Wegener's Granulomatosis

Joshua Lee Rosebrook, MD - Case Coordinator
Cheryl Ann Sadow, MD - Radiology Discussion
Gerald P Bailey, MD, PhD - Pathology Discussion
Philip Costello, MD - Attending Radiologist
Pablo R Ros, MD, MPH - Attending Radiologist

August 12, 2002

Presentation

Imaging Findings

A cavitary lesion is visible in the left mid-lung field. In the right lung, a curvilinear shadow may represent a pneumothorax, and a large, rounded opacity is visible at the base. The opacity obliterates the right hemidiaphragm, perhaps indicating another mass. CT confirms the right pneumothorax, as well as left and right cavitary masses. The mass is the left lung appears to be associated with some parenchymal consolidation or adelectasis. There may be some small adhesions, but the CT is not clear.

Differential Diagnosis

Diagnosis

Discussion

At autopsy, the lungs were approximately 3x normal weight. There are large, granulomatous, cavitary lesions with central necrosis in the left lung. More lesions are dispersed throughout the right lung; the vasculature of the hilum is almost completely obliterated by the lesions in that area. Closer images show enlarged lymph nodes and central areas of hemorrhage and necrosis within the lesions. The histology demonstrates the geographic necrosis that is typical of Wegener’s granulomatosis. The slides illustrate a virtual "cell soup" due to liquefactive necrosis. A variety of granulomas can be seen. Even at low power, it is clear that a vessel wall is obscured intermittently by transmural inflammation. This was an indolent, chronic process composed of lymphocytes. Virtually no elastic fibers remain around the vessel wall, and necrosis is evident.

Radiology Discussion

The differential diagnosis of cavitary lung lesions can be represented by the mnemonic "CAVITY."

• Carcinoma: squamous cell, melanoma, cervical, sarcoma metastasis
• Autoimmune: Wegener’s, rheumatoid lung
• Vascular: bland/septic emboli
• Infection: TB, fungal (coccidio, aspergillosis, cryptosporidia, nocardia), bacterial (esp. GNR, staph, strep)
• Trauma
• Young: in young patients, these are often congenital lesions, such as bronchogenic cyst or communicating sequestration

Wegener’s granulomatosis is generally diagnosed based on history that includes the classic clinical triad:

• Sinusitis
• Pulmonary symptoms
• Renal insufficiency

It is a rare disease, striking just 3/100,000 people in the US. The average age of onset is 50, but Wegener’s can occur at almost any age; it most common in adults. It is also slightly more common in men, with a male:female predominance of 4:3.

On radiography, the earliest lesions are bilateral reticulonodular opacities. As the disease progresses, bilateral multifocal nodules (5mm-10cm) develop. The lesions are generally sharply marginated; 20-50% of them cavitate. On CT, more nodules (predominantly subpleural) are generally visible. Cavitation typically occurs in nodules larger than 2 cm. Approximately 88% of lesions have feeding vessels

References

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