British Journal of Radiology (2007) 80, e173-e175
© 2007 British Institute of Radiology
doi: 10.1259/bjr/57686881
Atypical bronchial thickening and ulceration: a rare radiological finding in Wegener's granulomatosis
S Pauls, MD
1
S Krüger, MD
2
T F E Barth, MD
3
H-J Brambs, MD
1 and
M S Juchems, MD
1
1 Department of Diagnostic and Interventional Radiology, 2 Department of Internal Medicine II, 3 Institute of Pathology, University of Ulm, D-89081 Ulm, Germany
Correspondence: Dr Sandra Pauls, Department of Diagnostic and Interventional Radiology, University Hospital Ulm, Robert-Koch-Str. 8, D-89081 Ulm, Germany. E-mail: sandra.pauls{at}uniklinik-ulm.de
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Abstract
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We report the case of a 55-year-old male patient who presented with non-specific pulmonary symptoms (cough, haemoptysis, fever up to 39°C, night sweats and weight loss). After empirical antibiotic therapy prescribed by his primary care physician, the patient showed no improvement in symptoms. Laboratory findings were: elevated C-reactive protein and C-ANCA, leukocytosis and thrombocytosis, and anaemia. Chest radiography showed disseminated nodules bilaterally. On multidetector-row computed tomography (MDCT), the bronchial walls showed a significant thickening and extensive peribronchiolar consolidations. Bronchoscopy revealed diffuse erythema of the tracheobronchial mucosa with diffusely scattered white plaques. Histopathology described a multifocal ulcerative bronchitis with underlying chronic bronchitis. These findings in combination with the laboratory data lead to the diagnosis of Wegener's granulomatosis. Consequently, we started with an immunosuppressive therapy. Chest radiography after 10 days showed marked resolution of the infiltrates. Within 1 month, the patient became asymptomatic.
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Introduction
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Wegener's granulomatosis is a multisystem disease frequently affecting the respiratory tract and the kidneys. Manifestations in other systems, such as the nervous and musculoskeletal systems, are less common, occurring in up to 30% of cases. The peak incidence is between the fourth and sixth decade. Cytoplasmatic antineutrophil cytoplasmatic autoantibodies (C-ANCA) are detectable in about 90% of patients. The diagnosis is often delayed because of its non-specific initial symptoms.
In the present case report, we report on a patient who presented to the medical outpatient clinic with non-specific pulmonary symptoms after empirical antibiotic therapy prescribed by his primary care physician showed no improvement in symptoms.
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Clinical and laboratory findings
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The patient was a 55-year-old white male presenting with productive cough, haemoptysis, fever up to 39°C, night sweats and weight loss of approximately 5 kg within the last 4 weeks. In addition, he reported significant fatigue. His primary care physician had treated the persistent flu-like symptoms with antibiotics for 2 weeks, but the patient reported no improvement in his complaints. Medical history was unremarkable. The family history revealed bronchial carcinoma in his father and uncle. Clinical examination was unremarkable except for mild rales of the basal parts of both lungs. The examination of the skin was unremarkable. The results of biochemical screening tests showed a mild elevation of creatinine (138 µmol l–1), mild anaemia (haemoglobin 12.3 g dl–1), mild thrombocytosis (522 G l–1) and massively elevated C-reactive protein (240 mg l–1). C-ANCA (PR3) was significantly elevated (73 U l–1, norm 0–10 U l–1). The following tests were unremarkable: Ig analysis, rheumatoid factor, perinuclear cytoplasmatic autoantibody, antinuclear and anti-DNA antibodies. The results of serological screening tests for viruses, Mycoplasma, Legionella, Chlamydia, Aspergillus and Candida were negative. Repeated sputum cultures for aerobic and anaerobic organisms, fungi and mycobacterial organisms yielded no growth, tine testing was negative.
Chest radiography showed disseminated, hazy nodular infiltrates bilaterally which allowed no definite diagnosis (Figure 1
). Because of the severity of the patient's symptoms, a contrast-enhanced early arterial multidetector-row computed tomography (MDCT) of the thorax (16-row CT, MX 8000 IDT; Philips, Best, The Netherlands) with the following parameters was obtained: 140 mAs, 120 kV, slice thickness 2 mm, increment 1 mm, pitch 0.9. Intravenous contrast medium administration (Imeron 400®; Altana, Konstanz, Germany) took place after bolus tracking (140 ml, flow 3.5 s). On MDCT, the bronchial walls showed a significant thickening. There were diffuse peribronchial consolidations (Figures 2 and 3). In addition, there were enlarged mediastinal and hilar lymph nodes bilaterally. There were no other significant findings.
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Figure 2. Uncommon thickening of the bronchial walls(arrow). In addition, there are scattered peribronchiolar consolidations (arrow).
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Figure 3. Irregular outer shape of the thickened bronchial walls(arrow) with multifocal peribronchiolar consolidations.
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Flexible bronchoscopy was performed the next day as part of the infectious disease workup and revealed diffuse erythema of the mucosa of the entire tracheobronchial system. Diffusely scattered white plaques (up to 2 cm in diameter in the trachea) were biopsied. Histology demonstrated a multifocal ulcerative bronchitis with squamous metaplasia of the respiratory epithelium and ulceration of the epithelium (Figure 4). A large part of the biopsy showed a granulomatous inflammation with extensive necrotic changes. There were no signs of malignancy or tuberculosis. Histopathological findings in combination with the elevated C-ANCA led to the diagnosis of Wegener's granulomatosis.
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Figure 4. Histology of bronchoscopic biopsies.(a) Histology (haematoxylin-eosin staining) reveals an ulcerative bronchitis with squamous metaplasia of the respiratory epithelium (arrows) and ulceration of the epithelium (stars). Large part of the biopsy (upper part) shows a granulomatous inflammation with extensive necrotic changes (bar = 200 µm). (b) Insert shows a high power view of the granulomatous inflammation with necrosis, fibrin deposition with intermingled granulocytes, and a single giant cell (arrow; bar = 100 µm).
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Immunosuppressive therapy with cyclophosphamide and prednisolone was initiated. All of the above-described clinical symptoms improved rapidly and, within 1 month, the patient was completely asymptomatic. Chest radiography after 10 days showed marked resolution of the infiltrates and no consolidation was observed.
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Discussion
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First described in 1936, Wegener's granulomatosis is a clinical syndrome that consists of necrotizing granulomatous vasculitis of the upper respiratory tract, lungs and kidneys [1]. Typical histological findings are vasculitis, multifocal or patchy necrosis and granulomatous inflammation.
Chest radiography is abnormal in 75% of patients. The most common radiological finding is the presence of multiple non-calcified cavitating or non-cavitating nodules or masses of 2–4 cm in diameter [2]. These solid "tumours" are often bilateral and have a tendency to consolidate (in about 50% of cases). The cavities show thick walls. In the presence of a solitary cavitating mass, differentiation from tuberculosis or bronchial carcinoma can be difficult or impossible. Frequently, there is ground glass opacity due either to alveolitis or to alveolar haemorrhage secondary to vasculitis of the small vessels [3]. In addition, pleural thickening and areas of linear attenuation are very prevalent. The extent of the radiological abnormalities corresponds to disease severity [4].
Isolated thickening of the bronchial walls with peribronchiolar consolidations, which was observed in our patient, is rare [5]. The diffuse detection of such radiological changes in all lobes suggests systemic disease. The bronchial walls exhibit an irregular outer shape. The morphological correlate of this thickening of the bronchial wall was a granulomatous inflammation with extensive necrotic changes. The enlarged mediastinal and hilar lymph nodes we found in our patient on MDCT were also not typical findings for Wegener's granulomatosis. Clinical and laboratory findings lead us to the diagnosis of Wegener's granulomatosis with involvement of the lungs and renal involvement with only mildly elevated serum creatinine. The differential diagnosis must include, besides lung cancer and tuberculosis, different types of vasculitis and entities such as Churg-Strauss or Goodpasture syndrome and other forms of collagenous vascular disease.
Therapy with cyclophosphamide and steroids may result in complete normalization of radiological findings. However, a mixed response to immunosuppressive therapy is not uncommon. Atelectasis and transpulmonary bands tend not to improve [6]. Untreated, the 1 year survival rate of patients with Wegener's granulomatosis is only 20% [7, 8].
As this case shows, the differential diagnosis in patients with non-specific pulmonary symptoms and atypical radiological findings including bronchial wall thickening and peribronchiolar consolidations should include Wegener's granulomatosis, which should be excluded with an appropriate diagnostic workup. Biopsy of suspected lesions of the lung should be mandatory in these cases to obtain a definite diagnosis of Wegener's granulomatosis.
Received for publication December 13, 2005.
Revision received April 5, 2006.
Accepted for publication May 15, 2006.
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References
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Abstract
Introduction
