Granulomatous polyangiitis

  • 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
  • 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.