대한흉부영상의학회 Weekly Chest Cases

Case No : 1396 Date 2024-07-22

Courtesy of Seulgi You, Joo Sung Sun / Ajou University Hospital
Age/Sex 63 / F
Chief Complaint10days URI Sx, mild fever

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Diagnosis With Brief Discussion

Diagnosis: Granulomatosis with polyangiitis (GPA)
Radiologic Findings: Fig 1. Chest PA shows several nodular opacities in Lt lung.
Fig 2-8. CT scans reveals multiple nodular opacities and consolidations in both lungs. Lesions show enhancement. Some nodules showed well defined margin and consolidation showed irregular shape.
Brief Review: Granulomatosis with polyangiitis (GPA) was confirmed through surgical wedge resection. Five months after diagnosis, the patient complained of left eye swelling. An orbital MRI revealed an enhancing infiltrative lesion in the left orbital apex fat and diffuse mucoepithelial thickening with heterogeneous enhancement in bilateral paranasal sinuses. Surgical biopsy confirmed GPA involvement.

Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is an antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis. GPA is a multisystemic disease predominantly affecting small vessels and can involve any organ. Pulmonary involvement is observed in over 90% of patients. The most common imaging findings include multiple variable-sized nodules and masses throughout the lungs, with cavitation occurring in 25% of cases. Pulmonary hemorrhage may accompany nodules, leading to ground-glass opacities halo. GGO to consolidation occur in about 30% of patients, usually resulting from hemorrhage. Tracheobronchial tree involvement is a late complication of GPA, presenting as focal, segmental, multifocal, or elongated segments of stenosis, with possible mucosal ulceration.
The elevation of serum cytoplasmic ANCA (c-ANCA) titers is observed in up to 90% of patients with active GPA. While c-ANCA testing can contribute to the diagnostic process, a positive result alone is not conclusive. Negative c-ANCA test results are insufficient to exclude the diagnosis, and biopsy remains the standard diagnostic method.
References: 1. Guzman-Soto MI et al., Radiographics. 2021 Nov-Dec;41(7):1973-1991. doi: 10.1148/rg.2021210132.
2. Ananthakrishnan L et al., Am J Roentgenol. 2009 Mar;192(3):676-82. doi: 10.2214/AJR.08.1837.
Keywords

No. of Applicants : 59

▶ Correct Answer : 9/59, 15.3%

- Korea University Anam Hospital , Korea (South) KYU-CHONG LEE
- , Korea (South) HEONSEOK LEE
- The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
- Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
- , Sweden MOHAMAD TLASS
- University of Yamanashi , Japan HIROYUKI MORISAKA
- , Japan YOSHIKI ISHII
- Nankai medical center , Japan NORITAKA KAMEI
- Mie university , Japan SHIKO OKABE

▶ Correct Answer as Differential Diagnosis : 10/59, 16.9%

- Other , Korea (South) SEONGSU KANG
- Oita University, Faculty of Medicine , Japan FUMITO OKADA
- Kyoto University , Japan AKIHIKO SAKATA
- Osaka metropolitan university Hospital , Japan TATSUSHI OURA
- Other , Korea (South) HYEJOO PARK
- Kantou Rousai Hospital , Japan KAORU SUMIDA
- Osaka University , Japan AKINORI HATA
- Dokkyo Medical University , Japan HIROAKI ARAKAWA
- Hyogo Prefectural Kobe Children , Japan SHUHEI NORIMOTO
- University of Yamanashi , Japan HIROAKI WATANABE

▶ Semi-Correct Answer : 5/59, 8.5%

- Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
- MAGNUM DIAGNOSTICS - Goa INDIA , India PARESH K DESAI
- University of Yamanashi , Japan KOJIRO ONOHARA
- Kyoto University , Japan SHO KOYASU
- ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1396 Date 2024-07-22

Discussion

No. of Applicants : 59