Risk Factors for Recurrent Pulmonary Exacerbation in Idiopathic
Pulmonary Hemosiderosis
Abstract
Objectives: To evaluate the risk factors of recurrent pulmonary
exacerbation and poor prognosis in children with idiopathic pulmonary
hemosiderosis (IPH). Methods: In this multicenter study, 54 patinets
with diagnosis of IPH included. Medical records were retrospectively
reviewed from three tertiary care hospitals between 1979 and 2019. Also,
current information and the long-term progress of patients was
determined by contacting the families by telephone. Results: A total of
54 children were included. The median age of onset of symptoms was 4.5 ±
3.8 years. The median time from onset to diagnosis was 0.9 years ± 2.2.
The mean number of recurrent episodes per child in the
recurrence-positive group was 3.55 (1-15). Univariate analysis
demonstrated that patients presenting with hypoxia or requiring
transfusion at the time of presentation had significantly more
recurrence episodes (P=0.002). Multivariate analysis showed that the
presence of hypoxia at the time of initial presentation was a
significant independent predictor of recurrent episodes (P=0.027). The
median follow-up was 3.3 ± 4.8 years (0.75 months-27 years). There was a
significant relationship between the presence of hypoxia, transfusion
history, ANA positivity, and elevated transaminases at the time of
initial evaluation and treatment response. Conclusions: The present
study provides important information on the clinical course and outcome
of pediatric IPH, and substantial information regarding factors that
affect recurrent exacerbations and prognosis. Demonstrating of hypoxia
as an independent risk factor in recurrence episodes could be guide
physicians in the planning of treatment strategies.