CXR: Diffuse bilateral nodular densities right greater than left.

CT: There are diffuse multifocal ground-glass and consolidative
pulmonary opacities, many of which are nodular in appearance with
ill-defined fuzzy borders. More confluent areas of consolidation are
present in the perihilar regions in a bronchovascular distribution.
The opacities are more extensive in the right lung, particularly in
the right base. The periphery of the lung is spared. There is no
pleural fluid or thickening. No cavitary nodules are seen. There is
no fibrosis or bronchiectasis. No architectural distortion is seen.

Wegener's Granulomatosis with diffuse pulmonary hemorrhage.
Positive c-ANCA serology.

Wegener's Granulomatosis is a small vessel vasculitis which most commonly involves the respiratory and renal systems. The peak age of incidence is in the 4th to 5th decade, however, cases in the pediatric population have been well described. The most common presenting symptoms stem from involvement of the upper airways and include epistaxis, rhinitis, sinusitis and otitis media. Progression to involve the remainder of the respiratory tract occurrs in the majority of cases and can lead to symptoms of dyspnea, wheezing, hemoptysis, and postobstructive pneumonia. Glomerulonephritis is rarely present initially, however, it is the most common cause of death in untreated patients. Diffuse pulmonary hemorrhage is a rare but devistating complication of Wegener's in adults, however, interestingly, it is the most common presenting pulmonary finding in children.

The differential for diffuse pulmonary hemorrhage in a child should include: Idiopathic Pulmonary Hemosiderosis, Heiner's Syndrome, Stacybotyrs atra, Wegener's Granulomatosis, Goodpasture's Syndrome, Systemic Necrotizing Vasculitis, Systemic lupus Erythematosis, Henoch-Schonlein Purpura, and Cardiovascular anomalies.

Classic CT radiographic findings of non-hemorrhagic Wegener's are bilateral, interstitial, reticular or nodular opacities most often in the lung bases sparing the apicies. Cavitation of the nodules is also a common feature. Widespread ground-glass and consolidative opacities sparing the apicies and periphery (as seen in this case) are the classic findings of associated acute pulmonary hemorrhage.

Tissue biopsy remains the gold standard for diagnosis of Wegener's. Lung biopsy is the most reliable, although, high rates of morbidity and mortality in patients with vasculitis often preclude this option. Biopsy of upper airways, such as nose or sinus, is more feasible but reported to be diagnostic in only about 20 percent of cases.
Immunologic staining for Antinuclear Cytoplasmic Antibodies with cytoplasmic staining pattern (c-ANCA) is highly specific for Wegener's. sensitivity of this test is highly dependant on pre-test probability. This being said, if clinical suspicion is high, a positive c-ANCA titer can alone be considered diagnostic with a positive predictive value above 99 percent.

Case Number: 53391Last Updated: 03-11-2003
Anatomy: Lung   Pathology: Vascular
Modality: CT, Conventional RadiographExam Date: 03-04-2003Access Level: Readable by all users

The reader is fully responsible for confirming the accuracy of this content.
Text and images may be copyrighted by the case author or institution.
You can help keep MyPACS tidy: if you notice a case which is not useful (e.g. a test case) or inaccurate, please send email to alert@mypacs.net.