대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Courtesy

S

Chief Complaint

A 53-year-old male presented with headache for one week.

Imaging Findings

Initial chest PA view and enhanced CT scan show thick-walled cavitary nodule with irregular inner margin in anterior segment of RLL

T1-weighted sagittal and enhanced coronal MRI show well-demarcated soft tissue mass with homogenuous enhancement in the midline of skull base. The mass involves clivus, sphenoid sinus, sella, right cavernous sinus, and extend to right parapharyngeal space.

After seven days, the cavity changes thin-walled cavity at the same region.

In the earliest stage detectable radiographically pulmonary wegener's graulomatosis manifests as bilateral, interstitial, reticular or nodular opacities that are most prominent at the lung bases.

As the disease progress, bilateral multifocal nodules and masses ranging from 5mm to 10cm in diameter may develop.

These lesions are typically sharply maginated, demonstrate no zonal predilection, and cavitate in 25-50% of case. Cavitary lesions have thick walls and irregular inner borders and may evolve into thin-walled cysts or even disappear after treatment.

Widespread, bilateral air-space opacities that correspone to intraalveolar pulmonary hemorrhage are a well-known presenting manifestation.

Therefore, Wegener's granulomatosis must be considered in the differential diagnosis of diffuse pulomnary hemorrhage.

The trachobronchial lesions of Wegener's granulomatosis produce direct radiographic signs of airway narrowing or secondary postobstructive effects, such as oligemia, emphysema, and atelectasis.

Hilar and mediastinal adenopathy is very unusual and calcification within lesions is rare.

In 88% of cases in one series, feeding vesssels were observed entering nodular lesions, a finding that reflects the angiocentric distribution of the disease.

Pleural-based, wedge-shaped lesions are also common and may mimic pulomnary infarction. Pleural thickening and pleural effusion are unusual CT findings.

Past History

He had no specific past history of any specific respiratory disorder or systemic illness.
Chest PA At admission
Brain MR 1 days after Admission
Chest CT1 3 days after Admission
Chest CT2 7 days after Admission

Discussion

Wegner's granulomatosis is a multisystem disease with variable clinical expression, which, is characterized pathologically by necrotizing granulomatous inflammation of the upper and lower respiratory tracts, glomerulonephritis, and necrotizing vasculitis of the lungs and a varity of systemic organs and tissues.

Two clinical variants of Wegner's granulomatosis have been described in addition to the full-blown systemic disease. The more common is manifested primarily or solely in the respiratory tract and is thus known as limited (non-renal) Wegner's granulomatosis.

Reference

1. Frazier, Pare et al. Wegener's granulomatosis: Diagnosis of disease of the chest. Philadelphia: 1999;vol3:1491-1506
2. Frazier AA et al. Pulmonary angitis and granulomatosis: radiologic-pathologic correlation. Radiographics 1998;18:687-710.
3. Aberle DR et al. Thoracic manifestations of Wegener granulomatosis: diagnosis and course. Radiology 1990;174:703-709
4. Shankar L et al. Wegener's granulomatosis: Head and neck imaging. USA:1998:65-66

Keywords

Lung, Multiple organ, Vasculitis, ANCA associated vasculitis,