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 and 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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