대한흉부영상의학회 Korean Society of Thoracic Radiology

대한흉부영상의학회 Weekly Case 메뉴

Close

대한흉부영상의학회 Weekly Case 검색
대한흉부영상의학회 Weekly Case 검색
Advanced Search..

Close

Weekly Chest CasesArchive of Old Cases

Case No : 856 Date 2014-03-24

  • Courtesy of Misun Chung, Sang Young Oh, Hyun Joo Lee, Joon beom Seo / Asan Medical Center
  • Age/Sex 42 / M
  • Chief Complaintdry cough, night sweet (2 weeks age), bilateral otomastoiditis refractory to antibiotic therapy
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6
  • Figure 7

initial chest PA

Diagnosis With Brief Discussion

Diagnosis
ANCA-associated granulomatous vasculitis (Wegener granulomatosis)
Radiologic Findings
Chest PA shows patchy consolidation in right upper lobe field.
Chest PA after 1 month from the initial chest radiograph shows increased extent of previous noted patchy consolidation in right upper lung zone and newly developed multiple nodular and patchy opacities in both lungs.
Chest CT scan images at lung window setting show multiple variable sized consolidation and nodules in both lungs without definite zonal predominance.Chest CT scan images at mediastinal window setting show central low attenuation area within the nodules or consolidation.
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%) such as glomerulonephritis 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 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.
Please refer to
Case 90, Case 104, Case 678, Case 722, Case 763,
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, Wegener's granulomatosis

No. of Applicants : 103

▶ Correct Answer : 56/103,  54.4%
  • - Tokyo Metropolitan Police Hospital , Japan Akifumi Hagiwara
  • - SALMAN IBN ABDULAZIZ UNIVERSITY HOSPITAL , Saudi Arabia Elbagir Nasser
  • - Kyoto University , Japan Akihiko Sakata
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Samsung medical center , Korea (South) Hyun Su Kim
  • - NIMS, HYDERABAD , India ashish sarode
  • - Human medical imaging % intervention center , Korea (South) Suryoung Jun
  • - vims , India akhila d
  • - Hallym University Kangnam Sacred Heart Hospital , Korea (South) Jung Youn Kim
  • - Joeun Kumgang hospital , Korea (South) Ji Eun Seok
  • - Niigata City General Hospital , Japan Takao Kiguchi
  • - humiic , Korea (South) Ju Won Lee
  • - salem medical college , India karnan sudha
  • - National Hospital Organization Okinawa Hospital , Japan Yasuji Oshiro
  • - Hopital Bichat , France, Metropolitan Pauline Pradere
  • - McGill University Health Center , Canada Alexandre Semionov
  • - ICD,MCH CALICUT , India rikhy krishnan
  • - Seirei Hamamatsu General Hospital , Japan Kenichi Mizuki
  • - Shiga University of Medical Science , Japan Akitoshi Inoue
  • - Chonnam national university hospital , Korea (South) Wongi Jeong
  • - Chonnam Nationl University Radiology , Korea (South) juwon kim
  • - SNUH , Korea (South) Eui Jin Hwang
  • - Scans world , India Philson Mukkada
  • - , Korea (South) Chan Park
  • - , Korea (South) Kim sungmo
  • - Chonnam university hospital , Korea (South) Park young
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - Nagasaki University Hospital, Department of Pathology , Japan TOMONORI TANAKA
  • - Chonnam National University Hospital , Korea (South) Sungmin Moon
  • - CMC , Korea (South) SohYong Jeong
  • - Chonnam National Univ. Hospital , Korea (South) Jaemyeong Jo
  • - Hanyang universitiy, radiology department , Korea (South) HyunGyu Lee
  • - Chonbuk National University Hospital , Korea (South) Eunyoung Kim
  • - Niigata University , Japan Atsushi Uehara
  • - Chonnam National University Hospital, Radiology department , Korea (South) Park byungjin
  • - Chungbuk University Hospital , Korea (South) Yunhee Jang
  • - chungbuk national university hospital , Korea (South) soojung lee
  • - chungbuk national university hospital , Korea (South) jeehye kim
  • - 異⑸ , Korea (South) Junghwan Kim
  • - CHU Poitiers , France CHAN paul
  • - IRSA La Rochelle , France Denis Chabassiere
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - 異⑸ , China ZHANG YING
  • - Gachon Gil hospital , Korea (South) junhyung Ann
  • - Ishikawa Matto Central Hospital , Japan Manabu Akimoto
  • - 異⑸ , Korea (South) sooncheul baek
  • - Pneumologia Universitaria, Policlinico di Bari , Italy Mario Damiani
  • - The University of Tokyo Hospital , Japan Toshihiro Furuta
  • - NASA SCANS , India RAKESH BHATIA
  • - GHOL, Hopital de Nyon , Switzerland Benoit Rizk
  • - Chungbuk national university , Korea (South) Yong Kim
  • - Kyungpook National University Hospital , Korea (South) Jaekwang Lim
  • - Private sector , Greece Vasilios Tzilas
  • - Oita University , Japan Haruka Sato
  • - Teikyo University Mizonokuchi Hospital , Japan Kaoru Sumida
  • - Medicheck health care , Korea (South) Chae Lim
▶ Correct Answer as Differential Diagnosis : 15/103,  14.6%
  • - University of British Columbia , Canada Amr Ajlan
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - Seoul National University Hospital , Korea (South) Sang Min Lee
  • - Kyung Hee University Hospital at Gangdong , Korea (South) Seong Jong Yun
  • - CHU POITIERS , France SZTARK Guillaume
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
  • - UWO , Canada S Lee
  • - Ansan Sarang Hosp. , Korea (South) Jaecheon Oh
  • - Montreal , Canada Drago Le King
  • - Onomichi Municipal Hospital , Japan Yoshihisa Masaoka
  • - CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
  • - CLCC Rennes , France nicolas gautier
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - C.B.H. Dpt of Imaging-Clinica MATER DEI- Bari , Italy Carlo Florio
  • - CNUH , Korea (South) Kim Soo Hyun
  • Top
  • Back

Each Case of This Site Supplied by the Members of KSTR.
Copyright of the Images is in the KSTR and Original Supplier.
Current Editor : Sang Young Oh, M.D., Ph.D Email : sangyoung.oh@gmail.com

This website is optimized for IE 10 and above.