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Findings: Non-contrasted chest CT shows multiple parenchymal masses. The right lower lobe mass shows evidence of cavitation. There is thickening and irregularity of the left main stem bronchus, and there is cut off of the left upper lobe bronchus. There is unilateral hyperlucency involving the left lung.
Diagnosis: Wegener's granulomatosis.
Discussion: The differential of multiple, cavitary masses includes metastatic disease, particular squamous cell, septic emboli, fungal and gram- pneumonias, and vasculitis.. The finding of simultaneous tracheobronchial involvement would suggest a primary squamous cell origin, aspergillus infection, lymphoma, and Wegener's granulomatosis.
Wegener's granulomatosis was first described in 1939. Its cause is not known, but is clearly immunologically mediated. A strong association with certain HLA subtypes is recognized . It involves the upper and lower respiratory tract, small arteries and veins, and causes a focal glomerulonephritis. While the mean age of presentation is in the 5th decade, the age ranges from 8 to 75. Upper airway symptoms are most common, including sinusitis and otitis. The vast majority of patients will have lung involvement at some time during the disease, and renal disease develops in 85%. Isolated lung involvement occurs in 10% of patients. Patients will present with chest pain, dyspnea, and hemoptysis. While morbidity and mortality was historically high, the use of cyclophosphamide and steroids have markedly improved the prognosis. The association of Wegeners' to antibodies to neutrophil cytoplasmic components (c-ANCA) was established by Van der Woude in 1985, and the test for c-ANCA has become a highly sensitive test for Wegener's granulomatosis.
Radiographically, the chest radiograph shows multifocal pulmonary opacities, and cavitations occur in 40% of the time. Single mass-like opacities can occur. In the setting of cavitary nodules, feeding vessels are often present. When there is diffuse consolidation present, pulmonary hemorrhage should be considered. Narrowing of the airway should be evaluated. Subglottic tracheal stenosis s is seen in around 10-15% of patients, and 18% have involvement of the distal airways. Pleural effusions occur in up to 55% of patients while adenopathy is uncommon.
References:
Hasleton PS. Spencer's Pathology of the Lung. McGraw Hill,
New York, 1996.
Farrelly C, Foster DR. Atypical Presentation of Wegener's Granulomatosis.
Br J Radiol. 1980;53:721-722.
MacGregor MBB, Sandler G. Wegener's Granulomatosis:
A Clinical and Radiological Survey. Br J Radiol 1964;37:430-439.
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