title: [Pneumocystis carinii pneumonia in a patient with hyper-IgE syndrome].
abstract: A 15-year-old girl with abnormal findings detected on a medical check-up chest x-ray film was admitted to our center. High-resolution computed tomography, performed upon hospitalization, demonstrated panlobular nodular darkening in left lung fields, and an expanding, blended, map-like darkening near the pleura. Since a Grocott stain-positive cyst was confirmed histopathologically by transbronchial lung biopsy, the patient was given a diagnosis of Pneumocystis carinii pneumonia. Drug therapy was initiated with sulfamethoxaxole trimethoprim (Baktar), and on the 58th day, chest CT confirmed that the darkening observed at admission had virtually disappeared. Underlying diseases, such as AIDS, malignant lymphoma and secondary immunodeficiency caused by immunosuppressive agents or adrenocorticosteroids, were excluded as the cause of P. carinii pneumonia based on clinical/laboratory findings. Under the suspicion of the possibility of primary immunodeficiency, various immunological competence tests were performed. However, no abnormal findings indicating cell-mediated immunity, humoral immunity, complement immune function, neutrophil phagocytic capacity, or bactericidal capacity were recognized. Since significant increase of serum IgE suggested hyper-IgE syndrome, IgE antibody specific to Staphylococcal enterotoxin A and B, and the exotoxins of Staphylococcus aureus were measured with positive results. Since all three diagnostic criteria for hyper-IgE syndrome (i.e., high serum IgE values, positive IgE antibody specific to Staphylococcal enterotoxin and recurrent infection) were fulfilled, hyper-IgE syndrome was diagnosed. This is a rare case of hyper-IgE syndrome as a result of P. carinii pneumonia.
authors.affiliation: Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center.
authors.forename: Motoko
authors.initials: M
authors.lastname: Kubota
authors.forename: Noboru
authors.initials: N
authors.lastname: Takayanagi
authors.forename: Kazuyoshi
authors.initials: K
authors.lastname: Kurashima
authors.forename: Yousuke
authors.initials: Y
authors.lastname: Miyahara
authors.forename: Kenichirou
authors.initials: K
authors.lastname: Hara
authors.forename: Hiroo
authors.initials: H
authors.lastname: Saito
authors.forename: Daidou
authors.initials: D
authors.lastname: Tokunaga
authors.forename: Mikio
authors.initials: M
authors.lastname: Ubukata
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Yanagisawa
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Sugita
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Kawabata
mesh_terms: D000293:Adolescent; D005260:Female; D006801:Humans; D007589:Job Syndrome / Q000150:complications* / Q000175:diagnosis; D011020:Pneumonia, Pneumocystis / Q000209:etiology*