대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Wegeners Granulomatosis

Radiologic Findings

Multiple pulmonary nodules or consolidation with or without cavitation in both lungs
Diffuse thickening of the tracheal wall
Anterior mediastinal soft tissue attenuation

Brief Review

The mean age at presentation is in the fifth decade with a wide range. Sex incidence is equal or slightly male predominant. Onset is usually acute or subacute. More than 90% of patients with active Wegener's granulomatosis have positive test for cytoplasmic antineutrophil cytoplasmic antibodies (cANCA).
During the course of the illness, 90% of patients have lung involvement. Other involved sites include upper respiratory tract such as paranasal sinus and kidney.
Most characteristic pulmonary radiological finding is nodular masses (60%) or consolidation (30%), either of which may cavitate (40%). Nodules are usually 2 to 4 cm in diameter and less than 10 in number. They have no specific predilection site and may have necrotic centers.
Other reported findings include pleura-based and wedge-like consolidation resembling pulmonary infarct, diffuse bilateral consolidation corresponding to diffuse pulmonary hemorrhage, tracheal narrowing, pleural effusion, pneumothorax, and lymphadenopathy.
A combination of cytotoxic and corticosteroid therapy has dramatically improved the long-term survival of patients with Wegener granulomatosis. With extended survival, patients now experience diverse cardiopulmonary abnormalities that represent primary or secondary manifestations of the disease or that result from diagnosis and treatment.
Aberle et al [3] reviewed the medical histories and chest radiologic findings of 19 patients with the histologic diagnosis of Wegener granulomatosis.
In these patients thoracic images demonstrated parenchymal nodules or consolidations with cavitation, diffuse interstitial disease, mediastinal or hilar adenopathy, and isolated stenoses of the larynx or tracheobronchial tree.
Intrathoracic relapse occurred in 18 cases; in one-third of these patients, findings at relapse differed from those at initial presentation.
Complications from diagnosis or therapy occurred in nine patients.
Pulmonary infection was the most frequent complication causing morbidity and was often clinically indistinguishable from the primary disease; it complicated relapse in five patients. The successful radiologic follow-up of patients with Wegener granulomatosis requires a consideration of the varied thoracic manifestations of both the primary disease and the complications of its treatment.

References

1. Farrelly CA. Wegeners granulomatosis: a radiological review of the pulmonary manifestations at initial presentation and during relapse. Clin Radiol 1982;33:545-551

2. Kuhlman JE, Hruban RH, Fishman EK. Wegener granulomatosis: CT features of parenchymal lung disease. J Comput Assist Tomogr 1991;15:948-952

3. Aberle DR, Gamsu G, Lynch. Thoracic manifestations of Wegener granulomatosis: diagnosis and course. Radiology 1990;174:703-709

Keywords

Lung, Vasculitis, ANCA associated vasculitis, Wegener's Granulomatosis