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Wegener Granulomatosis Thoracic Imaging

  • Author: James G Ravenel, MD; Chief Editor: Eugene C Lin, MD  more...
 
Updated: Nov 04, 2015
 

Overview

First described in the 1930s by Friedrich Wegener as a rhinogenic granulomatosis, Wegener granulomatosis is a disease of unknown etiology that is characterized by necrotizing granulomatous vasculitis of the upper and lower respiratory tract, glomerulonephritis, and small-vessel vasculitis of variable degree (classic Wegener granulomatosis). A limited form has also been described, in which the disease is primarily confined to the lung. In this form, involvement of the kidney, skin, and tracheobronchial tree is distinctly unusual. (See the image below.)[1, 2, 3, 4, 5, 6, 7, 8]

Wegener granulomatosis, thoracic. Posteroanterior Wegener granulomatosis, thoracic. Posteroanterior chest radiograph in a middle-aged man with Wegener granulomatosis shows heterogeneous airspace opacity, which occurs predominately in the lower lobes, and a focal ill-defined opacity in the right upper lobe. Findings are suggestive of pulmonary hemorrhage.

Although the list of differential diagnoses and other problems is exceedingly broad and varied, the differential diagnosis can usually be limited by evaluating the radiographic findings in the context of the clinical history.

There have been discussions about renaming the disease because of Wegener's association with the Nazi party in the 1930s. Wegener, a German pathologist, joined the so-called brownshirts, of the early Nazi party, and he was a member of the National Socialist Party. Working in the city of Lodz, where a closed ghetto for Jews was established, Wegener was said to have performed autopsies on some victims, and he may have reviewed a manuscript that was based on Nazi experiments. Although not implicated directly in war crimes, Wegener's involvement in Nazi Germany is considered by some to be a reason to desist from using his name eponymously as the name of this disease. An award in his honor was subsequently abandoned.[9, 10, 11]

Preferred examination

Because most patients have respiratory symptoms, chest radiographs are usually obtained first. These may be followed by computed tomography (CT) scans of the chest for better delineation of the abnormalities. Radiographic findings are nonspecific. The differential diagnosis includes various infections and malignancies. If the clinical findings are suggestive of Wegener granulomatosis, further evaluation with biopsy or laboratory studies (eg, cytoplasmic-antineutrophil cytoplasmic antibody [c-ANCA] testing) is required.[12, 13, 14, 15, 8, 16]

The major limitation of radiographic techniques is the broad differential diagnosis of abnormalities in Wegener granulomatosis. In addition, chest radiographic findings may be normal in as many as 20% of patients with the disease.

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Radiography

Pulmonary nodules are the most common chest radiographic manifestation of Wegener granulomatosis; they occur in 40-70% of the cases. Nodules may be solitary or multiple; they are cavitated in as many as 50% of patients with nodules. Both thick- and thin-walled cavities may be present. Their size varies, ranging from 1.5-10 cm, and the nodules may wax and wane over time. Pneumothorax in association with cavitary nodules and subpleural blebs has been reported. (See the images below.)[17, 18, 19]

Wegener granulomatosis, thoracic. Posteroanterior Wegener granulomatosis, thoracic. Posteroanterior chest radiograph in a middle-aged man with Wegener granulomatosis shows heterogeneous airspace opacity, which occurs predominately in the lower lobes, and a focal ill-defined opacity in the right upper lobe. Findings are suggestive of pulmonary hemorrhage.
Wegener granulomatosis, thoracic. Image obtained 4 Wegener granulomatosis, thoracic. Image obtained 4 months later in the same patient as in the previous image shows nearly complete resolution of lower lobe airspace disease, with partial resolution of the right upper lobe opacity. A new cavity is present in the left upper lobe.
Wegener granulomatosis, thoracic. Same patient as Wegener granulomatosis, thoracic. Same patient as in the previous 2 images. This radiograph was obtained 1 year after the image immediately previous to this one; it shows that the left upper lobe cavity has enlarged, without a change in wall thickness. Multiple new cavitary and noncavitary nodules are present in the right lung.
Wegener granulomatosis, thoracic. Thick-walled rig Wegener granulomatosis, thoracic. Thick-walled right upper lobe cavity in Wegener granulomatosis.
Wegener granulomatosis, thoracic. Wegener granulom Wegener granulomatosis, thoracic. Wegener granulomatosis is present as a single pulmonary mass. Chest radiograph shows a single right lower-lobe mass.

Airspace opacities are a second manifestation of Wegener granulomatosis. Usually, these findings involve a localized region of consolidation that may occasionally show central necrosis that mimics a lung abscess. Most frequently, this finding is the result of pulmonary hemorrhage, although pulmonary edema secondary to renal involvement may also occur. In one study of children, the airspace pattern was slightly more common. Over time, several of these opacities evolved into thin-walled cavitary lesions.

Other less common pulmonary manifestations include atelectasis and reticular interstitial opacities. Tracheal-bronchial abnormalities are rarely noted on chest radiographs, although tracheal stenosis may occasionally be visualized. Mediastinal adenopathy and pleural abnormalities are uncommon (< 10% of cases) and should prompt consideration of other diagnoses.

Degree of confidence

Because Wegener granulomatosis is a rare disease, its appearance at the top of a differential diagnosis is unusual. Exceptions involve patients with sinusitis or renal disease and cavitary nodules or those with any of the mentioned radiographic findings and the appropriate clinical and laboratory findings. Most often, radiographs are helpful in confirming the diagnosis and in assessing the extent of pulmonary involvement.

False positives/negatives

As previously mentioned, chest radiographs may be normal in as many as 20% of individuals with Wegener granulomatosis.

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Computed Tomography

The major value of CT scanning is in the further characterization of lesions found on chest radiography, as well as in the depiction of unsuspected or radiographically occult abnormalities. Occasionally, CT scan findings are normal. As with chest radiographic findings, the predominant CT manifestations of Wegener granulomatosis include pulmonary nodules with or without cavitation and airspace consolidation. (See the images below.)[20, 21, 16]

Wegener granulomatosis, thoracic. CT scan shows a Wegener granulomatosis, thoracic. CT scan shows a well-circumscribed right lower-lobe mass. Biopsy revealed necrotizing granulomatous vasculitis, which is consistent with Wegener granulomatosis.
Wegener granulomatosis, thoracic. CT image obtaine Wegener granulomatosis, thoracic. CT image obtained with lung window settings shows a typical appearance of nodules in Wegener granulomatosis. Multiple ill-defined peripheral nodules have a halo with a ground-glass appearance. The halo is thought to represent adjacent pulmonary hemorrhage.

At CT, pulmonary nodules and masses range from 5 mm to 10 cm, and they are often cavitated. The lesions tend to be multiple and well defined, although the presence of more than 10 lesions is unusual.

Airspace disease may include the following: (1) bilateral and diffuse disease caused by pulmonary hemorrhage, (2) scattered parenchymal disease with eventual coalescence of lesion, or (3) localized disease with ill-defined margins and air bronchograms or central cavitation. In the last case, the lesions may be surrounded by a halo of ground-glass opacity, which presumably occurs secondary to hemorrhage.

Interstitial abnormalities are often present in Wegener granulomatosis. They include interlobular septal thickening, parenchymal bands, and bronchial wall thickening. High-resolution CT (HRCT) scanning may be helpful in better defining these lesions. Pleural thickening, pleural effusion, and adenopathy may be present, but they are not usually associated with Wegener granulomatosis.

Tracheobronchial abnormalities are better evaluated with CT than with other modalities. Although as many as 60% of patients with Wegener granulomatosis have tracheobronchial abnormalities, to the authors' knowledge, no good data about the sensitivity and specificity of CT are available. In cases of suspected tracheobronchial involvement, thin, overlapping, axial sections with 2-dimensional and 3-dimensional reformations may provide better delineation of the length and degree of stenosis. These are particularly helpful when a tight stricture precludes the passage of a bronchoscope. (See the image below.)

Wegener granulomatosis, thoracic. Three-dimensiona Wegener granulomatosis, thoracic. Three-dimensional shaded-surface display of the trachea shows eccentric narrowing of the subglottic trachea in this patient with airway involvement from Wegener granulomatosis.

Although nodules occur more frequently in patients with active disease and although parenchymal bands are more often seen in patients with quiescent disease, findings at various disease stages overlap considerably. Therefore, CT scan findings cannot be used to determine disease activity. CT scanning has greater utility in determining the response to corticosteroid and cytotoxic drug therapy. An increase in the size or number of parenchymal abnormalities suggests relapse, whereas decreasing nodule size, thickening of cavity walls, and increasing spiculation of lesions have all been described as improvement indications.

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Magnetic Resonance Imaging

Magnetic resonance imaging (MRI) may be useful in documenting myocardial lesions. Lesions may be diffuse or focal, involving the midventricular wall; they are not typical of patterns associated with myocardial ischemia. Cardiac MRI findings overlap with those of other nonischemic myocardial diseases, such as sarcoidosis and amyloidosis. MRI of the brain and/or spine should be considered in cases of extrapulmonary disease that potentially involves the central nervous system.

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Ultrasonography

In a retrospective study, Oliveira et al found that in a highly selected population, there was a high prevalence of echocardiographic abnormalities thought to be directly related to Wegener granulomatosis. These abnormalities included regional wall motion abnormalities; left ventricular systolic dysfunction with decreased ejection fraction; and pericardial effusion. In this population, mortality was increased among patients who had cardiac involvement.[22]

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Nuclear Imaging

Lesions in Wegener granulomatosis are gallium avid, as demonstrated in case reports. A negative gallium scan may be helpful in excluding active disease.[23]

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Angiography

Although large pulmonary vessels may be attenuated on pulmonary angiography, catheter angiography has no role in the diagnosis or management of Wegener granulomatosis. Rare cases of overlap involving Takayasu arteritis and Wegener have been described.

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Contributor Information and Disclosures
Author

James G Ravenel, MD Associate Professor of Radiology, Vice Chair for Education, Chief of Thoracic Imaging, Department of Radiology, Medical University of South Carolina

James G Ravenel, MD is a member of the following medical societies: American College of Radiology, Association of University Radiologists, Association of Program Directors in Radiology, American Roentgen Ray Society, Radiological Society of North America, Society of Thoracic Radiology

Disclosure: Received grant/research funds from VuComp for salary support for research.

Coauthor(s)

Abid Irshad, MD Associate Professor, Department of Radiology, Medical University of South Carolina College of Medicine

Abid Irshad, MD is a member of the following medical societies: Radiological Society of North America, Society of Breast Imaging

Disclosure: Nothing to disclose.

Specialty Editor Board

Bernard D Coombs, MB, ChB, PhD Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand

Disclosure: Nothing to disclose.

Chief Editor

Eugene C Lin, MD Attending Radiologist, Teaching Coordinator for Cardiac Imaging, Radiology Residency Program, Virginia Mason Medical Center; Clinical Assistant Professor of Radiology, University of Washington School of Medicine

Eugene C Lin, MD is a member of the following medical societies: American College of Nuclear Medicine, American College of Radiology, Radiological Society of North America, Society of Nuclear Medicine and Molecular Imaging

Disclosure: Nothing to disclose.

Additional Contributors

Jeffrey A Miller, MD Associate Adjunct Professor of Clinical Radiology, University of Medicine and Dentistry of New Jersey-New Jersey Medical School; Faculty, Department of Radiology, Veterans Affairs of New Jersey Health Care System

Jeffrey A Miller, MD is a member of the following medical societies: American Roentgen Ray Society, Radiology Alliance for Health Services Research, Society of Thoracic Radiology

Disclosure: Nothing to disclose.

References
  1. Aberle DR, Gamsu G, Lynch D. Thoracic manifestations of Wegener granulomatosis: diagnosis and course. Radiology. 1990 Mar. 174(3 Pt 1):703-9. [Medline].

  2. Allen NB. Wegener's granulomatosis. In: Goldman L Bennett JC, eds. Cecil Textbook of Medicine. 21st ed. 2000: 1529-32.

  3. Cordier JF, Valeyre D, Guillevin L, et al. Pulmonary Wegener''s granulomatosis. A clinical and imaging study of 77 cases. Chest. 1990 Apr. 97(4):906-12. [Medline].

  4. Langford CA. Wegener''s granulomatosis: current and upcoming therapies. Arthritis Res Ther. 2003. 5(4):180-91. [Medline].

  5. Woywodt A, Haubitz M, Haller H, Matteson EL. Wegener's granulomatosis. Lancet. 2006 Apr 22. 367(9519):1362-6. [Medline].

  6. Cordier JF. [Pulmonary manifestations of the vasculitides]. Rev Prat. 2008 Mar 15. 58(5):492-8. [Medline].

  7. Pagnoux C. [Wegener's granulomatosis and microscopic polyangiitis]. Rev Prat. 2008 Mar 15. 58(5):522-32. [Medline].

  8. Gómez-Gómez A, Martínez-Martínez MU, Cuevas-Orta E, Bernal-Blanco JM, Cervantes-Ramírez D, Martínez-Martínez R, et al. Pulmonary manifestations of granulomatosis with polyangiitis. Reumatol Clin. 2014 Sep-Oct. 10 (5):288-93. [Medline].

  9. Feder BJ. A Nazi Past Casts a Pall on Name of a Disease. New York Times. January 22, 2008. Available at http://www.nytimes.com/2008/01/22/health/22dise.html?_r=2&oref=slogin&oref=slogin.

  10. Rosen MJ. Dr. Friedrich Wegener, the ACCP, and History. Chest. 2007 Sep. 132(3):739-41. [Medline].

  11. Rosen MJ. Dr. Friedrich Wegener and the ACCP, Revisited. Chest. 2007 Dec. 132(6):1723; discussion 1723-4. [Medline].

  12. Allen SD, Harvey CJ. Imaging of Wegener's granulomatosis. Br J Radiol. 2007 Sep. 80(957):757-65. [Medline].

  13. Frazier AA, Rosado-de-Christenson ML, Galvin JR, Fleming MV. Pulmonary angiitis and granulomatosis: radiologic-pathologic correlation. Radiographics. 1998 May-Jun. 18(3):687-710; quiz 727. [Medline].

  14. Cortese G, Nicali R, Placido R, Gariazzo G, Anrò P. Radiological aspects of diffuse alveolar haemorrhage. Radiol Med (Torino). 2008 Feb. 113(1):16-28. [Medline].

  15. Martinez F, Chung JH, Digumarthy SR, Kanne JP, Abbott GF, Shepard JA, et al. Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation. Radiographics. 2012 Jan-Feb. 32(1):51-69. [Medline].

  16. Ozmen O, Tatci E, Gokcek A, Koksal D, Dadali Y, Ozaydin E, et al. Integration of 2-deoxy-2-[18F] fluoro-D-glucose PET/CT into clinical management of patients with Wegener's granulomatosis. Ann Nucl Med. 2013 Dec. 27 (10):907-15. [Medline].

  17. Wadsworth DT, Siegel MJ, Day DL. Wegener''s granulomatosis in children: chest radiographic manifestations. AJR Am J Roentgenol. 1994 Oct. 163(4):901-4. [Medline].

  18. Agarwal R, Aggarwal AN, Gupta D. Another cause of reverse halo sign: Wegener's granulomatosis. Br J Radiol. 2007 Oct. 80(958):849-50. [Medline].

  19. Hayashi H, Morimoto K, Nakazawa K, Fujita J, Mori T, Sunada K, et al. [A case of limited form of Wegener's granulomatosis showing repeated occurrence and disappearance of nodules in chest X ray with no medication]. Nihon Kokyuki Gakkai Zasshi. 2007 Sep. 45(9):726-30. [Medline].

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  22. Oliveira GH, Seward JB, Tsang TS, Specks U. Echocardiographic findings in patients with Wegener granulomatosis. Mayo Clin Proc. 2005 Nov. 80(11):1435-40. [Medline].

  23. Slart RH, Jager PL, Poot L. Clinical value of gallium-67 scintigraphy in assessment of disease activity in Wegener''s granulomatosis. Ann Rheum Dis. 2003 Jul. 62(7):659-62. [Medline].

 
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Wegener granulomatosis, thoracic. Posteroanterior chest radiograph in a middle-aged man with Wegener granulomatosis shows heterogeneous airspace opacity, which occurs predominately in the lower lobes, and a focal ill-defined opacity in the right upper lobe. Findings are suggestive of pulmonary hemorrhage.
Wegener granulomatosis, thoracic. Image obtained 4 months later in the same patient as in the previous image shows nearly complete resolution of lower lobe airspace disease, with partial resolution of the right upper lobe opacity. A new cavity is present in the left upper lobe.
Wegener granulomatosis, thoracic. Same patient as in the previous 2 images. This radiograph was obtained 1 year after the image immediately previous to this one; it shows that the left upper lobe cavity has enlarged, without a change in wall thickness. Multiple new cavitary and noncavitary nodules are present in the right lung.
Wegener granulomatosis, thoracic. Thick-walled right upper lobe cavity in Wegener granulomatosis.
Wegener granulomatosis, thoracic. Cut surface of a gross pathologic specimen that shows a thick-walled cavity with internal hemorrhage and necrosis. Courtesy of Russell Harley, MD.
Wegener granulomatosis, thoracic. Wegener granulomatosis is present as a single pulmonary mass. Chest radiograph shows a single right lower-lobe mass.
Wegener granulomatosis, thoracic. CT scan shows a well-circumscribed right lower-lobe mass. Biopsy revealed necrotizing granulomatous vasculitis, which is consistent with Wegener granulomatosis.
Wegener granulomatosis, thoracic. CT image obtained with lung window settings shows a typical appearance of nodules in Wegener granulomatosis. Multiple ill-defined peripheral nodules have a halo with a ground-glass appearance. The halo is thought to represent adjacent pulmonary hemorrhage.
Wegener granulomatosis, thoracic. Three-dimensional shaded-surface display of the trachea shows eccentric narrowing of the subglottic trachea in this patient with airway involvement from Wegener granulomatosis.
 
 
 
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