대한흉부영상의학회 Weekly Chest Cases

Case No : 1472 Date 2026-01-05

Courtesy of Jinwoo Yoon, Young Joo Suh / Severance Hospital
Age/Sex 58 / F
Chief ComplaintFever

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Diagnosis With Brief Discussion

Diagnosis: Granulomatosis with polyangiitis (GPA)
Radiologic Findings: Fig 1. Chest radiograph demonstrates subpleural nodules in right apex and increased opacities in both lower lung fields.
Fig 2-5. Chest CT scan reveals diffuse bronchial wall thickening bilaterally, multiple small nodules with peripheral distribution, and clustered centrilobular opacities in the right lung.
Brief Review: Follow-up chest CT obtained one month later demonstrates progression with diffuse soft tissue thickening along the bronchovascular bundle and airway narrowing. Enlargement of multiple nodules is also noted.

The patient presented with fever and symptoms of paranasal sinusitis. Paranasal sinus (PNS) CT (not shown) revealed bilateral nasal polyps and maxillary and ethmoid sinusitis. Video-assisted thoracoscopic surgery (VATS) lung biopsy was performed to exclude infection and malignancy, revealing granulomatous inflammation with multinucleated giant cells and necrotizing vasculitis. Notably, the patient’s serum cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) testing was negative, while perinuclear ANCA (p-ANCA) was positive.

Granulomatosis with polyangiitis (GPA) is a small‑vessel ANCA‑associated vasculitis characterized by necrotizing granulomatous inflammation of the respiratory tract and necrotizing vasculitis, often with pauci‑immune glomerulonephritis. The 2022 ACR/EULAR classification criteria require established small‑/medium‑vessel vasculitis (after excluding mimics), using a weighted scoring system where positive c‑ANCA/PR3‑ANCA and pulmonary nodules/masses/cavitation on chest imaging are major positive items, while p‑ANCA/MPO‑ANCA and marked eosinophilia are negative discriminators. Crucially, GPA can be classified even without c‑ANCA positivity if sufficient points are accrued from clinical, imaging, histologic, and laboratory findings. Thoracic imaging features include bilateral pulmonary nodules or masses (often subpleural or peribronchovascular, frequently cavitary), patchy consolidations or ground‑glass opacities, and less commonly interstitial lung disease; CT is the modality of choice, with pulmonary nodules/masses/cavities and sinonasal inflammation serving as key radiologic discriminators.

Airway involvement occurs in 15–55% of GPA patients and includes both large and small airway disease. The most characteristic lesion is circumferential subglottic stenosis, which begins as friable, edematous mucosa with ulceration and can progress to fixed fibrotic stenosis causing life‑threatening obstruction. Other tracheobronchial manifestations include segmental stenoses, mucosal edema, cobblestoning, ulcers, pseudomembranes, submucosal tunnels, and inflammatory pseudotumors. On CT, airway disease appears as concentric wall thickening of bilateral segmental and subsegmental bronchi with possible atelectasis or air‑trapping. Bronchoscopy is essential for mucosal evaluation and tissue sampling, though endobronchial biopsies often show only nonspecific granulation tissue. Management combines systemic immunosuppression (glucocorticoids with cyclophosphamide/rituximab for severe disease, methotrexate/azathioprine for mild disease) with bronchoscopic interventions or open surgical reconstruction as needed.
References
Keywords

No. of Applicants : 94

▶ Correct Answer : 20/94, 21.3%

- Funabashi General Hospital , Japan MANABU AKIMOTO
- medical scanning , Japan HIROAKI ARAKAWA
- , Japan HIROKI SAKURADA
- Shiga University of Medical Science , Japan AKITOSHI INOUE
- Kyoto University , Japan AKIHIKO SAKATA
- Oita University, Faculty of Medicine , Japan FUMITO OKADA
- , Japan HIROKI MIYAZAKI
- The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
- Nara Medical University , Japan AYA YAMADA
- , Japan YOSHIKI ISHII
- , Japan KEISUKE OSHIMA
- Etlik City Hospital, Ankara , Turkey MERIC TUZUN
- University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
- Osaka metropolitan university Hospital , Japan TATSUSHI OURA
- Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
- Kantou Rousai Hospital , Japan KAORU SUMIDA
- Sotiria Hospital, Athens , Greece VASILIOS TZILAS
- The Catholic University of Korea Yoeuido St. Mary , Korea (South) CHAWOONG JEON
- the first affiliatited hospital of nanjing medical univercity , China HAI XU
- University of Yamanashi , Japan KOJIRO ONOHARA

▶ Correct Answer as Differential Diagnosis : 25/94, 26.6%

- Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
- The University of Tokyo Hospital , Japan ISSEI FUKUDA
- ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
- Kyoto University Hospital , Japan YUSUKE UTSUNOMIYA
- , Italy PAOLO BALDASSARI
- Other , Korea (South) HEE SEOK CHOI
- Other , Korea (South) SEONGSU KANG
- Secomedic Hospital , Japan FUMINORI MIYOSHI
- Seoul Medical Center , Korea (South) HATAEK JEONG
- University of Yamanashi , Japan TAKAAKI HASHIMOTO
- University of Yamanashi , Japan HIROAKI WATANABE
- Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
- Japanese Red Cross Otsu Hospital , Japan JUN YOSHIDA
- , Japan KENTARO KOTANI
- , Japan SHUNJIRO NOGUCHI
- , Korea (South) HEONSEOK LEE
- Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI
- , Japan MASAMICHI IWAI
- GHICL , France MANUEL TOLEDANO
- , Japan MATSUNO MAI
- Kyoto University , Japan SHO KOYASU
- University of Tsukuba Hospital , Japan SODAI HOSHIAI
- Fukuoka university , Japan KEISUKE SATO
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- Ehime University , Japan TOMOHISA OKADA

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1472 Date 2026-01-05

Discussion

No. of Applicants : 94