Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Granulomatosis with polyangiitis (formerly, Wegener's granulomatosis)
- Radiologic Findings
- Fig. 1. Chest radiograph showing multifocal nodular increased opacities in both upper lung fields.
Figs. 2
- Brief Review
- Granulomatosis with polyangiitis is an uncommon necrotizing vasculitis that classically manifests as a clinical triad consisting of upper airway involvement, lower airway involvement, and glomerulonephritis. Because thoracic involvement is often predominant, chest radiographic findings are often the first to suggest the diagnosis. Common pulmonary radiologic findings include waxing and waning nodules, masses, ground-glass opacities, and consolidation. Airway involvement is usually indicated by circumferential tracheobronchial thickening, which can be smooth or nodular. Pleural effusion is the most common manifestation of pleural disease and can result from primary involvement or occur secondary to renal failure. Mediastinal lymphadenopathy is a nonspecific finding and is usually reactive. Uncommon thoracic radiologic manifestations include involvement of the heart and great vessels.
Ocular and orbital manifestations are common in almost half of the patients with granulomatosis with polyangiitis, affecting every structure of the eye, from the eyelid and orbit to the retina, choroid, and optic nerve, with a wide range of severity.
Elevation of serum cytoplasmic ANCA (c-ANCA) titers, usually directed toward proteinase 3 and myeloperoxidase (found in neutrophils), occurs in up to 90% of patients with active Wegener granulomatosis. The correlation between c-ANCA and Wegener granulomatosis has been well established. Although c-ANCA testing can aid in the diagnosis, a positive result is not conclusive. Negative c-ANCA test results are not sufficient to exclude the diagnosis, and biopsy remains the standard diagnostic method.
- References
- Common and Uncommon Manifestations of Wegener Granulomatosis at Chest CT: Radiologic-Pathologic Correlation RadioGraphics 2012; 32:51
- Please refer to
- Case 1078 Case 1067 Case 1026 Case 763 Case 722 Case 678
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- Keywords
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