Granulomatosis with polyangiitis (pulmonary manifestations)
This article discusses the pulmonary manifestations of granulomatosis with polyangiitis (previously known as Wegener 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: renal manifestations
- granulomatosis with polyangiitis: upper respiratory tract manifestations
- granulomatosis with polyangiitis: CNS manifestations
- granulomatosis with polyangiitis: orbital manifestations
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.
Unfortunately, the radiographic appearances of GPA are widely variable, making a diagnosis by imaging alone often difficult. Four patterns are recognized, although the first two are most common 3,4:
- nodules +/- cavitation
- pulmonary hemorrhage
- reticulonodular pattern
- 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 hemorrhage, and in some instances, pulmonary hemorrhage dominates the presentation and radiographic appearance.
Although a reticulonodular interstitial infiltrates 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 cavitatory nodules, patients may relapse with a different pattern of involvement to their initial presentation 3.
- chest radiographs may show multiple nodules or masses that can be extremely variable in size (from a few millimeters to many centimeters)
- although cavitation is present in ~ 50% of cases, is seen less frequently on plain film
- airspace opacities may represent consolidation or pulmonary hemorrhage
- 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)
- diffuse interstitial/alveolar opacities are a more common manifestation in children 2
- mild bronchiectasis
ground glass changes
- frequently as a consequence of hemorrhages
- may relate to nodules or regions of consolidation
- may be the main abnormality
- focal atelectasis from airway stenoses
- tracheobronchial wall thickening: can be smooth or nodular
- pleural effusions
- hilar and mediastinal lymphadenopathy 8
Treatment and prognosis
For a general discussion of treatment and prognosis, please refer to granulomatosis with polyangiitis.
- superimposed pulmonary infection
- airway stenosis / tracheobronchial stenosis 9-11
- subglottic stenosis is the most common with an estimated occurrence of around ~20% (range 16-23%) 9
- pulmonary hemorrhage
The differential depends on the dominant feature.
- differential of multiple pulmonary nodules
- differential of a single pulmonary nodule (uncommon presentation)
- differential of a cavitating lung mass
- differential of chronic air space consolidation
In cases of peripheral consolidation, appearances are very similar to pulmonary infarcts.
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