대한흉부영상의학회 Weekly Chest Cases

Case No : 1078 Date 2018-06-25

Courtesy of Cherry Kim, Ki Yeol Lee / Korea University Ansan Hospital
Age/Sex 62 / M
Chief ComplaintHemoptysis, dyspnea, and history of herbal medication

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Diagnosis With Brief Discussion

Diagnosis: Granulomatosis with Polyangiitis (Wegener's granulomatosis)
Radiologic Findings: Initially, axial CT images show ill-defined ground-glass opacity in the right lung. After two months, CT images show increased extent of ground-glass opacity in both lungs, and new ill-defined consolidation in the right lower lobe.

In this patient, the presence of serum antineutrophilic cytoplasmic antibody (C-ANCA) was detected. The results of renal biopsy was pauci-immune crescentic glomerulonephritis, and bilateral maxillary sinusitis was also noted in the PNS series.
Brief Review: Granulomatosis with Polyangiitis (GPA) or Wegener granulomatosis is a rare, multisystem disease of unknown cause that may be associated with involvement of the upper and lower respiratory tracts, glomerulonephritis, and necrotizing vasculitis that affects small and medium-size vessels and a variety of organs and tissues. Lung disease associated with GPA is characterized by neutrophilic capillaritis, granulomatous inflammation, necrotizing vasculitis affecting small and medium-sized vessels, and geographic parenchymal necrosis. The presence of C-ANCA is characteristic and seen in as many as 90% to 95% of patients with active disease. Radiographic manifestations include multiple pulmonary nodules or masses, often cavitary; solitary nodule or mass; and focal or diffuse consolidation. The typical appearance is that of multiple nodules, usually limited in number, ranging in size from a few millimeters to 10 cm in diameter, without a zonal predominance, and having a random distribution.
Consolidation or ground-glass opacity is also a common manifestation of GPA, usually related to pulmonary hemorrhage. This may be the result of small vessel vasculitis or capillaritis. Lee et al. reported that patchy areas of consolidation and ground-glass opacity were seen in 7 of 30 (23%) patients. Consolidation may occur as an isolated finding or in association with pulmonary nodules. The distribution of consolidation is variable, being lobular, patchy, or diffuse in different patients.
References: 1. Martinez, Felipe, et al. "Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation." Radiographics 2011; 32: 51-69.
2. Ananthakrishnan, Lakshmi, Nidhi Sharma, and Jeffrey P. Kanne. "Wegener's granulomatosis in the chest: high-resolution CT findings." American Journal of Roentgenology 2009; 192: 676-682.
3. S D allen, et al. “Pictorial review: Imaging of Wegener’s granulomatosis.” The British Journal of Radiology, 2007; 80: 757–765.
4. Lee KS, Kim TS, Fujimoto K, et al. Thoracic manifestation of Wegener’s granulomatosis: CT findings in 30 patients. Eur Radiol 2003;13:43–51.
Keywords: Lung, Vasculitis, ANCA-associated granulomatous vasculitis ,

No. of Applicants : 89

▶ Correct Answer : 1/89, 1.1%

- Ichinomiya Nishi Hospital , Japan Takao Kiguchi

▶ Correct Answer as Differential Diagnosis : 5/89, 5.6%

- Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
- Higashi-Ohmi General Medical Center , Japan AKITOSHI INOUE
- McGill University Health Center , Canada Alexandre Semionov
- , Japan NAOMI YUASA
- the first affiliatited hospital of nanjing medical univercity , China HAI XU

▶ Semi-Correct Answer : 12/89, 13.5%

- Chonbuk National University Hospital , Korea (South) MINGI SHIN
- Other , Korea (South) SEONGSU KANG
- Freelance resident in Reunion Island , Reunion Fabien HO
- The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
- Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
- Mallinckrodt Institute of Radiology , United States Naganathan BS Mani
- Wuhan Union Hospital , China QIGUANG CHENG
- DECRH, Fredericton , Canada RAM P GALWA
- The First Affiliated Hospital of Nanjing Medical University , China ZHANG TENG
- Hamamatsu University Hospital , Japan YUKI HAYASHI
- Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
- Jiangsu province hospital , China WANGJIAN ZHA

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1078 Date 2018-06-25

Discussion

No. of Applicants : 89