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Granulomatosis with Polyangiitis (GPA) with Airway Stenosis

Granulomatosis with Polyangiitis (GPA) with Airway Stenosis▶ Watch on YouTube — age-restricted, cannot embed here

Imaging findings

The patient has a history of a left pulmonary artery stent and chronic destruction of the left lung. An early-phase CT shows a 'smoke phenomenon' (early filling of the left heart) due to altered flow, but no pulmonary embolism. The trachea and main bronchi demonstrate circumferential soft tissue thickening, characteristic of GPA, with involvement of the posterior membrane. A new, short-segment, tight stenosis with a 'web' or 'shelf' is noted just below the thoracic inlet, which had worsened compared to prior studies.

Key takeaways

In patients with granulomatosis with polyangiitis and known airway involvement, meticulous evaluation of the airways, especially with multiplanar reformats, is crucial to detect worsening stenosis. Such narrowing, even if short-segment, can be the cause of acute dyspnea and stridor. The 'smoke phenomenon' in the pulmonary circulation can result from underlying chronic lung disease altering vascular resistance and shunting.

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