ANCA-Associated Granulomatous Vasculitis

CT Findings

On CT, amyloidomas appear as nodules with sharp or lobulated margins in a peripheral or subpleural location [22]. Nodules are variable in shape and size, measuring from 0.5 to 15 cm in diameter (Fig. 19.5). The nodules show slow growth often over several years without regression. CalciÞcation is often central or in an irregular pattern within the nodule and is seen in approximately 50 % of cases. Cavitation may occur, but is very rare.

CT–Pathology Comparisons

Nodular appearance of amyloidoma on CT represent _ replacement of the parenchyma by amyloid admixed with multinucleated giant cells and plasma cells.

Patient Prognosis

Nodular parenchymal amyloidosis rarely requires intervention or medical therapy.

ANCA-Associated Granulomatous Vasculitis

Pathology and Pathogenesis

In classic form of ANCA-associated granulomatous vasculitis, multiple irregular but well-circumscribed masses of various sizes are shown in the lungs. They consist of grayindurated tissue surrounding a soft, friable, gray, or hemorrhagic necrotic center which may cavitate. Microscopically, the nodules show irregular areas of necrosis surrounded by inßammatory granulation tissue. The outlines of necrotic vessels or other structures may be evident centrally, and there is often extensive karyorrhexis resulting in the accumulation of Þne hematoxyphilic nuclear dust. Variable numbers of multinucleate foreign-body or Langerhans giant cells may be seen [23].

include fever, arthralgia, myalgia, and weight loss and ocular involvement are common. Massive pulmonary hemorrhage, although uncommon, is a life-threatening manifestation.

CT Findings

The most common CT Þnding at the initial presentation is the presence of multiple, bilateral nodules or masses [6, 24] (Fig. 19.6). The nodules tend to have a random distribution, but they occasionally have a subpleural or peribronchovascular distribution. With progression of disease, the nodules tend to increase in size and number. Cavitation occurs in approximately 50 % of cases. The cavities are usually thick walled and tend to have an irregular, shaggy inner lining. Other common Þndings include bronchial wall thickening, large airway involvement, airspace consolidation, and areas of GGO. Centrilobular small nodules and a tree-in-bud pattern may be seen in up to 10 % of patients. After treatment, these lesions usually show a decrease in the extent.

CT–Pathology Comparisons

Nodules or masses on CT represent inßammatory nodules composed of large areas of parenchymal necrosis, granulomatous inßammation, and vasculitis. Airspace consolidation and areas of GGO represent diffuse alveolar hemorrhage caused by necrotizing capillaritis. Centrilobular nodules and the tree-in-bud sign may result from bronchiolar inßammatory changes rather than from vasculitis [24].

Patient Prognosis

The pharmacologic treatment of vasculitis necessitates the use of cytotoxic medications and systemic corticosteroids. The 5-year survival of ANCA-associated granulomatous vasculitis has been reported to be 74Ð91 % [25]. Main factors affecting survival are age and target organ damage.

Symptoms and Signs

ANCA-associated granulomatous vasculitis is characterized by necrotizing granulomatous inßammation with the classic triad of upper airway involvement (sinusitis, otitis, ulcerations, bony deformities, subglottic, or bronchial stenosis), lower respiratory tract involvement (cough, chest pain, dyspnea, hemoptysis), and glomerulonephritis (hematuria, RBC casts, proteinuria, azotemia). Constitutional symptoms that

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