Granulomatosis with polyangiitis (pulmonary manifestations)

This article discusses the pulmonary manifestations of granulomatosis with polyangiitis (previously known as Wegener's granulomatosis). It is classified as a type of pulmonary angiitis and granulomatosis.

For a general discussion of the condition, please refer to the main article on granulomatosis with polyangiitis (GPA). For other organ-specific radiographic features, please refer to individual articles:

Granulomatosis with polyangiitis (Wegener granulomatosis), is a multi-system systemic necrotizing non-caseating granulomatous vasculitis affecting small to medium-sized arteries, capillaries and veins 1, and the lungs are the most frequently involved organ, seen in 95% of cases.

Occasionally haemoptysis due to pulmonary haemorrhage can be the presenting symptom 3.

Unfortunately, the radiographic appearances of Wegener granulomatosis are very varied, making a diagnosis by imaging alone often difficult. Four patterns are recognised, although the first two are most common 3-4:

  1. nodules +/- cavitation
  2. pulmonary haemorrhage
  3. reticulonodular pattern
  4. peripheral wedge-like consolidation

The most common radiological presentation is with multiple nodules randomly distributed throughout the lungs of variable size. They range from well to poorly defined and may be distributed along bronchovascular bundles 5. In approximately half of cases, some of the nodules demonstrate cavitation, which is best seen with CT.

Both nodules and regions of consolidation can have surrounding haemorrhage, and in some instances, pulmonary haemorrhage dominates the presentation and radiographic appearance.

Although a reticulonodular interstitial infiltrate at the bases is often the first manifestation, it is usually asymptomatic.

In some cases focal patchy, often peripheral regions of alveolar consolidation are seen, which may also cavitate.

Pleural effusions are seen in 10-25% of cases, usually as a result of cardiac or renal involvement.

Tracheal and upper respiratory tract thickening may also be seen (see upper respiratory tract manifestations of GPA).

It is important to note that although the most common manifestation of recurrent/relapsing disease is cavitary nodules, patients may relapse with a different pattern of involvement to their initial presentation 3.

Plain radiograph
  • chest radiographs may show multiple nodules or masses that can be extremely variable in size (from a few millimetres to many centimetres)
  • although cavitation is present in ~ 50% of cases, is seen less frequently on plain film
  • airspace opacities may represent consolidation or pulmonary haemorrhage
CT
  • nodules or masses: variable size but typically ~2-4 cm
    • multiple in 75% 1
    • no zonal predilection
    • irregularly margined
    • cavitation with irregular / thick-walled cavity margins seen in ~40-50% of cases
    • cavitation may be seen in 25% of nodules > 2 cm 7
    • can have a peribronchovascular distribution
  • air space consolidation
    • peripheral wedge-shaped opacities (mimicking pulmonary infarcts)
    • focal
    • peribronchial
    • diffuse interstitial/alveolar opacities are a more common manifestation in children 2
  • mild bronchiectasis
  • ground glass changes
    • frequently as a consequence of haemorrhages
    • may relate to nodules or regions of consolidation
    • may be the main abnormality
  • focal atelectasis from airway stenoses

For a general discussion of treatment and prognosis, please refer to Wegener granulomatosis.

Complications

The differential depends on the dominant feature.

In cases of peripheral consolidation, appearances are very similar to pulmonary infarcts.

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Article Information

rID: 8952
System: Chest
Section: Pathology
Synonyms or Alternate Spellings:
  • Lower respiratory tract manifestations of Wegener granulomatosis
  • Wegener granulomatosis (pulmonary manifestations)
  • Wegener's granulomatosis (pulmonary manifestations)
  • Pulmonary manifestations of Wegener's granulomatosis
  • Pulmonary manifestations of granulomatosis with polyangitis
  • Pulmonary manifestations of Wegener granulomatosis

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Cases and Figures

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    Figure 1: gross pathology - Wegener granulomatosis
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    Figure 2: histology - granulomatosis with polyangitis
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