Wegener's granulomatosis

Spontaneous pneumothorax in Wegener granulomatosis (WG) is uncommon. Three cases of pneumothorax that occurred early in the course of this vasculitis are reported. In the first patient, the disorder was disclosed by a pyopneumothorax. In the second patient, a rupture of the subpleural cavitary nodule into the pleural space was observed. In the third patient, the pneumothorax was discovered at the same time as a pulmonary hemorrhage. The three patients improved with immunosuppressive therapy. In WG, the pulmonary nodules are predominantly in the subpleural location, which entails the risk of pneumothorax and therefore require particular attention.

Key words: pyopneumothorax; spontaneous pneumothorax; vasculitis; Wegener granulomatosis

Abbreviations: ANCA = antineutrophil cytoplasmic antibody; CRP = C-reactive protein; PR3 = proteinase 3; WG = Wegener granulomatosis

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The lung, together with the upper airways and the kidneys, is one of the main targets of Wegener granulomatosis (WG). The clinical features of pulmonary involvement are nonspecific and comprise cough, dyspnea, hemoptysis, and bloody sputum. In approximately one third of cases, chest radiographs show abnormalities that are asymptomatic. Usual radiologic features include single or multiple nodules that frequently cavitate and areas of consolidation on CT scan. There are few documented reports of spontaneous pneumothorax.

CASE REPORTS

Case 1 is a 46-year-old man admitted for a right lung cavitary lesion. For 3 months, he had had hemoptysis. C-reactive protein (CBP) level was 80 mg/L. Eosinophil count was 2,000/[micro]L. Plasma creatinine level was normal. Antineutrophil cytoplasmic antibodies (ANCAs) determined by indirect immunofluorescence were positive at a titer > 1:500 with a cytoplasmic pattern and proteinase 3 (PR3) specificity. CT scan of the lungs showed a very bulky cavitary nodule in the right lung. Bronchoscopy findings were normal. Two days after bronchoscopy, the patient became febrile. Antibiotic therapy and prednisone were begun. CT scan of the lung showed pyopneumothorax (Fig 1). A right upper pulmonary lobectomy was rapidly performed, and pathologic examination revealed lesions typical of WG. IV cyclophosphamide was started. One year later, the patient is alive and well.

[FIGURE 1 OMITTED]

Case 2 is a 25-year-old man who presented with bloodstained rhinitis, right-side deafness, and otalgia. Renal function was normal. Urinalysis revealed microscopic hematuria. The C-ANCA titer was 1:160 with PR3 specificity. Chest radiography showed a tight subpleural cavitary nodule and another nodule located at the left hila. CT scan of sinus showed thickened turbinate and a left maxillary polyp. Biopsy of nasal septum revealed a necrotizing granulomatous vasculitis. Combined therapy with corticosteroid and monthly cyclophosphamide pulse was initiated. Six weeks later, the patient complained of dyspnea. Chest CT scan showed a pneumothorax resulting from rupture of the subpleural nodule, which had collapsed into the pleural space. The treatment was pursued and the patient improved.

Case 3 is a 70-year-old man admitted to the ICU for ARDS. In the ICU, assisted ventilation was required. Edema of the epiglottis and tracheal stenosis were diagnosed during tracheal intubation. Chest radiography showed reticulonodular opacities. The patient improved with IV antibiotic therapy and prednisolone. After 1 week, he was extubated. A lung radiograph showed resolution of the pulmonary opacity. Following initial improvement, the reticulonodular images subsequently worsened and bilateral pulmonary infiltrates and right pneumothorax developed. BAL showed features of alveolar hemorrhage. Renal function was normal. C-ANCAs were positive at 1:160. Cyclophosphamide was added to the corticosteroid therapy. The pneumothorax resolved without specific treatment.

DISCUSSION

In WG, pulmonary nodules represent > 70% of lung lesions. They are predominantly subpleural and are cavitated in nearly 50% of cases. (1) Pleural involvement in WG is described in < 10% of cases and consists of effusion and pleura] thickening. The occurrence of pneumothorax is a rare complication. A literature search retrieved a few cases in large patient series and six single case reports (2-6); surprisingly, all were male patients (Table 1). Pneumothorax occurs early in the course of active WG. In one exceptional case, (3) pneumothorax was reported as the sole pulmonary manifestation of WG; actually, the patient had hydropneumothorax and chest CT scan was not available. Several pathogenic mechanisms have been suggested to explain the formation of pneumothorax in WG, and our three observations provide a representative illustration of the putative causes.

In our first case, WG was disclosed by a pyopneumothorax. The patient had undergone bronchoscopy 48 h before the occurrence of pneumothorax. However, bronchial biopsy was not performed, and while bronchoscopy cannot be ruled out as the cause of his pneumothorax, it is an unlikely candidate. It is more likely that the pyopneumothorax was due to infection of the cavitary nodule. Pyopneumothorax was also seen in two of the six cases from the literature. (5,6) It was a serious complication because it arose during the course of the active disease and required immunosuppressive therapy with both patients receiving high doses of corticosteroid and daily cyclophosphamide. The outcome was unfavorable, and the two patients died of infection and WG, as indicated by the active vasculitis found at the postmortem examination. In such circumstances, and despite the risk of infection, immunosuppressive therapy should be associated with the antibiotic treatment.

In case 2, pneumothorax occurred as the result of the rupture of a cavitary nodule. The preferential sites of nodules in WG are cortical and subpleural, which favors contact between the nodules and the pleura. Hence, subpleural nodules require particular attention, especially when excavated. In our third patient, pneumothorax was discovered at the same time as pulmonary hemorrhage, an association that has not been described in previous reports. In conclusion, pneumothorax is a rare but potentially severe event in WG that reflects active disease and requires immunosuppressive therapy associated, in pyopneumothorax, with antibiotic therapy.

ACKNOWLEDGMENT: We thank Jeffrey Watts for help with the English version of the article.

Manuscript received September 19, 2004; revision accepted May 16, 2005.

REFERENCES

(1) Courthaliac C, Aumaitre O, Andre M, et al. Apport de la tomodeusitometrie dans le suivi evolutif des lesions pleuropulmonaires de la granulomatose de Wegener. Rev Med Interne 1999; 20:571-578

(2) Ogawa M, Azemoto R, Makino Y, et al. Pneumothorax in a patient with Wegener's granulomatosis during treatment with immunosuppressive agents. J Intern Med 1991; 229:189-192

(3) Epstein DM, Gefter WB, Miller WT, et al. Spontaneous pneumothorax: an uncommon manifestation of Wegener granulomatosis. Radiology 1980; 135:327-328

(4) Bulbul Y, Ozlu T, Oztuna F. Wegener's granulomatosis with parotid gland involvement and pneumothorax. Med Princ Pract 2003; 12:133-137

(5) Jaspan T, Davison AM, Walker WC. Spontaneous pneumothorax in Wegener's granulomatosis. Thorax 1982; 37:774-775

(6) Wolffenbuttel BH, Weber RF, Kho GS. Pyopneumothorax: a rare complication of Wegener's granulomatosis. Eur J Respir Dis 1985; 67:223-227

Isabelle Delevaux, MD; Mehdi Khellaf, MD; Marc Andre, MD; Jean-Luc Michel, MD; Jean Charles Piette, MD; and Olivier Aumaitre, MD

* From the Department of Internal Medicine (Drs. Delevaux, Andre, and Aumaitre) and Department of Radiology (Dr. Michel), CHU de Clermont-Ferrand, Hopital G. Montpied, Clermont-Ferrand; Department of Internal Medicine (Dr. Khellaf), Hopital Henri Mondor, Creteil; and Department of Internal Medicine (Dr. Piette), CHU Pitie-Salpetriere, Paris, France.

Correspondence to: Isabelle Delevaux, MD, Service de Medecine Interne, CHU Clermont-Ferrand, Hopital G Montpied, 58 rue Montalembert, 63003 Clermont-Ferrand France; e-mail: idelevaux@chu-clermontferrand.fr

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