Asian Cardiovasc Thorac Ann 2002;10:277-279
© 2002 Asia Publishing EXchange Pte Ltd
Wegener’s Granulomatosis Presenting as Necrosis of the Left Mainstem Bronchus
Paul Schneider, MD3,
Jörn Gröne, MD,
Jürgen Braun, MD1,
Alejandra Perez-Canto, MD2,
Heinz J Buhr, MD
Department of General, Vascular and Thoracic Surgery
1 Department of Internal Medicine
2 Department of Pathology Benjamin Franklin Medical Center Freie Universit t Berlin Berlin, Germany
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For reprint information contact: Paul Schneider, MD Tel: 49 30 8445 2543 Fax: 49 30 8445 2740 email: p.schneider{at}ukbf.fu-berlin.de Department of General, Vascular and Thoracic Surgery, Benjamin Franklin Medical Center, Freie Universitt Berlin, Hindenburgdamm 30, Berlin D-12200, Germany.
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ABSTRACT
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A patient with pansinusitis, nasal septum necrosis, and saddle nose deformity showed necrosis of the left mainstem, upper, and lower bronchi, with complete loss of left lung perfusion and ventilation. Pneumonectomy was performed. Histological findings showed extensive necrotizing and granulomatous bronchial inflammation with vasculitis of the bronchial arteries and the pulmonary vein. Wegener’s granulomatosis was diagnosed, despite a negative cytoplasmic pattern of antineutrophil cytoplasmic antibodies and the lack of renal involvement.
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INTRODUCTION
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Wegener’s granulomatosis (WG) is an uncommon systemic disease characterized by necrotizing granuloma-tous vasculitis of the upper and lower respiratory tracts and by glomerulonephritis. In addition, variable degrees of disseminated vasculitis involving both small arteries and veins may occur. We present an exceptional case of WG with necrosis of the left mainstem bronchus.
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CASE REPORT
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A 58-year-old man presented with cough and progressive dyspnea on exertion. He noted a loss of 10 kg in 2 months, weakness, and night sweats. He had a 1-year history of recurrent epistaxis and subtotal necrosis of the nasal septum. He later developed clavus. The patient stopped smoking 5 years ago after consuming a packet a day for 20 years. He had no history of tuberculosis.
On examination, the patient had a saddle nose deformity and inflammation of the nasal mucosa. The nasal septum showed near transmural perforation and necrosis. Pulmonary examination revealed severe respiratory obstruction with a loss of breathing sound over the left hemithorax. Besides sinus tachycardia (126 beatsámin-1), cardiovascular examination revealed no further pathological findings. Neurological examination was normal. There was no synovitis or deformity of the joints.
The chest radiograph showed no abnormalities. A high-resolution computed tomographic scan (Figure 1
) revealed high bronchial narrowing in the left mainstem bronchus and a 1.5-cm hilar mass infiltrating this bronchus and extending into the upper and lower lobe bronchi. Quantitative scintiscan showed absence of perfusion and ventilation of the left lung. A severe concentric stenosis of the left mainstem bronchus was found at bronchoscopy 3 cm distal to the carina. No inflammation or endoluminal tumor was seen. Biopsies showed a normal mucosa. Magnetic resonance imaging of the cranial bones revealed obstruction of the nasal cavity and paranasal sinuses. Abnormal laboratory findings included anemia (hemoglobin, 10.9 gádL-1) and leukocytosis (13.9 nL-1). The platelet count was 497 nL-1. C-reactive protein was elevated (167 mgáL-1), but creatinine, urea, and electrolyte levels were normal. Urinalysis was negative for protein and blood. The following were normal: cytoplasmic and perinuclear patterns of antineutrophil cytoplasmic antibody (cANCA and pANCA) titers (using enzyme-linked immunosorbent assay and indirect immunofluorescence); antinuclear, antimitochondrial, and cardiolipin antibody titers; C3c; and the tumor markers carcinoembryonic antigen, neuron-specific enolase, and cytokeratin-19 fragment. C4 was slightly elevated (0.488 gáL-1).
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Figure 1. Computed tomographic scan of the chest showing severe narrowing of the left mainstem, upper, and lower bronchi with thickening of the bronchial wall. No enlarged lymph nodes in the mediastinal or hilar area and no pulmonary infiltrates were found.
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The patient underwent diagnostic left thoracotomy to exclude bronchogenic cancer narrowing the left mainstem bronchus. This procedure revealed peribronchial inflammation without neoplastic cells. Without a diagnosis of malignancy, resection was not carried out. Post-operatively, a flexible 4.9-mm bronchoscope was able to bridge the stenosis and demonstrated mucosal destruction involving both upper and lower lobe bronchi. Pieces of cartilage protruded into the bronchial lumen with subsequent obstruction. The patient was reexplored and underwent pneumonectomy. Intraoperatively, the plane between the bronchus and the left pulmonary artery was seen to be obliterated by bronchial cartilage destruction (Figure 2). After surgery, the patient developed progressive left external ophthalmoplegia.
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Figure 2. Operative specimen of the left lung with incised mainstem bronchus showing destruction of the bronchial wall. Cartilage (arrows) protrudes into the lumen (L), and the pulmonary artery wall forms the bronchial wall.
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Histological examination showed nonspecific ulcerating inflammation with granulated tissue in the nasal septum specimen. Vasculitis and granulomas were not seen. The left mainstem bronchus in the pneumonectomy specimen revealed extensive necrotizing and granulomatous inflammation, which almost completely destroyed the wall, including the cartilage. There was also vasculitis of the bronchial arteries. The necrotizing inflammation had spread to the proximal parts of the left upper and lower lobe bronchi. Inflammatory infiltrates were also found in the wall of the pulmonary vein, which showed a small parietal thrombus. The pulmonary parenchyma and its vessels were entirely devoid of inflammation.
Intravenous immunosuppressive therapy with 800 mg cyclophosphamide and 250 mg prednisolone daily was initiated 2 weeks postoperatively. Prednisolone was subsequently reduced to 60 mg/day. At the 6-month follow-up, the patient’s renal function was stable and C-reactive protein had decreased.
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DISCUSSION
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The differential diagnosis of tracheobronchial stenosis includes neoplasm, infection (e.g., tuberculosis), trauma, extrinsic compression, and immunological disease (e.g., WG). Pulmonary involvement characterized clinically by diffuse infiltrates or nodules on the chest radiograph is common in patients with WG. Stenosis of the subglottis and proximal trachea is seen in 16% of patients during the course of the disease.1 However, stenosis of the distal trachea and one of the mainstem bronchi is a rare initial manifestation of WG.2 In contrast to the good response to conventional treatment with steroids and oral cyclophosphamide of pulmonary involvement in WG, medical treatment of severe bronchial stenosis due to destruction of the bronchial wall is ineffective. Metallic stents have been used to treat benign airway obstruction of various causes. Anecdotal reports suggest that intraluminal stenting in case of severe bronchial stenosis due to inflammatory lesions is feasible and efficient. However, significant complications can occur, including stent migration with airway erosion.2,3
The diagnosis of WG is based on the classical clinical triad: granulomatous inflammation with involvement of the upper and lower airways, glomerulonephritis, and systemic vasculitis involving the small arteries, veins, and capillaries. The diagnosis is confirmed by histological detection of vasculitis, granulomatous inflammation, and necrosis. The presence of cANCA has a nearly 95% specificity for WG, and the titer correlates well with the disease activity;4 while pANCA is usually found in only 5% of WG cases. Relapsing polychondritis also presents with narrowing of the airways, but the airway obstruction is commonly diffuse, involving the upper airways.5
Neurological findings are uncommon in WG: they are seen in 1% of the patients at initial presentation and develop in another 20%. External ophthalmoplegia was detected in only 16 of 324 patients in one study, and tissue ischemia due to vasculitis of the vasa nervorum was discussed as a mechanism of neurological dysfunction.6
Our patient initially presented with stenosis of the left mainstem bronchus and complete loss of perfusion and ventilation of the left lung with subsequent impairment of pulmonary function. Diagnostic thoracotomy revealed benign inflammation involving the mainstem, upper, and lower bronchi. The nonfunctioning lung was resected. Intraluminal stenting7 of the bronchial stenosis was ruled out because of the risk of bleeding and narrowing of the distal airway.
Since our patient had a history of recurrent epistaxis with mucosal inflammation and nasal septum necrosis, WG was included in the differential diagnosis. However, cANCA and pANCA were negative, and the histological findings in the nasal septum were not specific. Renal involvement such as rapidly progressive glomerulo-nephritis was not present, nor were there other manifesta-tions of WG. Despite these findings and in view of the presence of necrotizing and granulomatous inflammation of the bronchi and vasculitis of the bronchial arteries and the pulmonary vein, the diagnosis of WG seems to be justified according to the Chapel Hill nomenclature.8
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Acknowledgments
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The authors thank Dr. W L Gross for his laboratory support.
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REFERENCES
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- Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med
1992;116:488–98.
- Daum TE, Specks U, Colby TV, Edell ES, Brutinel MW, Prakash UB, et al. Tracheobronchial involvement in Wegener’s granulomatosis. Am J Respir Crit Care Med
1995;151(Pt 1):522–6.[Abstract]
- Lehman JD, Gordon RL, Kerlan RK Jr, Laberge JM, Wilson MW, Golden JA, et al. Expandable metallic stents in benign tracheobronchial obstruction. J Thorac Imaging
1998;13:105–15.[Medline]
- Nolle B, Specks U, Ludemann J, Rohrbach MS, DeRemee RA, Gross WL. Anticytoplasmic autoantibodies: their immunodiagnostic value in Wegener granulomatosis. Ann Intern Med
1989;111:28–40.
- Sarodia BD, Dasgupta A, Mehta AC. Management of airway manifestations of relapsing polychondritis: case reports and review of literature. Chest
1999;116:1669–75.[Abstract/Free Full Text]
- Nishino H, Rubino FA, DeRemee RA, Swanson JW, Parisi JE. Neurological involvement in Wegener’s granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol
1993;33:4–9.[Medline]
- Herridge MS, Pearson FG, Downey GP. Subglottic stenosis complicating Wegener’s granulomatosis: surgical repair as a viable treatment option. J Thorac Cardiovasc Surg
1996;111:961–6.[Abstract/Free Full Text]
- Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum
1994;37:187–92.[Medline]
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ABSTRACT
INTRODUCTION
