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J Allergy Clin Immunol. 2007 May;119(5):1234-40. Epub 2007 Mar 1.

Causes of death in hyper-IgE syndrome.

Author information

1
National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892-1684, USA.

Abstract

BACKGROUND:

Hyper-IgE syndrome (HIES) is characterized by recurrent pyogenic infections, eczema, increased serum IgE levels, and a variety of connective tissue and skeletal system abnormalities. Little has been published regarding the causes of death in these patients or pathologic findings.

OBJECTIVE:

To identify the cause of death in patients with HIES and to describe pathologic findings in fatal HIES.

METHODS:

We reviewed the medical records and autopsy slides of 6 patients with HIES with autopsies performed at our institution.

RESULTS:

All 6 patients with HIES were women and ranged in age from 24 to 40 years. All patients had a history of cystic lung disease and had pneumonia at the time of death, with Pseudomonas aeruginosa and fungal organisms predominating. Pulmonary fungal vascular invasion with fatal hemorrhage was observed in 3 patients, and metastatic fungal disease to the brain was observed in 2 patients caused by Aspergillus fumigatus and Scedosporium prolificans. Four patients had evidence of renal tubular injury, which was likely from amphotericin B toxicity; 3 patients had glomerulosclerosis; and 1 patient had 2 kidney angiomyolipomas.

CONCLUSIONS:

Our series highlights the important role Pseudomonas and Aspergillus species play in patients with HIES with cystic lung disease. Intensified antifungal and gram-negative bacterial prophylaxis need evaluation as possible strategies to prevent these infectious complications in patients with cystic lung disease.

CLINICAL IMPLICATIONS:

Fungal and Pseudomonas infection of cystic lung disease in HIES may be life threatening, and the proper management and prevention of these infections need continued investigation.

PMID:
17335882
DOI:
10.1016/j.jaci.2006.12.666
[Indexed for MEDLINE]

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