Wegener's Granulomatosis - MedPix® Medical Image Database and Teaching Files

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Disease Topic 3610
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Contributor: Seth D. O'Brien - National Capital Consortium

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Wegener's Granulomatosis
Factoid 3610 - Created: 2002-02-03 13:38:44-05 - Modified: 2002-02-07 08:28:46-05

ACR Codes: 68.622
Wegener’s Granulomatosis is a rare necrotizing granulomatous and small-vessel arteritis disease that predominately affects the lungs, upper respiratory tract and kidneys. The mean age of presentation is the fifth decade with slightly male predominance. Characterized by a set of four clinical criteria: 1) abnormal urine sediment (glomerulonephritis) 2) abnormal chest radiograph 3) oral ulcers/nasal discharge and 4) granulomatous inflammation by histology patients are usually. Although cANCA is nonspecific to Wegener’s (also seen in Churg-Strauss, polyarteritis nodosa and other inflammatory diseases) the cANCA level correlates with disease activity. Classic radiographic findings include multiple focal opacities that may consolidate and have a lower lung predominance. Consolidation may be secondary to hemorrhage. Cavitation is a common phenomena with a rate of up to 40% on presentation. Pleural nodularity or effusions can occur, but are likely a rare occurrence of about 5%. Specific CT findings include ground-glass halo opacities representing hemorrhage and “feeding vessels” classically described by Kuhlman. Lastly, tracheal narrowing is also often found. During the course of the illness the radiographic manifestations of the disease will often have mixed findings of disease remission and relapse. Infectious processes and drug toxicities can further complicating the matter.
Although treatment has improved for Wegener’s granulomatosis with the addition of cytotoxic drugs, relapse is common and patients will usually progress to renal failure and it’s associated comorbidity.

Reference(s):
Armstrong. Imaging Diseases of the Chest. Mosby-Yearbook. 3rd Ed. 2000. pp550-55.

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Location: Chest, Pulmonary (ex. Heart)	Sublocation: none selected	Category: Inflammatory, autoimmune
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Prepared by: Seth D. O'Brien
Affiliation: National Capital Consortium - || -
Approved by: David S. Feigin, M.D.
Affiliation: Johns Hopkins Hospitals - || -

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