Ann Thorac Surg 2007;84:288-290
© 2007 The Society of Thoracic Surgeons
Case Reports
Unusual Pathogenesis of Spontaneous Pneumothorax Secondary to Wegener’s Granulomatosis
Erica Storelli, MDa,
Christian Casali, MDa,
Pamela Natali, MDa,
Giulio Rossi, MDb,
Uliano Morandi, MDa,*
a Department of General Surgery and Surgical Specialties, Division of Thoracic Surgery, University of Modena and Reggio Emilia, Modena, Italy
b Department of Diagnostic and Laboratory Services and Legal Medicine, Section of Pathologic Anatomy, University of Modena and Reggio Emilia, Modena, Italy
Accepted for publication January 22, 2007.
* Address correspondence to Dr Morandi, Division of Thoracic Surgery, Policlinico di Modena, Largo del Pozzo 71, Modena, 41100, Italy (Email: uliano.morandi{at}unimore.it).
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Abstract
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Spontaneous pneumothorax represents a rare and potentially severe complication of Wegener’s granulomatosis. A 31-year-old man with Wegener’s granulomatosis on immunosuppressive therapy was admitted for a right massive spontaneous pneumothorax. After chest drainage he presented with a prolonged air leak that required a surgical treatment. Histologic findings did not reveal any necrotizing granulomatous vasculitis, but only subpleural fibrous tissue. We hypothesize that pneumothorax could be related to the subpleural fibrous retraction induced by immunosuppressive therapy.
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Introduction
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Wegener’s granulomatosis (WG) is an idiopathic systemic inflammatory disease that primarily affects the upper and lower respiratory tract and the kidneys. The pleuro-pulmonary lesions of WG are generally parenchymal nodules, mostly cortical, with irregular margins that are usually excavated. Less frequently, parenchymal consolidation or pleural involvement can occur. Spontaneous pneumothorax is a very rare complication of WG with only few cases reported in the literature [1–5]. This usually occurs in the active phase of the disease and its prognosis can be potentially severe. Different up-to-date mechanisms have been purposed to explain pathogenesis of spontaneous pneumothorax secondary to WG [1, 2].
A 31-year-old man was admitted to our institute for sudden chest pain associated with respiratory failure. At admission, the arterial oxygen saturation was 65% and the blood pressure was 190/100. The chest roentgenogram showed a massive right pneumothorax with complete collapse of lung parenchyma and a contralateral mediastinal shift (Fig 1). A right chest drain was emergently positioned with the restoration of his vital measurements and a partial re-expansion of the right lung. A diagnosis of Wegener’s granulomatosis was obtained when he was 18 years old with a nasal septum biopsy (Fig 2A). At admission the patient assumed methotrexate and steroids. Large spectrum antibiotic therapy was administered. Antineutrophil cytoplasmatic antibodies, determined by indirect immunofluorescence, were at a titre of 1:160 with a cytoplasmic pattern and a proteinase 3 specificity. The hospital stay was characterized by a prolonged air leak despite chest drain aspiration. After 7 days of refractory air leak the patient underwent a surgical treatment. A pulmonary wedge-resection of the superior right lobe and a partial pleurectomy (from the apex to the fifth intercostal space) through lateral muscle-sparing thoracotomy were performed. At histology the resected lung showed pleural bullae with dense fibrosis and pulmonary fibroelastosis with chronic bronchiolitis without vasculitis, granumolatous inflammation, or neutrophilic abscesses, as one can see instead in active WG (Fig 2B). The postoperative period was uneventful. A postoperative chest roentgenogram after the removal of chest drains showed a complete expansion of the right lung with no signs of pneumothorax and the absence of newly diagnosed parenchymal consolidation related to WG. The patient was discharged on the fifth postoperative day and is currently alive with no signs of recurrent pneumothorax after 3 months.
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Fig 2. (A) Nasal biopsy showing a neutrophilic microabscess consisting of central basophilic necrosis with a collection of neutrophils and multinucleated giant cells at the edge (Hematoxylin and eosin, x200). (B) The lung biopsy showed a dense fibrotic reaction with formations of pleural bullae and dense fibrosis in the underlying pulmonary parenchyma without signs of inflammation (Hematoxylin and eosin, x40 magnification).
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Comment
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Wegener’s granulomatosis is a distinct clinicopathologic entity that primarily affects the respiratory tract and the kidneys. It frequently presents an inflammatory syndrome with positive anti-neutrophil cytoplasmic antibodies and other inflammatory markers. Pulmonary involvement generally presents cavitated nodules mimicking lung cancer. Despite that the diagnosis should be suspected by laboratory values and roentgenogram findings, a definitive diagnosis requires histologic confirmation generally obtained with a nasal biopsy. The histologic aspect of Wegener’s granulomatosis is characterized by necrotizing granulomatous vasculitis.
Pleural lesions are present in only 10% of patients with pulmonary involvement, with pleural effusion, and pleural thickenings being the most frequently encountered lesions. However an episode of pneumothorax in the course of WG is a vary rare complication and only a few cases in literature have been reported [1–5]. Spontaneous pneumothorax often reflects an active disease during the course of high-dose immunosuppressive therapy. Although asymptomatic pneumothorax can be incidentally discovered on a computed tomographic thoracic scan associated with one or more excavated nodules, it is generally symptomatic and can represent a life-threatening clinical condition with a significant mortality as reported in literature [1]. Septic complications, such as pleural empyema, favored by immunosuppressive therapy and a massive hemothorax secondary to the pleural rupture of a cavitated nodule, represent the leading causes of death in these patients. For these reasons the clinical and surgical management of this rare clinical entity is particularly challenging.
Many mechanisms have been suggested to explain the pathogenesis of pneumothorax in WG. The most commonly reported pathogenic mechanism is the rupture of a parenchymal cavitary nodule in the pleural space. Because of the immunosuppressive therapy, the nodule can sometimes be infected and a pyopneumothorax can develop [5]. Other pathogenetic hypotheses refer to a primary development of a granuloma in the pleura or to the presence of a bronchopleural fistula [6].
In our case there were neither histologic evidence of necrotizing granulomatous vasculitis in the resected pulmonary specimen nor pleural granulomatous lesions, whereas the main pathologic findings consisted of a dense pleural fibrosis with bullae and fibroelastosis of the underlying lung parenchyma. As suggested by Travis and colleagues [7], these histologic features could be related to effective immunosuppressive therapy inducing fibrotic resolution of the active inflammation. Then we speculate that the pathogenesis of spontaneous pneumothorax in our case could be related to a fibrous pleural retraction due to iatrogenic effects of chemotherapy.
In conclusion, spontaneous pneumothorax is a very rare complication of WG. When a prolonged air leak occurs after chest drainage, a surgical treatment with partial pleurectomy is indicated and can be performed with low postoperative morbidity. Wide spectrum antibiotic prophylaxis is useful to decrease the risk of postoperative septic complications. Spontaneous pneumothorax can be correlated with several different pathogenic mechanisms, but it is possible that immunosuppressive therapy could induce this complication due to its fibrosing effect on pleuro-pulmonary granulomatous lesions.
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References
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- Wolffenbuttel BH, Webr RF, Kho GS. Pyopneumothorax: a rare complication of Wegener’s granulomatosis Eur J Respir Dis 1985;67:223-227.[Medline]
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- Travis WD, Hoffman GS, Leavitt RY, et al. Surgical pathology of the lung in Wegener’s granulomatosisReview of 87 open lung biopsies from 67 patients. Am J Surg Pathol 1991;15:315-333.[Medline]
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Abstract
Introduction
