Wegner’s Granulomatosis

Dr/ Hytham Nafady

Hertz

Definition

Multisystem disease of unknown etiology characterized by

• Granulomatous necrotizing small vessel vasculitis of the upper & lower respiratory tract &

• Glomerulonephritis.

Wegner’s granulomatosis involves the

• upper respiratory tract in 100% of cases,

• lower respiratory tract in 90% of cases &

• kidneys in 80% of cases, &

• Tracheo-bronchial tree in 60% of cases.

Demographics

• Age: around 45.

• M>F

C.P

• Rhinitis, sinusitis & epistaxis.

• Cough & hemoptysis.

• Hematuria & Chronic renal failure, which is the cause of death.

• Lab: elevated (c-ANAC) antineutrophil antibodies.

• Renal biopsy: glomerulonephritis, which is diagnostic.

Radiological manifestations

Respiratory manifestations of

Wegner’s granulomatosis

Nodules Alveolar opacities

Airways

Ground glass

opacitiesConsolidation

Subglottic tracheal stenosis

Bronchial stenosis

Cavitating

Non cavitating

Rare manifestations of WG

• Interstitial abnormalities (thickening of interlobular sepat).

• Hilar lymphadenopathy.

• Pleural thickening or effusion.

Nodules

• Single or multiple.

• Small or large (few mm to 10 cm).

• Cavitating or non cavitating.

• Thin walled (1-3 mm) or thick walled (> 3 mm) cavities.

• Halo sign (due to surrounding alveolar hemorrhage).

• Reversed halo sign.

Halo sign

Reversed halo sign

Alveolar WG

Due to alveolar hemorrhage.

Alveolar WG

Ground glass opacities Consolidation

Centrilobular nodules Patchy distribution

Centrilobular nodules of GG density

GGO with patchy distribution

Consolidation

Airway WG• Subglottic tracheal stenosis.• Bronchial stenosis

Case

27-year-old woman involved in a severe motor vehicle collision years ago with a prolonged hospital course now presents with a several months history of progressive dyspnea.

• What is the pertinent radiologic finding?

• What is your differential diagnosis?

• What is your favorite diagnosis?

DD of tracheal stenosis