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Weekly Chest CasesArchive of Old Cases

Case No : 888 Date 2014-11-03

  • Courtesy of Kyung Eun Shin, Dong Wook Sung / Kyung Hee University Hospital
  • Age/Sex 49 / F
  • Chief ComplaintNasal obstruction, generalized weakness
  • Figure 1
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  • Figure 3
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  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
ANCA-associated granulomatous vasculitis (Wegener's granulomatosis)
Radiologic Findings
Chest CT shows a nodule and peribronchial consolidation with peripheral ground glass opacity. A nodule shows central low attenuation area at mediastinal window setting.
PNS CT shows sinusitis (Fig).

The patient underwent renal biopsy and pathologic result was ANCA (pauci-immune) associated focal necrotizing and crescentic glomerulonephritis. Also C-ANCA test was positive.
Brief Review
ANCA-associated granulomatous vasculitis (Wegener granulomatosis) is an uncommon necrotizing vasculitis affecting multiple organ systems. Classical manifestation is a clinical triad consisting of upper airway (almost 100%) and lower airway involvement (90%) and renal involvement (80%) although it may involve any part of the body. Patient symptom varies and depends on the organ system affected. Patients with pulmonary involvement often complain of cough with or without hemoptysis, dyspnea, fever, and chest pain. Elevation of serum cytoplasmic ANCA (c-ANCA) titers, usually directed toward proteinase 3 and myeloperoxidase, occurs in up to 90% of patients with active ANCA-associated granulomatous vasculitis.
Pulmonary nodules and masses are the most common CT findings of ANCA-associated granulomatous vasculitis and are seen in up to 70% of patients. Waxing and waning of the single or multiple pulmonary nodules and masses with random distribution are features of the disease. Central cavitation occurs in up to 50% of cases and most noncavitated nodules or masses show central low attenuation reflecting extensive central necrosis. CT halo sign (up to 15%) or reverse halo sign, radiating linear scarring, and pleural tags can be ancillary findings. Diffuse ground-glass opacity and consolidation (50%) may result from pulmonary hemorrhage or infection and bilateral perihilar and peribronchovascular distributions are the most common.
The tracheobronchial tree is the second most commonly affected area in the thorax (16-23% of cases) and subglottic portion of the trachea is most often common location. Segmental, focal, circumferential wall thickening involving the posterior membrane of the trachea is the typical radiologic findings. Pleural involvement (12-20% of the patient) causing pleural effusion and pleuritis and cardiac involvement as a form of pericarditis, coronary arteritis and myocardial ischemia owing to the vasculitis in small to medium-sized coronary artery could be also presented.
Without treatment, approximately 90% of patients with ANCA-associated granulomatous vasculitis die within 2 years of diagnosis. Aggressive immunosuppressive treatment 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 ANCA-associated granulomatous, but relapse is still common.
References
1. Martinex F, Jonathan H, Digumarthy S, et al. Common and uncommon manifestations of wegener granulomatosis at chest CT: Radiologic-pathologic correlation Radiographics 2012;32:51-69
2. Ananthakrishnan L, Sharma N, Kanne J, et al. Wegener granulomatosis in the Chest; High resolution CT findings.AJR 2009;192:676-682
3. Lohrmann C, Uhl M, Kotter E, et al. Pulmonary manifestations of wegener granulomatosis: CT findings in 57 patients and a review of the literature EJR 2005;53:471-477
Keywords
Lung, Vasculitis, ANCA associated vasculitis,

No. of Applicants : 72

▶ Correct Answer : 54/72,  75.0%
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - Ibaraki-gazou-shindan , Japan Shoichi Katoh
  • - Avrasya Hospital , Turkey Murat Ulusoy
  • - Government Medical College, Nagpur, India , India Krishna Prasad Bellam
  • - Tong young Red cross hospital , Korea (South)
  • - sahyadri speciality hospital , India kothareddy dileepreddy
  • - Kyoto University , Japan Akihiko Sakata
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - �stanbul , Turkey Ayhan Yilmaz
  • - Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
  • - SALMAN IBN ABDULAZIZ UNIVERSITY HOSPITAL , Saudi Arabia Elbagir Nasser
  • - Ondokuz Mayis University , Turkey Cetin Celenk
  • - University of Tsukuba Hospital , Japan Sodai Hoshiai
  • - GHICL , France manuel toledano
  • - Otsu Red Cross Hospital , Japan Hirotsugu Nakai
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - McGill University Health Center , Canada Alexandre Semionov
  • - 援 , Korea (South) Sungkwan Kim
  • - SNUH , Korea (South) Eui Jin Hwang
  • - Niigata City General Hospital , Japan Takao Kiguchi
  • - The University of Tokyo Hospital , Japan Takeyuki Watadani
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Asan medical center , Korea (South) Sang Young Oh
  • - Asan Medical Center , Korea (South) Jooae Choe
  • - Kmc chennai , India chellaraja c
  • - Kyung Hee University Hospital at Gangdong , Korea (South) Seong Jong Yun
  • - Wuhan Union Hospital , China Qiguang Cheng
  • - Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - Onomichi Municipal Hospital , Japan Yoshihisa Masaoka
  • - Chonnam national university hospital , Korea (South) Gunsoo Kim
  • - Seoul National University Hospital , Korea (South) Sang Min Lee
  • - GRMC , India shailesh gupta
  • - VHS medical center , Korea (South) Seong hee Jeon
  • - CHU Poitiers , France CHAN paul
  • - National Hospital Organization Okinawa Hospital , Japan Yasuji Oshiro
  • - Kizawa Memorial Hospital , Japan Yo Kaneko
  • - Ewha Womans University Hospital , Korea (South) YOOKYUNG KIM
  • - Inha university hospital , Korea (South) Eugene Kim
  • - Ishikawa Matto Central Hospital , Japan Manabu Akimoto
  • - IRSA La Rochelle , France Denis Chabassiere
  • - HUEC , Brazil Diogo Pinheiro
  • - CHRU Lille , France Julien Pagniez
  • - Teikyo University Mizonokuchi Hospital , Japan Noriko Kobayashi
  • - CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Fortis hospital , Mohali , India Shaleen Rana
  • - Seirei Hamamatsu General Hospital , Japan Kenichi Mizuki
  • - Northern Yokohama Showa university , Japan Kota Watanabe
  • - Univ Teach Hospital of Reunion Island , Reunion HO Fabien
  • - ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
  • - CIF-VSG , Switzerland Louis Locatelli
  • - Medicheck health care , Korea (South) Chae Lim
  • - Pneumologia Universitaria, Policlinico di Bari , Italy Mario Damiani
▶ Correct Answer as Differential Diagnosis : 9/72,  12.5%
  • - Fortis Hospital , India Vaibhav Singh
  • - The University of Tokyo Hospital , Japan Toshihiro Furuta
  • - the First Affiliated Hospital of NanJing Medical University , China Aiping Chen
  • - DAYA General Hospital,Thrissur,Kerala , India Raveendran TK
  • - 異⑸ , Korea (South) Yunhee Jang
  • - Chungbuk national university , Korea (South) Yong Kim
  • - National Center hospital of Neurology and Psychiatry , Japan Kaoru Sumida
  • - Gifu Central Hospital , Japan Haruo Watanabe
  • - NASA SCANS , India RAKESH BHATIA
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