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Hyperimmunoglobulinaemia E syndrome: pulmonary imaging features.

K S Jhaveri¹, D V Sahani, P G Shetty

¹Department of Imaging, PD Hinduja National Hospital, Mumbai, India. jhaverikartik@hotmail.com

Australasian Radiology

|September 7, 2000

Summary

Hyperimmunoglobulinaemia E syndrome, a rare infant-onset condition, causes severe infections and high IgE levels. Pulmonary imaging reveals lung infections, pneumatocoeles, and pneumothorax, aiding diagnosis.

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Area of Science:

Pediatric Pulmonology
Immunology
Radiology

Background:

Hyperimmunoglobulinaemia E (hyper-IgE) syndrome, also known as Job's syndrome, is a rare primary immunodeficiency.
It typically manifests in infancy with recurrent sinopulmonary and cutaneous infections.
A hallmark of the condition is extreme elevation of immunoglobulin E (IgE) levels.

Observation:

Pulmonary manifestations are common and include recurrent alveolar lung infections.
Pneumatocoeles (air-filled cysts in the lungs) are a characteristic finding.
Pneumothorax (collapsed lung) can also occur.

Findings:

Clinical history and other diagnostic data help exclude other causes of pneumatocoeles in children.
Computed tomography (CT) scans provide valuable insights into the pulmonary findings.

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CT imaging aids in the accurate diagnosis and management of patients with hyper-IgE syndrome.

Implications:

Early identification of pulmonary features is crucial for timely intervention.
CT imaging plays a significant role in characterizing lung abnormalities in hyper-IgE syndrome.
Understanding these imaging features improves patient management and outcomes for this rare disease.