CT OF NONINFECTIOUS GRANULOMATOUS LUNG DISEASE

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SARCOIDOSIS

Sarcoidosis is a systemic disease of unknown cause that is characterized by the presence of noncaseating granulomas.92 Thoracic involvement occurs in up to 90% of affected individuals.22 The disease is relatively common with an estimated prevalence of 3 to 50 per 100,00022 and occurs worldwide, more frequently in temperate than tropical climates. Because approximately 50% of cases are asymptomatic, the true incidence of sarcoidosis likely is underestimated. There is no specific gender

HYPERSENSITIVITY PNEUMONITIS

Hypersensitivity pneumonitis, also known as extrinsic allergic alveolitis, is an allergic inflammatory granulomatous reaction of the lungs caused by the inhalation of specific antigens contained in a number of organic dusts. Numerous inciting agents have been identified, but the more common include thermophilic actinomycetes, avian proteins, and fungi.38 The clinical, radiologic, and pathologic features of HP are classically divided into three stages: (1) acute, (2) subacute, and (3) chronic.19

BERYLLIOSIS

Beryllium is a light weight metal with excellent thermal and electrical conductivity. Initially used in the manufacturing of fluorescent lamps, beryllium is now widely used in the ceramics, nuclear weapons, computer, and aerospace industries, and is a common component of many household appliances.3, 79

Berylliosis is caused by exposure to aerosols, dusts, or fumes of beryllium metal or salts3 and can occur in acute and chronic forms. Acute disease follows an overwhelming airborne exposure, which

WEGENER'S GRANULOMATOSIS

Wegener's granulomatosis is a systemic disease of unknown cause characterized by the triad of necrotizing granulomatous inflammation of the upper and lower respiratory tracts, glomerulonephritis, and disseminated small vessel vasculitis.13, 25 In the United States, the prevalence of WG is estimated to be 3 per 100,000 with a male to female ratio of about 1:1.47 Most affected patients are white and the mean age at diagnosis is 40 to 55 years.21, 47

The onset and course of WG vary depending on the

CHURG-STRAUSS SYNDROME

Churg-Strauss syndrome, also known as allergic angiitis and granulomatosis, is an idiopathic multisystem disorder characterized histologically by necrotizing vasculitis, extravascular granuloma formation, and tissue infiltration by eosinophils, which occurs almost exclusively in patients with asthma or other allergic diseases.15 CSS is the least common of the angiocentric granulomatoses with an estimated annual incidence of 3 per million.90 No gender predilection exists and mean age of onset

BRONCHOCENTRIC GRANULOMATOSIS

Bronchocentric granulomatosis is a rare disease of the lungs characterized by a necrotizing granulomatous reaction centered on the airways.53 Rather than a specific disease, BCG represents a pattern of inflammatory response to a sustained and intense insult to the airways.16 Affected patients can be divided into those with and those without concomitant asthma.40 Asthmatic patients with BCG typically present at a young age (childhood to early adulthood) and invariably have underlying allergic

NECROTIZING SARCOID GRANULOMATOSIS

Necrotizing sarcoid granulomatosis is a rare disease of unknown cause that is characterized histologically by the presence of confluent sarcoid-like granulomas associated with variable amounts of parenchymal necrosis and vasculitis.53 Because of some shared similarities in clinical, immunologic, and histologic features, NSG has been postulated to represent a variant of classic sarcoidosis.14, 89 Affected patients usually present between the third and seventh decades of life with women more than

LYMPHOMATOID GRANULOMATOSIS

Lymphomatoid granulomatosis, also known as angiocentric immunoproliferative lesion (AIL), polymorphic reticulosis, or angiocentric lymphoma, refers to a group of disorders characterized histologically by a necrotizing angiocentric lymphoproliferative process that primarily affects the lungs.37, 39 Onset of disease is usually in the fifth or sixth decades of life with men affected approximately twice as frequently as women.39, 65

Most patients with LYG present with respiratory symptoms, such as

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      Chronic hypersensitivity pneumonitis may be confused with IPF but is usually associated with the presence of poorly defined centrilobular nodules and areas of ground-glass opacities, features usually not seen in IPF.14,27 In contrast to IPF, HRCT findings in sarcoidosis include nodules along bronchovascular bundles (lymphatic distribution), coarse linear opacities involving mainly the perihilar regions of middle or upper lung zones and the bilateral hilar and mediastinal adenopathy.24,28 A nodular pattern may also be seen with hypersensitivity pneumonitis, pneumoconioses, infections, respiratory bronchiolitis, metastases, and alveolar microlithiasis (Figure 4).8

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      Lung lesions often resolve spontaneously, but some may progress to pulmonary fibrosis. The main histologic features are noncaseating granulomas, most of them located in the interstitium.12-14 The chest radiogram shows fine nodular, reticulonodular, or acinar opacities and rarely a pure miliary pattern.15

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    Address reprint requests to Ann N. Leung, MD Department of Radiology, S072A Stanford University Medical Center Stanford, CA 94305–5105 e-mail: [email protected]

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