- Earlier called Wegener’s granulomatosis
- Primary (idiopathic) small vessel vasculitides
- Necrotizing granulomatous inflammation of small vessels of upper and lower respiratory tract
- M=F
- Any age of presentation
- Lungs affected in 90% of cases
- Cough, dyspnea, pleuritic chest pain, hemoptysis
- c-ANCA directed against proteinase-3 is positive
Imaging
- Chest Xray
- Bilateral nodules or masses
- Nodules from few mm to 10 cm
- No zone predilection
- Nodules larger than 2 cm cavitate
- CT
- Ground glass halo seen around nodules
- Feeding vessel sometimes seen leading to a nodule
- Linear bands
- Spiculations
- Pleural tags
- Residual parenchymal scarring after treatment
- Consolidation – multifocal with or without cavitation
- Peripheral wedge shaped opacities mimicking infarcts
- Peribronchovascular predilection
- Reverse halo
- Stenosis of large airways – subglottic, tracheal or bronchial narrowing
- Bronchiectasis in 40% cases
- Less common: lobar or segmental atelectasis, pleural effusion or thickening, hilar and mediastinal lymph node enlargement
Read more
Guzman-Soto, Mahatma I., et al. “From Head to Toe: Granulomatosis with Polyangiitis.” RadioGraphics 41.7 (2021): 1973-1991.
Martinez, Felipe, et al. “Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation.” Radiographics 32.1 (2012): 51-69.
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