대한흉부영상의학회 Weekly Chest Cases

Case No : 1325 Date 2023-03-14

Courtesy of Jae Wook Lee, Kyung Eun Shin, Jai Soung Park / Soonchunhyang University Bucheon Hospital
Age/Sex 24 / F
Chief ComplaintFever, polyarthralgia

Figure 1
Figure 2
Figure 3
Figure 4
Figure 5
Figure 6
Figure 7

Diagnosis With Brief Discussion

Diagnosis: Granulomatosis with polyangiitis
Radiologic Findings: Fig 1. Chest radiograph showing multifocal nodular increased opacities in both lung fields.

Fig 2-5. Chest computed tomography (CT) scan with a lung window setting showing multifocal irregular and mass-like consolidations and multiple small nodules in both lungs with peribronchovascular and subpleural distribution. Contrast-enhanced CT scan with a mediastinal window setting showing hypodense necrosis within nodules..

Fig 6-7. Abdomen computed tomography (CT) scan showing splenic non-enhancement, wedge-shaped perfusion defects in both kidneys, and layered wall thickening of small and large bowel loops, suggesting findings related to vasculitis.

The patient underwent computed tomography-guided lung biopsy, and the pathologic result was granulomatous inflammation with vasculitis. The serum Antineutrophil Cytoplasmic Antibodies (c-ANCA) test was positive.
Brief Review: Granulomatosis with polyangiitis is an uncommon necrotizing vasculitis that classically manifests as a clinical triad consisting of upper airway involvement, lower airway involvement, and glomerulonephritis. Because thoracic involvement is often predominant, chest radiographic findings are often the first to suggest the diagnosis. Common pulmonary radiologic findings include waxing and waning nodules, masses, ground-glass opacities, and consolidation. Airway involvement is usually indicated by circumferential tracheobronchial thickening, which can be smooth or nodular. Pleural effusion is the most common manifestation of pleural disease and can result from primary involvement or occur secondary to renal failure. Mediastinal lymphadenopathy is a nonspecific finding and is usually reactive. Uncommon thoracic radiologic manifestations include involvement of the heart and great vessels.
Ocular and orbital manifestations are common in almost half of the patients with granulomatosis with polyangiitis, affecting every structure of the eye, from the eyelid and orbit to the retina, choroid, and optic nerve, with a wide range of severity.
Elevation of serum cytoplasmic ANCA (c-ANCA) titers, usually directed toward proteinase 3 and myeloperoxidase (found in neutrophils), occurs in up to 90% of patients with active Wegener granulomatosis. The correlation between c-ANCA and Wegener granulomatosis has been well established. Although c-ANCA testing can aid in the diagnosis, a positive result is not conclusive. Negative c-ANCA test results are not sufficient to exclude the diagnosis, and biopsy remains the standard diagnostic method.
References: Common and Uncommon Manifestations of Wegener Granulomatosis at Chest CT: Radiologic-Pathologic Correlation RadioGraphics 2012; 32:51–69
Ocular Manifestations of Granulomatosis with Polyangiitis: A Review of the Literature Ophthalmol Ther (2019) 8:227–234
Keywords

No. of Applicants : 85

▶ Correct Answer : 15/85, 17.6%

- , Japan HIROAKI ARAKAWA
- , Japan KYOKO NAGAI
- , Japan CHIAKI SATO
- , Japan FUMINORI MIYOSHI
- , United Arab Emirates GAYATHRI GANAPATHY
- Kaneda hospital , Japan HIROFUMI MIFUNE
- Soonchunhyang University Hospital Bucheon , Korea (South) HYEJOO PARK
- Ajou University Hospital , Korea (South) MINJI KIM
- Ajou University Hospital , Korea (South) JAYOUNG MOON
- Gifu University Hospital , Japan Yo Kaneko
- Seoul National University Hospital , Korea (South) SUNGHO HONG
- Chonbuk National University Hospital , Korea (South) SEOYUN CHOI
- the first affiliatited hospital of nanjing medical univercity , China HAI XU
- Hanyang University Hospital , Korea (South) SEUNG JIN YOO
- Jiangsu province hospital , China WANGJIAN ZHA

▶ Correct Answer as Differential Diagnosis : 16/85, 18.8%

- Jichi Medical University Hospital , Japan JUN KANZAWA
- Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
- , Italy PAOLO BALDASSARI
- Tiger Gate Hospital , Japan SHIN-ICHI CHO
- Vita Hospital , Brazil DIOGO LAGO PINHEIRO
- Other , Korea (South) SEONGSU KANG
- Seoul Medical Center , Korea (South) HYUK GI HONG
- Ajou University Hospital , Korea (South) HAEIN LEE
- Ajou University Hospital , Korea (South) HYUNSEUNG LEE
- , Japan KENTARO KOTANI
- Kyoto University Hospital , Japan YASUHISA KURATA
- Ajou University Hospital , Korea (South) YOU NA KIM
- Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
- University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
- Chonnam National University Hospital , Korea (South) KIM SANG GYUN
- , Korea (South) JIN WOO YOON

▶ Semi-Correct Answer : 14/85, 16.5%

- , Japan SUZUNE TSUKAMOTO
- ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
- National Center of Neurology and Psychiatry , Japan MOTO NAKAYA
- Seoul National University Hospital , Korea (South) HYUNGIN PARK
- Hukuda memorial hospital , Japan SHINTARO KANO
- Chungbuk National University Hospital , Korea (South) MIHYEON PARK
- Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
- Eskisehir Sehir Hastanesi , Turkey MURAT SAHIN
- Others , India PURUSHOTHAMA RAO TUMMALA
- Korea University Anam Hospital , Korea (South) KYU-CHONG LEE
- Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
- Toranomon Hospital , Japan KAORU SUMIDA
- , Japan YUKI HAYASHI
- Kyoto university , Japan AKIHIKO SAKATA

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1325 Date 2023-03-14

Discussion

No. of Applicants : 85