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.

Association between idiopathic pulmonary hemosiderosis and celiac disease in pediatric patients: A scoping review of the literature over the past 50 years

“…Surprisingly, we did not find any patients with autoantibodies other than those consistent with CD in our cohort. A recent study has shown a higher incidence of recurrence and worse outcomes in patients with ANA positivity 48 . Similarly, the presence of ANCA antibodies has also been associated with a poorer prognosis 64,65 .…”

“…Several retrospective observational studies have reported the prevalence of celiac antibodies in pediatric patients with IPH. 11,42,48,49 The prevalence has varied between 17.6% and 40%. 50 Although only a small number of patients were tested and two of the studies 42,49 reported patients from the same French database, these studies suggest fairly high seropositivity for CD among patients with IPH.…”

“…This constellation is, however, uncommon in the pediatric population. 48 Hemoptysis is present in approximately 80% of adult patients. 1 In contrast, pediatric patients can inadvertently swallow the sputum and may not present with hemoptysis.…”

“…A recent study has shown a higher incidence of recurrence and worse outcomes in patients with ANA positivity. 48 Similarly, the presence of ANCA antibodies has also been associated with a poorer prognosis. 64,65 These findings have made us wonder whether IPH patients can be divided into three distinct subtypes, (a)…”

“…The classic presentation of IPH is the combination of hemoptysis, radiologic chest abnormalities, and anemia. This constellation is, however, uncommon in the pediatric population 48 . Hemoptysis is present in approximately 80% of adult patients 1 .…”