Study objectives: Previously, IPH patients have been
reported to have an average survival of 2.5 years. However, at our
institution, many IPH patients have survived longer than that.
Therefore, we conducted this study to determine the clinical course and
current mortality of pediatric IPH patients treated with
Design: Retrospective chart
Setting: Children’s hospital.
Participants: Seventeen patients in whom IPH was diagnosed
between 1972 and 1998.
Measurements and results: Mean
age at diagnosis was 4.5 ± 3.5 years, and 12 patients were female.
At diagnosis, all patients had anemia and pulmonary infiltrates; 85%
had hypoxemia, 65% had hemoptysis, and 70% had fever. The diagnosis
was made by open lung biopsy in 13 patients (76%), hemosiderin-laden
macrophages in BAL fluid in 1 patient (6%), hemosiderin-laden
macrophages in gastric aspirate in 2 patients (12%), or by clinical
presentation alone in 1 patient (6%). The mean duration of follow-up
for all patients was 3.6 ± 3.4 years (range, 0.7 to 10.2). Initial
treatment consisted of prednisone only in 14 patients (82%), and
prednisone and hydroxychloroquine in two patients (12%). Thirteen
patients (76%) required long-term corticosteroids because of recurrent
hemoptysis. Eight patients (47%) required other immunosuppressants
(hydroxychloroquine or azathioprine) in addition to prednisone to
control their hemoptysis. One patient who was not treated with
prednisone remained asymptomatic for 1.8 years. Three patients (17%)
died of acute massive pulmonary hemorrhage (4.1 ± 5.0 years
Conclusion: Five-year survival for IPH
patients in our study was 86% (by Kaplan-Meier method). We conclude
that these IPH patients who received long-term treatment had a better
outcome than those previously reported who were not treated with
extended courses of immunosuppressive therapy. We speculate that
long-term immunosuppression therapy may improve the prognosis in