title: Pneumocystis jiroveci infection in patients with hyper-immunoglobulin E syndrome.
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.
authors.affiliation: National Institute of Allergy and Infectious Disease, National Institutes of Health, 9000 Rockville Pike, Bethesda, MD 20892, USA. freemaal@mail.nih.gov
authors.forename: Alexandra F
authors.initials: AF
authors.lastname: Freeman
authors.forename: Joie
authors.initials: J
authors.lastname: Davis
authors.forename: Victoria L
authors.initials: VL
authors.lastname: Anderson
authors.forename: William
authors.initials: W
authors.lastname: Barson
authors.forename: Dirk N
authors.initials: DN
authors.lastname: Darnell
authors.forename: Jennifer M
authors.initials: JM
authors.lastname: Puck
authors.forename: Steven M
authors.initials: SM
authors.lastname: Holland
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D007589:Job Syndrome / Q000150:complications*; D008297:Male; D045363:Pneumocystis carinii; D011020:Pneumonia, Pneumocystis / Q000175:diagnosis / Q000209:etiology*