INTRODUCTION: SLE is a chronic inflammatory disease that can affect any organ in the body. It is an autoimmune disorder that involves multi-system with production of antibodies against self. The specific cause is unknown, but genetic, racial, hormonal and environmental factors may be implicated. Among pulmonary manifestations of the disease eg: pleurisy, pleural effusion, spontaneous pneumothorax is not well recognized manifestation. In this case report we will describe a 27 years old female with recurrent bilateral pneumothorax discovered to have SLE who is treated surgically.
CASE PRESENTATION: A 27 year old female presented to our hospital with left side chest pain, shortness of breath and cough for one day. No symptoms related to other system were involved and no family history of similar condition. Routine investigations and CXR were requested and the diagnosis of left sided pneumothorax was established. A chest tube was inserted, the patient improved and discharged home accordingly. Three months later she began to feel right sided chest pain of the same nature, again CXR requested and a diagnosis of bilateral pneumothorax was established. A CT scan was requested and it was found that there were left sided pneumothorax and moderate size pneumothorax on the right side with multiple bullae and pleural blebs. As a routine investigation during hospital stay, urine general was obtained to find that there is hematuria and protienuria. A blood sample was taken to detect anti-DNA antibodies and it was positive to be diagnosed as having SLE. Immunosuppressive drugs were prescribed. Surgery was done through median sternotomy and there were numerous blebs and a moderate size bulla on the lingua of the lung. Bilateral pleurectomy was done ,with pleurodesis,large bulla has been resected using GIA disposable stapler.Postoperative course was steady and unremarkable and the patient improved.
DISCUSSIONS: Primary spontaneous pneumothorax occurs in otherwise healthy people with no history of chest trauma. It commonly affects tall, thin males who are usually smokers between the ages of 20 and 40.Secondary spontaneous pneumothorax occurs in people with already affected lungs especially emphesema. Other causes are: TB, pneumonia, asthma, cystic fibrosis, lung cancer and interstitial lung disease. Pneumothorax could be familial. It has been suggested that familial spontaneous pneumothorax may be caused by dominantly inherited connective tissue disorders (1) (2). SLE which is a non- organ specific connective tissue disease involves many organs e.g.: kidney, skin and brain. The lungs are , yet, another organs that may be involved. A number of pulmonary complications are associated with SLE e.g.: pleurisy, pneumonitis, pulmonary hemorrhage, pulmonary hypertension and pneumothorax have been reported in patients with SLE (3). It has been reported that pleuropulmonary manifestations are seen in approximately 50–70% of adult patients with SLE (4) (5). In our case the patient hasn't got any history suggestive of the disease apart from hematuria and protienuria. There was no history of any skin or musculoskeletal disorder. The only pulmonary manifestation that she got is bilateral spontaneous pneumothorax due to bullous disease. The association of SLE and spontaneous pneumothorax has not been described extensively in the literature. There are some cases reported of similar condition (6) that state this association and the relationship between spontaneous pneumothorax and connective tissue disease (7).
CONCLUSION: Any patient with Primary Spontaneous Pneumothorax Connective Tissue Disease, including SLE, must be included in the differential diagnosis and should be investigated and Spontaneous Pneumothorax should not be considered as primary.
DISCLOSURE: Mohamed Elamin Ahmed, No Financial Disclosure Information; No Product/Research Disclosure Information