대한흉부영상의학회 Weekly Chest Cases

Case No : 859 Date 2014-04-14

Courtesy of Hee Yeon An, Young Jin Kim / Severance Hospital, Yonsei University College of Medicine
Age/Sex 48 / F
Chief Complaintabnormal findings on chest radiograph

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Diagnosis With Brief Discussion

Diagnosis: ANCA associated granulomatous vasculitis (Wegener's granulomatosis)
Radiologic Findings: Chest x-ray shows multiple nodular opacities in both lungs. Chest CT scan reveals multiple nodules in both lungs. Some nodules have central necrotic component and peripheral GGO (CT halo sign). PET-CT shows intense FDG uptake in the multiple nodules in both lungs.
The patient was confirmed with ANCA-associated granulomatous vasculitis, named Wegener granulomatosis in the past.
Pathologic findings are geographic necrosis with areas of vasculitis and eosinophilic infiltrates. EVG special stain demonstrates necrotizing vasculitis in small- and medium-sized arteries.
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, ANCA associated vasculitis, Wegener's granulomatosis

No. of Applicants : 80

▶ Correct Answer : 4/80, 5.0%

- Dongnam Institute of Radiological and Medical Sciences , Korea (South) Dae-Wook Yeh
- McGill University Health Center , Canada Alexandre Semionov
- Medicheck health care , Korea (South) Chae Lim
- Onomichi municipal hospital , Japan Hirofumi Mifune

▶ Correct Answer as Differential Diagnosis : 25/80, 31.3%

- The University of Tokyo Hospital , Japan Akifumi Hagiwara
- Niigata City General Hospital , Japan Takao Kiguchi
- University of British Columbia , Canada Amr Ajlan
- sahyadri speciality hospital , India kothareddy dileepreddy
- Oita University, Faculty of Medicine , Japan Fumito Okada
- Nagasaki University Hospital, Department of Pathology , Japan TOMONORI TANAKA
- Avrasya Hospital , Turkey Murat Ulusoy
- Shiga University of Medical Science , Japan Akitoshi Inoue
- Royal Perth Hospital , Australia Yuranga Weerakkody
- Seoul National University Hospital , Korea (South) Sang Min Lee
- Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
- Asan medical center , Korea (South) Hyun Joo Lee
- National Hospital Organization Okinawa Hospital , Japan Yasuji Oshiro
- Seoul National University Gangnam Center , Korea (South) Hua Sun Kim
- jaslok hospital & research centre mumbai , India JAINENDRA JAIN
- chungbuk national university hospital , Korea (South) soojung lee
- Kizawa Memorial Hospital , Japan Yo Kaneko
- IRSA La Rochelle , France Denis Chabassiere
- Mallinckrodt Institute of Radiology , United States Naganathan Mani
- CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
- IRSA LA ROCHELLE , France, Metropolitan BIGOT
- 異⑸ , Korea (South) Junghwan Kim
- Pneumologia Universitaria, Policlinico di Bari , Italy Mario Damiani
- Okayama University , Japan Toshiyuki Komaki
- Private sector , Greece Vasilios Tzilas

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 859 Date 2014-04-14

Discussion

No. of Applicants : 80