Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation

Radiographics. Jan-Feb 2012;32(1):51-69. doi: 10.1148/rg.321115060.

Abstract

Wegener granulomatosis is an uncommon necrotizing vasculitis that classically manifests as a clinical triad consisting of upper and lower airway involvement and glomerulonephritis. Other less frequently involved organ systems include the central and peripheral nervous system and large joints. The diagnosis is based on a combination of clinical and laboratory findings. Because thoracic involvement often predominates, chest radiographic findings are often the first to suggest the diagnosis. However, chest computed tomography (CT) has superior sensitivity and specificity for evaluation of the airways, lung parenchyma, and mediastinum, particularly with the use of multiplanar reformatted and three-dimensional images. Common pulmonary radiologic findings include waxing and waning nodules, masses, ground-glass opacities, and consolidation. Airway involvement is usually characterized by circumferential tracheobronchial thickening, which can be smooth or nodular. Pleural effusions are the most common manifestation of pleural disease and can result from primary involvement or be 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. CT is the imaging modality of choice for diagnosis, surveillance, and follow-up in patients with Wegener granulomatosis.

MeSH terms

  • Adult
  • Female
  • Granulomatosis with Polyangiitis / complications*
  • Granulomatosis with Polyangiitis / diagnosis*
  • Humans
  • Male
  • Middle Aged
  • Radiography, Thoracic / methods*
  • Rare Diseases / diagnostic imaging
  • Rare Diseases / pathology
  • Statistics as Topic
  • Thoracic Diseases / complications*
  • Thoracic Diseases / diagnosis*
  • Tomography, X-Ray Computed / methods*