide, and glucocorticoids is dubious for Behcet diseases [4].

As far as it is known in the English literature, no case of spontaneous bilateral pneumothorax has been reported for Behcet disease. Although they are rare, cases of Wegener granulomatosis reported pneumothorax [5]. Because both diseases are different types of vasculitis, pneumothorax is quite salient for both diseases as a rare complication.

Most of pneumothorax cases that emerge in Wegener granulomatosis offer cavitary or nodular lesions in pulmonary parenchyme, whereas there are rare cases where pneumothorax emerges without any pathologic and radiologic finding [5]. Although pulmonary parenchymal changes are not unique in Behcet disease, generally, they are reported as unspecific nodular and reticular shadows, focal or diffuse alveolar infiltrates, linear shadows, atelectasis, and parenchymal hypovascularized areas [1].

Abadoglu et al [6] presented a case of Behcet disease with right hydropneumothorax. This case was a 40-year-old male patient with a 7-year diagnosis of Behcet disease on colchicine therapy, when pneumothorax first emerged. That was a case with suspicion of uncon- trolled disease and multiple-system involvement due to uveitis, oral aphthous lesions, and intermittent genital lesions [6]. Our case was a younger patient diagnosed with Behcet disease for 1 year and provided with colchicine therapy. The patient had uveitis, oral aphthous lesions, and intermittent genital lesions. In the light of the aforementioned findings, this patient can be regarded as a case with an uncontrolled disease and multiple-system involvement like other case.

The fact that a rare complication like pneumothorax for Behcet disease emerged at the time of colchicine therapy both in 1 single case reported in the literature [6] and our case as well suggests that the

0735-6757/(C) 2016

Image of Fig. 1 Image of Fig. 2

Fig. 1. Bilateral pneumothorax at thoracic computed tomography.

Fig. 2. Bilateral Chest tubes at lung x-ray.

colchicine therapy for Behcet disease may be a risk factor for pneumo- thorax. Although the literature offers no precise information about it, rare case presentations [7] point to the relation between the administra- tion of colchicine and pneumothorax.

Ozer et al [8] reported in their studies over the review of high resolution computed tomography findings for Behcet patients that the air trapping had been detected more commonly than expected. For Behcet disease, the mechanism for air trapping might be associated with bronchospasm, inflammation, and fibrosis induced by a decrease in blood flow. It might be considered that an increasing risk is likely to be in question in terms of pneumothorax.

In addition to all the findings, the spontaneous bilateral pneumotho- rax accounts for approximately 1.3% of all spontaneous pneumothorax cases [5].

The fact that our case had spontaneous bilateral pneumothorax points to the pulmonary involvement, although there is no other coexisting pulmonary radiologic finding.

Although pneumothorax is not common for Behcet disease, the

spontaneous pneumothorax should be kept in mind at the ED as a differential diagnosis for Behcet patients with the administration of colchicine as well as sudden-onset chest pain and dyspnea that could not be explained for any other reason.

Omer Cenap Gulyuz, MD Hitit University Training and Research Hospital, Corum, Turkey E-mail address: [email protected]

Sertac Arslan, MD Pulmonology Department, Medical Faculty, Hitit University, Corum, Turkey Corresponding author. HITIT Universitesi TIP Fakultesi, Egitim Arastirma Hastanesi, Gogus Hastaliklari Poliklinigi, Corum, Turkey

Tel.: +90 532 3666955; fax: +90 364 2230323

E-mail address: [email protected]

http://dx.doi.org/10.1016/j.ajem.2015.11.049

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