Introduction

Spontaneous pneumothorax is a rare complication in patients with systemic sclerosis (SSc), most likely secondary to subpleural cyst rupture(1). Our patient is the second reported case caused by rupture of a giant bullae(2). Spontaneous pneumothorax in SSc, caused by the rupture of a giant bullae and treated by bullae exicion operation, as our patient, has not been reported previously.

Systemic sclerosis is a multisystem disorder of unknown cause characterized by fibrosis of the skin, blood vessels, and visceral organs. It can be had features of the CREST syndrome, an acronym standing for calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactytly, and telangiectasia. Women overall are affected approximately three times as often as men. With pulmonary involvement, diffuse intersititial fibrosis, thickening of the alveolar membrane, and peribronchial fibrosis are observed. Rupture of septa produces small cysts and areas of bullous emphysema(3).

Case Report

A 23-year-old woman admitted to our hospital with an 8-year history of SSc manifested as sclerodermatous skin changes, Raynaud’s phenomenon, hand finger movement difficulty, pitting ulcers on hands, dysphagia, and antinuclear antibodies. There was no telangiectasia on face and extremites. Her complaints were begun 8 years before, but aggrevated for a year. She had treated with systemic oral colchicine, and local prednisone, with no improvement in her symptoms. There was no history of smoking. She was examinated biochemically and radiologically. Chest computed tomography (CT) revealed; 9x11 cm diametered giant bullae originated lower lobe’s anterobasal segment of the right lung, and bilaterally subpleural paraseptal bullous emphysema (figure 1A). An echocardiography revealed normal cardiac function with normal pulmonary arteriel pressure. Laboratory studies at that time notable for; anti-centromer antibody ( positive), anti-nuclear antibody (>1/640, positive), anti-Scl-70 (negative), anti-SSA(negative), anti-SSB (negative), anti-SM (negative), anti-PM-Scl (negative), anti-dsDNA (negative), anti-histones (negative), and erythrocyte sedimentation rate of 26 mm/hour. Pulmonary function test definited rescrictive pattern. Abdominopelvic Ultrasonography was normal. Bullae exicion operation was planned but patient refused it.

On the 15th day of hospital staying, she presented to our thoracic surgery clinic complaining of acute onset of shortness of breath, dry cough, and chest pain without any trauma. Her first step physical examination revealed tachycardia (144/minute), tachypnea (34/minute), decrease of right-sided breath sounds, sclerodactyly, and sclerodermatous skin changes on face and extremites (figure 1B). A posteroanterior plain chest radiography showed right-sided tension-pneumothorax (figure 2A). It was not a catamenial pneumothorax, according to the patient verbal investigation. A chest tube was inserted with subsequent expansion failure, air leakage into chest tube and symptoms were decreased. Air leakage continued for 7 days and operation was planned because of the prolonged air leakage, and expansion failure (figure 2B). Giant bullae exicion was performed with lineer staplers via right-sided thoracotomy. There was no air laekage by the over pressure flash breathing and reexpansion seen in the operation, therefore pleurodesis was not be done. Chest tube was removed 4th day in post-operation period, and patient was discharged at the 6 th day. She had no recurrent pneumothorax in the 6th month’s control.

Figure 1

Figure 1A: Chest CT showing giant bullae originated from lower lobe

Figure 2

Figure 1B: Picture showing skin changes on face and extremites

Figure 3

Figure 2A: Posteroanterior chest radiography showing right-sided tension pneumothorax

Figure 4

Figure 2B: Chest CT showing expansion failure

Discussion

Exertional dyspnea and dry cough are the most common presenting symptoms in patients with SSc who develop pulmonary involvement. The most frequent and serious pulmonary complications of scleroderma are pulmonary hypertension (PH) and intersitisyel lung disease (ILD). Both are present in the vast majority of patients with scleroderma at autopsy. Clinically significant PH affects 15–20% of scleroderma patients, and results in dyspnea, impaired exercise tolerance, and a high risk of death^4-5.

The pathophysiology of pulmonary fibrosis in SSc is related to activation of T cells, macrophages, and endothelial cells, with subsequent cytokine release that stimulates fibroblast chemotaxis and proliferation⁶. Pulmonary involvement is the major cause of the death in SSc. Although spontaneous pneumothorax is rare in SSc, it is not an uncommon manifestation in other conditions such as chronic obstructive lung disease, cystic fibrosis, tuberculosis, pneumocystis carini pneumonia, Marfan’s syndrome, and chronic intersititial disease with cyst formation⁷.

Spontaneous pneumothorax is a rare reported pulmonary manifestation of SSc, with only a few reported cases in the English-language literature. Most of them are caused rupture of subpleural cysts. All 6 patients received chest tube inserting, however 3 later required pleurodesis and 2 required partial lobectomy^1-10. According to Milanez JR, et al, chest tube insertion and eventual talc pleurodesis had been shown to be a successfull treatment of recurrent spontaneous pneumothorax in SSc¹¹.

Our patient’s spontaneous pneumothorax was caused by rupture of a giant bullae, and there was prolonged air leakage into chest tube, and needed thorotomy operation for bullae exicion. Also there was no radiological evidence for pulmonary fibrosis in CT scanning. Chest CT is the prefered scanning because; CT scanning is a sensitive diagnostic tool because it clearly shows the full extent and anatomical severity of bullous disease, features often not discernible on simple posteroanterior and lateral radiographs. CT scanning may also help in differentiating a pneumothorax from a large emphysematous bulla¹². There have been no respiratory problem in 6^th month’s post operative period.

Conclusion

Physicians should take into consideration SSc patients with acute onset of shortness of breath, dry cough, and chest pain for spontaneous pneumothorax. Physical and radiological examinations have to be obtained for diagnosing. Chest tube inserting is the first step treatment fort he first episode spontaneous pneumothorax in SSc.

Prolonged air leakage (continued more than 7 days), high incidance of recurrence, and infection of the bullae are the operation indications for surgery in bullous diseases of the lung.

Pleurodesis may not the best choiced treatment for operative bullous diseases of the lung in SSc, it should be done in recurrenced cases.