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Adult Chronic Granulomatous Disease Demonstrates Nodules and Consolidation

July 29, 2009
Written by: , Filed in: Chest Radiology
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A recent study was conducted to determine the radiologic findings of chronic granulomatous disease (CGD) in adults.

Design
Retrospective study.

Participants
4 adults with X-linked CGD. All patients were men who had CGD diagnosed during infancy or early childhood. The age range was 20-29 years, and mean age was 24. All patients were nonsmokers.

Methodology
Acute pulmonary infection was diagnosed based on clinical symptoms, findings of new abnormalities on chest radiograph, and resolution of symptoms after antimicrobial therapy or progression to death. A total of 5 episodes of pulmonary infection were analyzed.

Ten chest radiographs were obtained (5 during acute infection and 5 during follow-up), and 12 CT scans were obtained (5 during acute infection and 7 during follow-up). For the CTs, follow-ups were performed at a mean of 8.8 months (range, 1-20 months).

Review for Practicing Radiologists The University of California, San Francisco School of Medicine, Department of Radiology and Biomedical Imaging Review for Practicing Radiologists an intensive clinical radiologic review and self-assessment covering the following radiology subspecialties: Vascular-Interventional, Breast, Neuro, Gastrointestinal and Pulmonary Imaging. The program is designed for radiologists in clinical practice. Click here to read more or order: Review for Practicing Radiologists --

 

Body Imaging: Abdominal, Thoracic and Vascular University of California, San Francisco, Department of Radiology Course Director: Judy Yee, MD This program is designed for the radiologist in clinical practice, and is intended to provide an overview and update on clinically relevant topics in diagnostic imaging and interventional techniques. Click here to read more or order: Body Imaging

Results
All patients had abnormal findings on imaging. The chest radiographic findings were consolidation involving mainly the lower lobes in 60%, diffuse bilateral reticulonodular opacities in 40%, unilateral pleural effusion in 20%, and pulmonary artery enlargement in 20%.

The CT findings were consolidation involving mainly the lower lobes in 60%, ground-glass opacities in 40%, random pulmonary nodules (measuring up to 1.4 cm in diameter) in 60%, centrilobular nodules in 60%, tree-in-bud opacities in 40%, interlobular septal thickening in 40%, small unilateral pleural effusion in 20%, mediastinal or hilar adenopathy in 60%, areas of scarring, bandlike opacity, and bronchiectasis in 100% (upper lobe predominant), emphysema in 75% (upper lobe predominant), decreased attenuation associated with air trapping in 50%, pulmonary artery enlargement in 50%, and splenomegaly in 100%.

Consolidation, when present, demonstrated a slow decrease in size on follow-up in 2 patients and an increase in size in 1 patient who ultimately died due to respiratory failure and septic shock. On histology, consolidation and nodules were associated with either infection or granulomatous inflammation.

Conclusions
The most prominent findings for adults with CGD during episodes of infection are consolidation and nodules, which are due to infection or chronic granulomatous inflammation.

Reviewer’s Comments
The authors have nicely demonstrated the findings of this rare disease during episodes of infection in patients who have survived until adulthood. The protracted appearance of consolidation and nodules despite antimicrobial coverage and the presence of emphysema despite no history of smoking are interesting.

Take-Home Pearl
Chronic granulomatous disease in adults who have pulmonary infection usually manifests as consolidation and nodules which are slow to resolve.

Author: Vineet R. Jain, MD

Reference:
Godoy MC, Vos PM, et al. Chest Radiographic and CT Manifestations of Chronic Granulomatous Disease in Adults. AJR; 2008;191 (November): 1570-1575

Review for Practicing Radiologists The University of California, San Francisco School of Medicine, Department of Radiology and Biomedical Imaging Review for Practicing Radiologists an intensive clinical radiologic review and self-assessment covering the following radiology subspecialties: Vascular-Interventional, Breast, Neuro, Gastrointestinal and Pulmonary Imaging. The program is designed for radiologists in clinical practice. Click here to read more or order: Review for Practicing Radiologists --

 

Body Imaging: Abdominal, Thoracic and Vascular University of California, San Francisco, Department of Radiology Course Director: Judy Yee, MD This program is designed for the radiologist in clinical practice, and is intended to provide an overview and update on clinically relevant topics in diagnostic imaging and interventional techniques. Click here to read more or order: Body Imaging
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