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Published online August 28, 2006
PEDIATRICS Vol. 118 No. 4 October 2006, pp. e1271-e1275 (doi:10.1542/peds.2006-0311)
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EXPERIENCE & REASON

Pneumocystis jiroveci Infection in Patients With Hyper–Immunoglobulin E Syndrome

Alexandra F. Freeman, MDa, Joie Davis, APRN, APNGb, Victoria L. Anderson, MSN, CRNPa, William Barson, MDc, Dirk N. Darnell, RN, MSNa, Jennifer M. Puck, MDb, Steven M. Holland, MDa

a National Institute of Allergy and Infectious Disease
b National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland
c Section of Infectious Diseases, Columbus Children's Hospital, Ohio State University College of Medicine, Columbus, Ohio

ABSTRACT

The hyper–immunoglobulin E syndrome is a primary immunodeficiency characterized by recurrent pyogenic skin and lung abscesses, dermatitis, and elevated serum immunoglobulin E levels. Pneumocystis jiroveci (formerly Pneumocystis carinii) is not typically associated with hyper–immunoglobulin E syndrome. We identified 7 patients with hyper–immunoglobulin E syndrome with P jiroveci detected in respiratory or pulmonary pathology specimens. In 5 patients it was the sole pathogen, and in 2 other patients it contributed to a polymicrobial etiology. No consistent prophylaxis was given, and there have been no recurrences on long-term follow-up. Our experience suggests that P jiroveci can cause pneumonia in patients with hyper–immunoglobulin E syndrome both with and without chronic lung disease.

Key Words: hyper-IgE syndrome • Pneumocystis jiroveci • immunodeficiency

Abbreviations: IgE, immunoglobulin E • HIES, hyper–immunoglobulin E syndrome • PJP, Pneumocystis jiroveci pneumonia • TMP/SMX, trimethoprim/sulfamethoxazole • ABPA, allergic bronchopulmonary aspergillosis • CT, computed tomography • FA, fluorescent antibody • DLCO, diffusion capacity • PCR, polymerase chain reaction

Accepted Apr 19, 2006.


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Pediatr. Rev., September 1, 2008; 29(9): 321 - 328.
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