대한흉부영상의학회 Weekly Chest Cases

Case No : 722 Date 2011-08-29

Courtesy of Kiyeol Lee MD. / Korea University Ansan Hospital
Age/Sex 55 / M
Chief ComplaintCough (duration: 1 month) Blood tinged sputum (duration: 7 days)

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Diagnosis With Brief Discussion

Diagnosis: ANCA-associated granulomatous vasculitis (Wegener granulomatosis)
Radiologic Findings: Chest x-ray shows multiple mass-like opacities with or without cavitation in both upper lung zone and small nodular opacities in left lower lobe. CT scan reveals multiple thick-walled cavities and nodules in both lung fields. Some masses of them have central necrotic component which are dominant on both upper lobes.
PNS CT shows ill-defined soft tissue lesion in the anterior nasal cavities.
The patient was confirmed with ANCA-associated granulomatous vasculitis, named “Wegener granulomatosis” in the past, by VATs lung biopsy; pathologic findings are vasculitis, multifocal necrosis and micro-abscess formation, granuloma and scattered giant cell.
Brief Review: ANCA-associated granulomatous vasculitis (AGV) is a systemic necrotizing vasculitis that usually involves upper airway disease (almost always involvement; nasal, oral, or sinus inflammation from chronic sinusitis to septal perforation), lower respiratory airway disease (90%; lung), kidney (80%; from segmental necrotizing glomerulonephritis to rarely RCC) and other less common organs including skin-purpura, PNS-sensory neuropathy and cardiac involvement. Lung biopsy of AGV shows a granulomatous small-vessel necrotizing vasculitis. AGV most commonly occurs in whites and affects men and women equally and the mean age is 40 years. Elevation of serum cytoplasmic antineutrophil antibodies against protease 3 in cytoplasmic granules(c-ANCA) is positive more than 90% of patients with active stage of AGV. Patients with AGV have respiratory symptom such as hemoptysis, cough and dyspnea.
CT is sensitive for detection of lung involvement. Lung nodules and masses are the most common manifestation of AGV (40~70%) presenting multiply, bilaterally and without a zonal predilection and usually measuring between 2 and 4cm. The CT halo sign (a rim of GGO surrounding a nodule) is seen in 15% and most noncavitated nodules or masses show central low attenuation reflecting extensive central necrosis. The nodules may occur in a centrilobular distribution. Cavitation with thin or thick wall occurs in 25% of nodules larger than 2 cm. Approximately 50% of the nodules and masses resolve responding to treatment, 40% decrease in size and 10% remain unchanged. If air-fluid level in cavity is more suggestive of pulmonary infection rather than primary cavity of AGV.
Airspace consolidation and ground-glass opacity (20-50%) may result from alveolar hemorrhage or pneumonitis. They may have a random distribution, a peribronchial distribution, or appear as peripheral wedge-shaped lesions mimicking pulmonary infarction.
Diffuse alveolar hemorrhage occurs in 10% of patients with AGV, airway involvement in 15-25% as form of subglottic tracheal and/or bronchial stenosis in the central airways.
Although pathogenesis is unclear, the immunosuppression with corticosteroid and cyclophosphamide is the best current therapy (remission rate; 87%). In April 2011, the FDA approved rituximab (a chimeric antibody to CD20 protein) in combination with steroids to treat patients with WG, but relapse is still common.
References: 1. Lakshmi Ananthakrishnan, Nidhi Sharma, Jeffrey P.Kanne. Wegener’s granulomatosis in the chest : high-resolution CT findings. AJR 2009;192:676-682
2. Eva Castaner, Anna Alguersuari at all. When to suspect pulmonary vasculitis: radiologic and clinical clues. RadioGraphics 2010;30:33-53
3. Paul A. Bacon. The spectrum of wegener’s granulomatosis and disease relapse. NEJM 2005;352:4
Keywords: lung, vasculitis, vasculitis,

No. of Applicants : 84

▶ Correct Answer : 37/84, 44.0%

- clinique de SAVOIE , France, Metropolitan gay-depassier philippe
- Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
- Kohka Public Hospital , Japan Akitoshi Inoue
- McGill University Health Center , Canada Alexandre Semionov
- China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
- National Jewish Health , United States Jonathan Chung
- Hopital Louis Pradel LYON , France matthieu ROUSSET
- Onomichi municipal hospital , Japan Hirofumi Mifune
- Hangang sacred heart hospital , Korea (South) You Mie Han
- DCA, , India Rajesh Gothi
- James Paget Hospital , U.K , United Kingdom nabil mahmood
- IRSA La Rochelle France , France Denis Chabassiere
- Fr Mullers Medical College , India Deepu Thomas
- snuh , Korea (South) Choo jiyung
- Emory University Department of Radiology , United States Travis Henry
- Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
- Ewha Womans University Hospital , Korea (South) YOOKYUNG KIM
- Brabois - CHU Nancy , France Alban Gervaise
- Louisiana State University - Shreveport , United States Carlos Previgliano
- Montreal , Canada D J
- Onomichi municipal hospital , Japan Ryotaro Kishi
- Virgin Mary Hospital Burgas , Bulgaria VLADISLAV RUSINOV
- Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
- SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OOKUBO
- CHR ANNECY FRANCE , France anne-line MORONI
- Myongi , Korea (South) soon Moon
- Bupyong Serim Hospital , Korea (South) Hee Seok Choi
- Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
- CTO Milano , Italy Elisabetta Impellizzeri
- UWO , Canada S Lee
- IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
- Pneumologia Universitaria, Policlinico di Bari , Italy Mario Damiani
- Hotel-Dieu Montreal , Canada nicolas gautier
- Mallinckrodt Institute of Radiology , United States Naganathan Mani
- UZ GASTHUISBERG , Belgium Johan COOLEN
- Fukuyama daiichi Hospital , Japan Mototsugu Saeki
- Seoul St Mary Hospital , Korea (South) Chae Lim

▶ Correct Answer as Differential Diagnosis : 19/84, 22.6%

- Radiologie Brabois adultes , France ROPION helene
- CHU NANCY , France, Metropolitan LEGOU Francois
- Okayama University , Japan Akihiro Tada
- radiologist, aditya imaging centre , India vivek patel
- jaslok hospital & research centre mumbai , India JAINENDRA JAIN
- Radnet Teleradiology , Turkey Murat Ulusoy
- Seoul National University Hospital , Korea (South) YongSub Song
- Youngdong Hospital , Korea (South) Sang Min Lee
- Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
- Toyama University Hospital, Laboratory of Pathology , Japan TOMONORI TANAKA
- CHRU lille , France manuel toledano
- ministry of health , United Arab Emirates jesia mansoor
- McGill , Canada ilan azuelos
- All India Institute of medical sciences , India Justin Moses
- Kizawa Memorial Hospital , Japan Yo Kaneko
- Humber River Hospital, Toronto, Ontario , Canada Sean Carr
- IRSA , France jean-luc BIGOT
- NASA SCANS , India RAKESH BHATIA
- Private sector , Greece Vasilios Tzilas

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 722 Date 2011-08-29

Discussion

No. of Applicants : 84