대한흉부영상의학회 Weekly Chest Cases

Case No : 763 Date 2012-06-13

Courtesy of Jin-Hwan KIm / Chungnam National Universitiy
Age/Sex 73 / M
Chief ComplaintMass on chest PA

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Figure 1.

Diagnosis With Brief Discussion

Diagnosis: ANCA-associated granulomatous vasculitis (Wegener granulomatosis)
Radiologic Findings: Chest radiograph shows masses and nodules in both lung zones. CT scan reveals multiple soft tissue density masses without cavitation in RLL and small nodules in BUL and RML.
The patient was confirmed with ANCA-associated granulomatous vasculitis, named Wegener granulomatosis in the past, by RLL lobectomy; pathologic findings are parenchymal necrosis, vasculitis, organizing pneumonia, and granulomatous inflammation.
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. 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 AGV, but relapse is still common.
References: 1. Ananthakrishnan L, Sharma N, Kanne JP. Wegener's granulomatosis in the chest : high-resolution CT findings. AJR 2009;192:676-682
2. Castaner E, Alguersuari A, Gallardo X, et al. When to suspect pulmonary vasculitis: radiologic and clinical clues. RadioGraphics 2010;30:33-53
3. Bacon PA. The spectrum of Wegener's granulomatosis and disease relapse. N Engl J Med 2005;352(4):330-332.
Keywords: lung, vasculitis,

No. of Applicants : 78

▶ Correct Answer : 19/78, 24.4%

- Montreal , Canada Drago Le King
- GM InHospital , Korea (South) Ju Won Lee
- Kyung Hee Universitiy Hospital at Gangdong , Korea (South) Seong Jong Yun
- Scansworld , India Philson Mukkada
- HUEC , Brazil Diogo Pinheiro
- Kyungpook National University Hospital , Korea (South) Jaekwang Lim
- Asan Medical Center , Korea (South) Ji Eun Kim
- Asan Medical Center , Korea (South) Eunyoung Kim
- Asan medical center , Korea (South) Hyun Joo Lee
- Kyunghee university medical center at Kangdong , Korea (South) Soyoun Shin
- Chungnam National University hospital , Korea (South) sunkyoung you
- HIA Legouest - METZ , France Alban Gervaise
- Ishikawa Matto Central Hospital , Japan Manabu Akimoto
- Fortis hospital , India Shaleen Rana
- IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
- McGill University Health Center , Canada Alexandre Semionov
- All India Institute of medical sciences , India Justin Moses
- Onomichi Municipal Hospital , Japan Yoshihisa Masaoka
- Chonnam National Univ. Hospital , Korea (South) Jaemyeong Jo

▶ Correct Answer as Differential Diagnosis : 15/78, 19.2%

- XiangYa hospital , China Xia Yu
- clinique de SAVOIE , France, Metropolitan gay-depassier philippe
- IRSA La Rochelle France , France Denis Chabassiere
- Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
- Onomichi municipal hospital , Japan Hirofumi Mifune
- China Medical University , Taiwan, R.O.C. , Taiwan Jun Jun Yeh
- Asan medical center , Korea (South) Sang Young Oh
- Asan Medical Center , Korea (South) Hyun Jung Koo
- Kizawa Memorial Hospital , Japan Yo Kaneko
- Himeji St.Mary Hospital , Japan Yuichiro Kanie
- Asan medical center , Korea (South) Jong Woo Kim
- IRSA , France, Metropolitan BIGOT
- Mallinckrodt Institute of Radiology , United States Naganathan Mani
- Radnet Teleradiology Istanbul , Turkey Armagan Sarac
- Private sector , Greece Vasilios Tzilas

▶ Semi-Correct Answer : 2/78, 2.6%

- Asan Medical Center , Korea (South) Sang Min Lee
- UWO , Canada S Lee

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 763 Date 2012-06-13

Discussion

No. of Applicants : 78