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Weekly Chest CasesArchive of Old Cases

Case No : 1067 Date 2018-04-09

  • Courtesy of Jae Heung Bae, MD., Jae-Kwang Lim, MD. / Kyungpook National University Hospital
  • Age/Sex 54 / M
  • Chief ComplaintGeneral weakness and cough for 6 months, recently complained ocular pain
  • Figure 1
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  • Figure 6
  • Figure 7

Diagnosis With Brief Discussion

Diagnosis
ANCA-associated granulomatous vasculitis (Granulomatosis with polyangiitis)
Radiologic Findings
Chest radiograph shows multifocal consolidation with internal cavitation or cystic changes in the both lung fields.
Chest CT shows a multiple, thick and irregular walled, pulmonary nodules or consolidation with cavitation in both lungs.
The patient was underwent wedge biopsy and confirmed with ANCA-associated granulomatous vasculitis.
Brief Review
ANCA-associated vasculitis (Wegener granulomatosis) is an uncommon necrotizing vasculitis that classically manifests as a clinical triad consisting of upper and lower airway involvement and glomerulonephritis. Other less frequently involved organ systems include the central and peripheral nervous system and large joints. The diagnosis is based on a combination of clinical and laboratory findings. Because thoracic involvement often predominates, chest radiographic findings are often the first to suggest the diagnosis.
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 is more common in nodules larger than 2 cm. Cavity walls may be smooth and thin or irregular and thick. 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.
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 ANCA-associated vasculitis, 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,

No. of Applicants : 129

▶ Correct Answer : 96/129,  74.4%
  • - Toranomon Hospital , Japan SHOHEI FUJITA
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - The University of Kyoto Hospital , Japan TOMOAKI OTANI
  • - ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Niigata University , Japan ATSUSHI UEHARA
  • - Higashi-Ohmi General Medical Center , Japan AKITOSHI INOUE
  • - Chungbuk National University Hospital , Korea (South) YEONGTAE PARK
  • - Chonbuk National University Medical School , Korea (South) HYEJIN YANG
  • - Ajou University School of Medicine , Korea (South) JUNG YONG JUN
  • - Gifu University Hospital , Japan TOMOHIRO ANDO
  • - Chonbuk National University Hospital , Korea (South) MINGI SHIN
  • - McGill University Health Center , Canada Alexandre Semionov
  • - NIMS, HYDERABAD , India BHASKAR K
  • - Chonbuk National University Hospital , Korea (South) SANGHEON KIM
  • - Chonbuk National University Hospital , Korea (South) EUN HA JUNG
  • - Soonchunhyang University College of Medicine , Korea (South) GOUN CHOI
  • - Seoul National University Hospital , Korea (South) JAE WON CHOI
  • - MD , India DEEPANDER SINGH RATHORE
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONGSU KANG
  • - Kashan University of Medical Sciences, kashan , Iran EBRAHIM RAZI
  • - Freelance resident in Reunion Island , Reunion Fabien HO
  • - Chungbuk National University Hospital , Korea (South) CHANGHOON OH
  • - 罹…stanbul , Turkey AYHAN YILMAZ
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Chonnam National University Hwasun Hospital , Korea (South) GANG SEONG WOONG
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - The Jikei university , Japan TAKU GOMI
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - Kyungpook national university hospital , Korea (South) Nari Jung
  • - Ajou University Hospital , Korea (South) Taeyang Ha
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - McGill University / University College London , Canada Ilan Azuelos
  • - Yonsei University, Wonju Severance Christian Hospital , Korea (South) JUYEON LEE
  • - Myongji Hospital , Korea (South) KI EON KWON
  • - HHS , Canada S LEE
  • - Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
  • - Chung buk national university hospital , Korea (South) hong gwon Byun
  • - Yeungnam University Medical Center , Korea (South) JONGSOO PARK
  • - Hospital Espa , Argentina JORGE PICOREL
  • - Gifu University Hospital , Japan Yo Kaneko
  • - VDC, HYDERABAD , India KARTHIK RAYASAM
  • - Samsung Medical Center , Korea (South) MIN YEONG KIM
  • - Dong-A University, College of Medicine , Korea (South) KI-NAM LEE
  • - , United Kingdom KRISHNA PRASAD PREMNATH BELLAM
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Chonbuk National University Hospital , Korea (South) YOUNGKWANG LEE
  • - Samsung Medical Center , Korea (South) KYOWON GU
  • - Ewha Womans University Medical Center , Korea (South) YOUNGSUN KO
  • - Medicheck health care , Korea (South) Chae hun Lim
  • - , Korea (South) DAYOUNG KIM
  • - National Taiwan University Hospital Hsin-Chu Branch , Taiwan LI-TA KENG
  • - Chungbuk National University Hospital , Korea (South) LEE JUNG HWAN
  • - , United States MAANSI P
  • - Tehran University of Medical Sciences , Iran ali mahdavi
  • - Mallinckrodt Institute of Radiology , United States Naganathan BS Mani
  • - CLINIQUE STE CLOTILDE , Reunion PATRICK MASCAREL
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - Kyeongpook National University Hospital , Korea (South) MIN A LEE
  • - Other , Korea (South) HAYEON LEE
  • - Eski , Turkey MURAT
  • - Soonchunhyang University Hospital Seoul , Korea (South) BODA NAM
  • - , Japan NAOMI YUASA
  • - Ajou University Hospital , Korea (South) HYERIN KIM
  • - Chungbuk national university hospital , Korea (South) Miran Yeon Yeon
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - Multimagem Diagn泥˜sticos , Brazil PEDRO PAULO TEIXEIRA E SILVA TORRES
  • - , Korea (South) JANG SEONG WON
  • - Wuhan Union Hospital , China QIGUANG CHENG
  • - Konkuk University Medical Center , Korea (South) YONG WONN KWON
  • - The University of Tokyo Hospital , Japan TAKU TAJIMA
  • - Yujin Yamazaki Hospital , Japan MASASHI TAKAHASHI
  • - , India RAJADIRAJAN SATHIYAMOORTHY
  • - Escola Paulista de Medicina , Brazil RENATO MASSON DE ALMEIDA PRADO
  • - , Korea (South) DAHYE SHIN
  • - Korea University Guro Hospital , Korea (South) KYU-CHONG LEE
  • - MBAL BURGAS , Bulgaria VLADISLAV BOYANOV RUSINOV
  • - Tashkent Medical Academy , Uzbekistan Rustam Azimovich Fayzullaev
  • - , Korea (South) SUNGEUN YUN
  • - Fortis hospital , Mohali , India SHALEEN RANA
  • - Kizawa Memorial Hospital , Japan Shoji Okuda
  • - Soonchunhyang University Hospital Seoul , Korea (South) JINAH KIM
  • - Teikyo University Hospital , Japan KAORU SUMIDA
  • - Kyoto Prefectural University of Medicine , Japan TADASHI TANAKA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - National Center for Global Health and Medicine , Japan HIROSHI TAKUMIDA
  • - Private sector , Greece VASILIOS TZILAS
  • - Grant medical college Mumbai , India VISHWANATH VASANT PUJARI
  • - Hamamatsu university school of medicine , Japan YUKI HAYASHI
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
  • - Chonnam National University Hwasun Hospital , Korea (South) JONG EUN LEE
  • - Kyemyung University Dongsan Hospital , Korea (South) DONG HYEON KIM
  • - Seoul Veterans Hospital , Korea (South) HYUN JUNG YOON
  • - Ajou University Hospital , Korea (South) YOO YOUNGJIN
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 15/129,  11.6%
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - King Abdulaziz University Hospital , Saudi Arabia Amr M. Ajlan
  • - Ondokuz Mayis University , Turkey CETIN CELENK
  • - , France GUILLAUME VESSELLE
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - NTUH , Taiwan KUEI-PIN CHUNG
  • - Other , Korea (South) MARI A
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - TB centre kasaragod. , India rikhy krishnan
  • - Ajou University Hospital , Korea (South) SUBIN HEO
  • - , Japan YUMI MAEHARA
  • - almouasat hospital , Syria wesam adel naser
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
▶ Semi-Correct Answer : 2/129,  1.6%
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - Holy Family Hopsital , India RAJESH GOTHI
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